Angelo Cuzalina, MD, DDS

• Private Practice - Tulsa Surgical Arts
• President, American Academy of Cosmetic Surgery 2011
• Chairman, AACS Cosmetic Surgery Fellowship Program
• Adjunct Clinical Assistant Professor of Surgery, Oklahoma
• State University
• Tulsa, Oklahoma

Abortive types were common in most epidemics anxiety pills purchase tofranil 25mg on-line, with symptoms capable of arousing suspicion during the epidemic but easily overlooked at other times anxiety symptoms everyday buy tofranil 75 mg on-line. Fever might accompany the prodromal phase or persist throughout social anxiety 50 mg tofranil overnight delivery, while other cases ran their whole course without pyrexia anxiety chat rooms purchase tofranil 75mg on line. Many abortive cases developed only the prodrome anxiety 8 year old son discount 75mg tofranil visa, while others recovered early after definite symptoms and signs had appeared anxiety symptoms similar to heart attack generic 25mg tofranil amex. Usually, however, the acute disturbances lasted for several weeks, with some months more before ocular palsies, lethargy and sleep disturbances resolved. A protracted convalescence was not uncommon, with repeated relapses and fresh exacerbations. Convalescence also brought prolonged asthenic states, incapacitating depressive illness and a variety of sleep disturbances: insomnia, sleep inversion and narcoleptic phenomena. Paralysis of external ocular movements or of isolated eye muscles was frequently permanent, also pupillary abnormalities, difficulty with accommodation and inability Chronic sequelae the most seriously disabling sequelae consisted of parkinsonian developments, change of personality and mental defect. The incidence of each varied in different epidemics, but a definite relationship emerged with regard to the age at which the acute infection had occurred. Adults tended to develop parkinsonism, children personality disturbances, and infants were left with mental defect. Generalised dementia did not appear to occur when the mature brain had been affected. Parkinsonism sometimes developed gradually out of the acute stage, or could set in unexpectedly after full recovery. In the interval the patient may have shown persistent symptoms such as headache, irritability and sleep disturbance but this was by no means invariable. Indeed as time went by it became apparent that sequelae could develop after many months or years of completely normal health. In contrast, personality change and mental defect were usually evident immediately after the acute infection. Postencephalitic parkinsonism this was the most common sequel and could develop even when parkinsonian symptoms had been absent during the acute phase. Its development was usually insidious, with weakness and slowing of movements or the gradual development of a stiff and unnatural posture. The ensuing picture closely resembled other forms of parkinsonism, with masklike face, stooping posture, festinant gait and excessive salivation. Tremor was less common than in paralysis agitans; the typical pill-rolling tremor was rarely seen, but coarser tremor and violent shaking of the limbs occasionally occurred. Paucity of movement was sometimes a striking sign even in the absence of paresis or marked rigidity. It appeared that in large degree this represented a primary disturbance of willed movement, such that the patient was unable to supply the volitional impulse despite a wish to perform. There might be much difficulty in passing from rest to activity, the patient remaining for minutes on end in a state of trance-like immobility. Or a movement once started might freeze halfway, as 446 Chapter 7 when raising a spoon to the mouth. Later typical rigidity developed, with extrapyramidal increase of tone that was obvious on examination. Characteristically, the akinesis and the rigidity could vary markedly, improving at some stage during the day, or allowing some activities while preventing others that required exactly the same musculature. Speech became slurred, jerky and monotonous, and writing was often strikingly small and cramped (micrographia). Other distinctive features were repetitive motor phenomena in the form of tics, blepharospasm, torticollis, spells of sighing and yawning, or complex respiratory spasms. Complicated motor stereotypies developed in advanced cases, for example stamping of the feet accompanied by writhing movements of the head and neck. Speech might show marked repetitive phenomena: of a phrase (echolalia), word (pallilalia) or syllable (logoclonia). A compulsive element was often very prominent indeed, and emerged in speech and thought as well as in motor behaviour. Compulsive thoughts and urges also appeared independently of the motor phenomena, with the patient ruminating endlessly on restricted themes or being driven to complex rituals. Compulsive urges sometimes led to trouble with the law, for example with repeated episodes of indecent exposure. It is of considerable theoretical interest that motor and psychological features of compulsion should so regularly have occurred together and in intimate association. Schilder (1938) considered that the compulsive phenomena could often be directly traced to motor sources. The encephalitic process liberated motor impulses, with a tendency towards impulsive actions of a sadistic nature, and when checked these led in turn to the compulsions. Oculogyric crises were another characteristic feature, again often intimately associated with compulsive phenomena. For a few minutes, or rarely hours, the eyes would deviate upwards or to the side, perhaps with contortions of the head, neck and extremities. At the onset the patient might be beset by some obsessive thought or enact some complex compulsive ritual. The crisis was sometimes accompanied by a fugue-like mental state, with inability to speak and lack of response to commands, or by marked affective disturbance: surges of depression, anxiety or fear, ideas of reference or feelings of persecution. Suggestibility was sometimes found to be an important factor, oculogyric attacks being provoked by talking about them or terminating in response to a sharp command. Attacks could also be precipitated by annoyance, shock or grief, and could be contagious in a ward of patients similarly affected. Thus again we see the complex admixture of motor and psychological phenomena that characterised the disease. The typical mental state in postencephalitic parkinsonism was of marked slowing (bradyphrenia) and lack of the normal fluidity of thought, though otherwise with good preservation of mental clarity. Torpor, irritability and disinclination for activity usually accompanied the compulsive elements of the disease (psychasthenia). Later, apathy became the striking emotional feature, with marked difficulty in arousing an affective reaction and little evidence of subjective distress. The parkinsonism itself usually advanced steadily, sometimes with intermittent progressions, but sometimes came to a halt with fixed residual defect. The combination of physical and mental disabilities inevitably meant that a large number of victims were permanently incapacitated for work, and such patients came to form a substantial proportion of the chronic mental hospital population. Postencephalitic personality change Children and young adolescents were mostly the victims of this serious development, but adults were not completely immune. It was estimated that approximately one-third of patients below the age of 16 developed some form of mental change after encephalitis lethargica. Frequently it was accompanied by other sequelae such as parkinsonism, sleep disturbance, obesity or other evidence of hypothalamic damage. The common change was towards overactivity and impulsive antisocial behaviour, as though the child now had lessened control over his instinctual drives. He became excited and restless, with inability to settle at school or remain occupied at any task for long. He was talkative, importunate and disinhibited, often indulging in stealing or sexual misbehaviour. Emotional lability was marked, with cheerful affectionate behaviour one moment and outbursts of anger the next. Moral and social senses were undermined, so that the child became destructive, abusive and hard to control. There was usually no primary intellectual deficit, although Intracranial Infections 447 as time went by education suffered severely or became impossible. Frequently the child appeared to be aware of the change in himself, to apologise repeatedly, yet immediately afterwards be compelled to err again. The subsequent course was often unfavourable, with worsening over the years leading eventually to institutionalisation. In later years some 50% developed parkinsonian changes, with ultimate benefit where the behaviour disorder was concerned (Slater & Roth 1969). Postencephalitic psychoses A variety of psychotic illnesses supervened in other patients on recovery from the acute stages. Depression and hypomania were relatively common, also paranoid­hallucinatory states and a variety of illnesses resembling schizophrenia. Hall (1929) described 18 patients from among 113 cases of encephalitis lethargica, mostly with manic­depressive psychoses or schizophrenia. They differed from the generality of psychoses in that delusions were more transient and variable, and even relatively mild depression was accompanied by profound retardation and immobility. Fairweather (1947) noted that 25% of men and 12% of women admitted to Rampton after encephalitis lethargica were deluded, mainly in a paranoid fashion. Paranoid­hallucinatory psychoses were estimated to occur in 15­30% of postencephalitics, and psychoses indistinguishable from paraphrenia or dementia praecox in 10% of those admitted to mental hospitals. All reported patients were selected for psychiatric disorder so the true frequency is unknown, but clearly such developments were not uncommon. Present-day encephalitis lethargica It would be a matter of some importance if sporadic cases of the disease were common and indeed some authors have suggested that the disease continues at a low endemic rate (Dale et al. This raises the possibility that there may be a substantial chance that the diagnosis could be overlooked, especially with mild affections, yet the sequelae might still dictate considerable psychiatric disability. The problem is difficult to resolve: the laboratory findings were variable when the disease was epidemic, and specific confirmatory tests were not achieved. Recently, however, the suggestion that the present-day encephalitis lethargica phenotype may be immunemediated has gathered momentum. Over half of these cases had suffered pharyngitis or tonsillitis prior to developing the illness, with an associated positive anti-streptolysin-O titre in two-thirds. Neuroimaging in 40% revealed inflammatory changes in basal ganglia and midbrain tegmentum. They suggest dopamine depletion followed by dopamine-receptor hypersensitivity as a neurochemical model for encephalitis lethargica. Dewar and Wilson (2005), in reporting a further case, review the cognitive outcomes of contemporary reports of encephalitis lethargica. They identify the limited formal evaluation of neuropsychological outcomes in the majority of case reports and go on to identify impaired executive function, concept formation, complex mental flexibility, verbal abstraction and non-verbal recall in their patient. One patient had a psychosis and a mute­akinetic syndrome associated with myoclonus. The second patient presented with a psychosis and fever, developing severe dyskinesias involving the mouth, trunk and limbs, together with respiratory irregularities and presumed hypothalamic disturbance and disturbance of consciousness. In case 1, levodopa improved the akinesia, while the myoclonus responded to clonazepam. In case 2, the severe dyskinesias failed to respond to a number of drugs, and she ultimately required paralysis to relieve her almost continuous movements. Both patients responded rapidly and dramatically to intravenous methylprednisolone. Pathological examination of the brain in two patients showed extensive loss of neurones from the substantia nigra and locus coeruleus, along with widespread neurofibrillary changes elsewhere in the brainstem, dentate nuclei and corpus striatum. They stressed that the diagnosis still rested essentially on the clinical features, and suggested that the following criteria be applied: an encephalitic illness, parkinsonism developing acutely or after a delay of months or years, alteration in the sleep cycle, oculogyric crises that are not drug induced, ocular or pupillary changes, respiratory disturbances, involuntary movements, corticospinal tract signs and mental abnormalities. While these represent the specific features of encephalitis lethargica, it is clear that not all will be present in every case. Johnson and Lucey (1987) have reported two suspected examples in young men, both presenting with severe catatonic stupor in the setting of depressive psychosis. One had a low-grade pyrexia and the other showed blepharospasm and complex compulsive rituals. The debate concerning latter-day examples was extended by Hunter and Jones (1966), who argued that sporadic cases might be appearing in mild or attenuated form and with clinical pictures increasingly dominated by psychiatric manifestations. Consequently, the neurological signs on which the diagnosis depends could readily be overshadowed. They reported six possible cases seen during a 3-month period in a psychiatric hospital. All had presented with psychiatric syndromes, and all had initially been seen at general hospitals where diagnoses of hypomania, depression and anxiety neurosis had been applied. All had worsened in the week or two before admission, with increasing agitation and depression, paranoid and bizarre bodily delusions, and nocturnal excitement and hallucinosis. On examination all showed some degree of mental confusion, three had mild pyrexia and all had some ocular abnormality. A variety of other neurological signs were present, often fluctuating from day to day. The authors suggested that the range of symptoms and presumed cerebral localisation was strongly reminiscent of encephalitis lethargica. It is extremely difficult to evaluate these examples, but perhaps encephalitic antecedents should more often be considered in the differential diagnosis of psychiatric patients. More than one-quarter showed minor biochemical abnormalities and some who underwent serial measures showed a return to normality when the clinical condition improved. Leigh (1946) and Espir and Spalding (1956) reported other cases in the context of acute influenza. Most had a history of progressive personality change over the course of several months, with irritability, emotionality, perplexed­paranoid developments and impaired memory and concentration. They complained of malaise, headache, lethargy, hypersomnia, insomnia, giddiness, blurred and double vision, and altered taste and A police cadet of 16 developed headache and later that afternoon was found unconscious in bed. In the evening he lost consciousness again and was admitted to the Radcliffe Infirmary, Oxford. All limbs were flaccid with normal tendon reflexes but with bilateral extensor plantar responses. The fever subsided next day but the level of consciousness fluctuated over the next 3 weeks. There were almost continuous involuntary movements of chewing, swallowing, yawning and writhing of the limbs.

Metabolic Hereditary Neuropathies Other members of this class are listed in the section on metabolic diseases (p anxiety symptoms in men buy 25mg tofranil free shipping. Porphyria Among the known porphyrias anxiety 5 senses discount 50 mg tofranil, four hepatic types are associated with encephalopathy and peripheral neuropathy: variegate porphyria anxiety symptoms ringing ears trusted tofranil 50 mg, acute intermittent porphyria anxiety symptoms brain fog 50 mg tofranil fast delivery, hereditary coproporphyria anxiety symptoms breathing cheap 50 mg tofranil with amex, and -aminolevulinic acid dehydrase deficiency (autosomal recessive; the others are autosomal dominant) anxiety while sleeping cheap tofranil 25 mg otc. Severe peripheral neuropathy is seen during attacks of acute porphyria, which are most often precipitated by medications and hormonal influences (also fasting, alcohol, and infection). The manifestations of porphyria include colicky abdominal pain, pain in the limbs, paresthesiae, tachycardia, and variable degrees of weakness. Encephalopathy is manifest as confusion, lack of concentration, somnolence, psychosis, hallucinations and/or epileptic seizures. The diagnosis of porphyria is based on the demonstration of porphyrin metabolites in the urine and feces. Neuropathy Due to Hereditary Disorders of Lipid Metabolism Polyneuropathy occurs in metachromatic leukodystrophy (p. The last is an autosomal recessive disorder of phytanic acid metabolism in which phytanic acid accumulation leads to tapetoretinal degeneration, night blindness, and a distal, symmetric polyneuropathy with peripheral nerve thickening. Peripheral Nerve and Muscle 333 Myopathies Myopathic Syndromes Myopathies are diseases of muscle. Many different hereditary and acquired diseases attack muscle, sometimes in combination with other organs. The diagnosis and classification of the myopathies have been transformed in recent years by the introduction of molecular biological tests for the hereditary myopathies, but their treatment remains problematic. The management of the hereditary myopathies currently consists mainly of genetic counseling and the attempt to provide an accurate prognosis. It may be local (restricted to the muscles of the eye, face, tongue, larynx, pharynx, neck, arms, legs, or trunk), proximal, or distal, asymmetric or symmetric. There may be muscle atrophy or hypertrophy, often in a typical distribution, whose severity depends on the type of myopathy. Skeletal deformity and/or abnormal posture may be a primary component of the disease or a consequence of weakness. Other features include acute paralysis, myoglobulinemia, cardiac arrhythmia, and visual disturbances. Causes For a list of causes of hereditary and acquired myopathies, see Tables 65 and 66, p. Various laboratory tests are helpful in myopathies due to biochemical abnormalities; imaging studies of muscle aid in the differential diagnosis of atrophy and hypertrophy. Peripheral Nerve and Muscle 335 Myopathies muscle biopsy is hardly ever necessary), and for prenatal diagnosis. Treatment the goal of treatment is to prevent contracture and skeletal deformity and to keep the patient able to sit and walk for as long as possible. The most important general measures are genetic counseling, social services, psychiatric counseling, and educating the patient on the special risks associated with general anesthesia. Physical therapy includes measures to prevent contractures, as well as breathing exercises (deep breathing, positional drainage, measures to counteract increased inspiratory resistance). Orthoses may be helpful, depending on the extent of weakness (night splints to prevent talipes equinus, seat cushions, peroneal springs, orthopedic corsets, leg orthoses). Home aids may be needed as weakness progresses (padding, eating aids, toilet/bathing aids, stair-lift, mechanized wheelchair, specially adapted automobile). Surgery may be needed to correct scoliosis, prevent contracture about the hip joint (iliotibial tract release), and correct winging of the scapula (scapulopexy/scapulodesis) and other deformities and contractures. Heart transplantation may be needed when severe cardiomyopathy arises in conjunction with certain types of muscular dystrophy (Becker, Emery­ Dreifuss; Table 68, p. Muscular Dystrophies the muscular dystrophies-myopathies characterized by progressive degeneration of muscle- are mostly hereditary. The functional features of dystrophin are not fully understood; it is thought to have a membrane-stabilizing effect. Emery­ Dreifuss muscular dystrophy is due to a mutation of the gene for emerin, a nuclear membrane protein whose exact function is unknown. Symptoms and Signs Muscular dystrophies may be characterized by atrophy, hypertrophy, or pseudohypertrophy and are further classified by their mode of inheritance, age of onset, and distribution. Other features such as myocardial involvement, contractures, skeletal deformity, endocrine dysfunction, and ocular manifestations may point to one or another specific type of muscular dystrophy. Peripheral Nerve and Muscle F-Actin Dystrophin Dystrobrevin Calpain-3 Hyperlordosis Myopathies the Myotonias (Table 69, p. Symptoms and Signs Symptoms and Signs In hypokalemic and hyperkalemic myotonia, there are irregularly occurring episodes of flaccid paresis of variable duration and severity, with no symptoms in between. In paramyotonia congenita, muscle stiffness increases on exertion (paradoxical myotonia) and is followed by weakness. Diagnosis the diagnosis can usually be made from the personal and family history, abnormal serum potassium concentration, and molecular genetic findings (mutation of the gene for a membrane ion channel). If the diagnosis remains in question, provocative tests can be performed between attacks. The induction of paralytic attacks by administration of glucose and insulin indicates hypokalemic paralysis, while their induction by potassium administration and exercise. The diagnosis of paramyotonia congenita is based on the characteristic clinical features (paradoxical myotonia, exacerbation by cold exposure), autosomal dominant inheritance, and demonstration of the causative point mutation of the sodium channel gene. Milder episodes of weakness in hypokalemic disorders need no treatment, while more severe episodes can be treated with oral potassium administration. Milder episodes of weakness in hyperkalemic disorders also need no treatment; more severe episodes may require calcium gluconate i. Hypokalemic paralysis: Low-salt, low-carbohydrate diet, avoidance of strenuous exercise; oral acetazolamide or spironolactone. Hyperkalemic paralysis: high-carbohydrate diet; avoidance of strenuous exercise and cold; oral hydrochlorothiazide or acetazolamide. Peripheral Nerve and Muscle the transient, involuntary muscle contractions are perceived as stiffness. Depolarizing muscle relaxants used in surgery can trigger severe myotonia in susceptible patients. Acute, generalized myotonia can also be induced by tocolytic agents such as fenoterol. Specific forms of myotonia are diagnosed by their mode of inheritance and clinical features, and molecular genetic analysis. The serum creatine kinase concentration is usually not elevated, and there is usually no muscle atrophy, except in myotonic dystrophy. Myotonic cataract is found in myotonic dystrophy and proximal myotonic myopathy; slit-lamp examination is indicated in patients with these disorders. Treatment Membrane-stabilizing drugs such as mexiletine alleviate myotonia; cardiac side effects may be problematic, particularly in myotonic dystrophy. Myopathies Unselective channel Chloride channel Potassium channel Cl- Sodium channel Calcium channel Na+ Ca2+ Extracellular matrix Cell membrane Action myotonia (delayed hand opening after grasping) Ion channels for maintenance of transmembrane potential Percussion myotonia (adduction of thumb on thenar percussion) Myopathic facies, weakness of lid closure, atrophy of anterior neck muscles, myotonic cataract Lingual percussion myotonia Cold exposure myotonia (delayed eye opening, facial rigidity) Paramyotonia congenita Predominantly distal muscular atrophy Myotonia congenita (generalized muscular hypertrophy) Myotonic dystrophy Rohkamm, Color Atlas of Neurology © 2004 Thieme All rights reserved. There is no etiological treatment for the mitochondrial myopathies at present; a lowfat, carbohydrate-rich diet is recommended in disorders with defective -oxidation, and carnitine supplementation in those with systemic carnitine deficiency. Coenzyme Q10, vitamin K3, vitamin C, and/or thioctic acid supplements are recommended in disorders with impaired respiratory chain function. Proximal flaccid weakness is usually present at birth (floppy baby); skeletal deformities may also be seen. Peripheral Nerve and Muscle 340 Metabolic Myopathies In most metabolic myopathies (Table 71, p. The mitochondrial myopathies are a heterogeneous group of disorders whose common feature is dysfunction of the respiratory chain, oxidation, or both. Circulating IgG autoantibodies to this receptor impair its function, speed its breakdown, and induce complement-mediated damage to the muscle cell membrane. The thymus plays an important role in this autoimmune disorder (it is normally a site of maturation and removal of autoreactive T lymphocytes). Weakness often appears first in the extraocular muscles and remains limited to them in some 15 % of cases (ocular myasthenia), but progresses to other muscles in the rest (generalized myasthenia). The facial and pharyngeal muscles may be affected, resulting in a blank facial expression, dysarthria, difficulty in chewing and swallowing, poor muscular control of the head, and rhinorrhea. Respiratory weakness leads to impairment of coughing and an increased risk of aspiration. It may become difficult or impossible for the patient stand up, remain standing, or walk, and total disability may ensue. The diagnosis is based on the characteristic history and clinical findings, supported by further tests that are listed in Table 74 (p. Further treatment depends on the degree of improvement achieved by these measures. It is characterized by proximal (leg) weakness that improves transiently with exercise but worsens shortly afterward. Small hemorrhages and telangiectasias are found in the nailbeds; affected children may have subcutaneous calcium deposits. The presence of antibodies in association with a connective tissue disease may be relevant to the diagnosis (p. Pathogenesis Most myositides found in the temperate zones are autoimmune diseases of unknown cause, characterized histologically by muscle inflammation and fibrosis and loss of muscle fibers. Vascular endothelial damage ultimately leads to ischemia and death of muscle tissue (¶ perifascicular atrophy). Inflammatory T cells and macrophages migrate into muscle and cause further damage. In overlap syndrome, myositis appears together with another autoimmune disease. Peripheral Nerve and Muscle 345 Lid edema Neuromuscular Disorders pain induced by normally nonpainful stimuli and is explained by the sensitization of nociceptors by pain-related substances such as bradykinin, serotonin, and prostaglandin. A "charleyhorse" is a type of myalgia that normally begins 8­24 hours after muscle overuse (simultaneous stretching and contraction) and lasts 5­7 days. Myalgia can be triggered by disorders whose primary pathology lies anywhere in the nervous system (peripheral nerve, spinal cord, brain). Pressure or traction on a muscle causes myalgia that subsides once the mechanical stimulus is removed, while inflammatory and other lesions in muscle cause persistent and gradually increasing myalgia. Muscle ischemia and/or metabolic dysfunction are reflected by myalgia occurring only during muscle activity. Myalgia includes allodynia, which is defined as Peripheral Nerve and Muscle Causes of Myalgia Type of Myalgia Localized myalgia ј Hematoma ј Myositis ј Ischemic ј Toxic-metabolic ј Overactivity ј Exerciseinduced ј Parkinsonian ј Muscle spasm ј Pain at rest Generalized myalgia ј Myositis ј Toxic-metabolic ј Other Selected Causes ј Trauma, coagulopathy ј Infectious: Streptococcal infection, trichinosis, influenza, epidemic pleurodynia. Noninfectious: Nodular focal myositis, eosinophilic fasciitis, sarcoidosis, myositis ossificans ј Arteriosclerosis (intermittent claudication), embolism ј Acute alcoholic myopathy, metabolic myopathy (pp. Rhabdomyolysis Local or generalized damage to skeletal muscle can cause myoglobinuria and an elevated serum concentration of creatine kinase, usually accompanied by the acute onset of proximal or diffuse weakness, with myalgia, muscle swelling, and general manifestations including nausea, vomiting, headache, and sometimes fever. The creatine kinase level may be chronically elevated in susceptible individuals, who can be identified with an in vitro contracture test performed in specialized laboratories. Malignant neuroleptic syndrome can also be induced by abrupt withdrawal of dopaminergic agents in patients with Parkinson disease. Remarkably, paraneoplastic syndromes sometimes appear months or years before the underlying malignancy becomes clinically manifest. Paraneoplastic neuromuscular syndromes typically present with marked weakness of subacute onset. Toxic Neuromuscular Syndromes the muscle fiber lesions regress if the responsible substance is eliminated in timely fashion (Table 75, p. Myopathy in Endocrine Disorders Hyperthyroidism or hypothyroidism, hyperparathyroidism, Cushing syndrome, steroid myopathy, and acromegaly all cause proximal weakness, while Addison disease and primary hyperaldosteronism usually cause generalized weakness. Timely correction of the endocrine disorder or withdrawal of steroid drugs is usually followed by improvement. It causes distal, symmetric weakness with prominent involvement of the muscles of respiration, resulting in prolonged ventilator dependence and delayed mobilization. Peripheral Nerve and Muscle 347 348 Rohkamm, Color Atlas of Neurology © 2004 Thieme All rights reserved. History and Physical Examination A detailed description of diagnostic evaluation procedures can be found in the textbooks listed on p. The goals of history-taking, physical examination, and additional testing (if necessary) are: ј Data collection (manifestations of disease) ј Localization of the lesion ј Provision of an etiological diagnosis Data Collection the diagnostic process begins with the history and physical examination. The physician engages the patient in a structured conversation about the manifestations of the illness. The physician must remember that the patient is the "expert" in this situation, as the patient alone knows what is troubling him (though perhaps helpful information can also be obtained from a close relative or friend). The physician aims to obtain accurate information on the nature, location, duration, and intensity of the symptoms by listening patiently and asking directed questions in an atmosphere of openness and trust. Questionnaires, computer programs, and ancillary personnel cannot be used for primary history-taking, as they do not enable the construction of a trusting physician­patient relationship (though they may provide useful additional information at a later stage). The general and neurological physical examination may yield important clues to the disease process, but only if the examiner has the requisite knowledge of the underlying principles of (neuro-)anatomy, (neuro-)physiology, and (neuro-)pathology. The unselective, "shotgun" application of every possible technique of neurological examination in every patient is not only a waste of time and money; it generally only creates confusion rather than clarifying the search for the diagnosis. The neurological examination of small children, patients with personality changes or mental illness, and unconscious patients poses special challenges. Orientation (to person, place, and time), attention, concentration, memory, thought processes, language function, level of consciousness. Olfaction, pupils, visual fields, eyegrounds, eye movements, facial movement, facial sensation, hearing, tongue movements, swallowing, speaking, reflexes. Muscular atrophy/hypertrophy, spontaneous movements, coordination, paresis, tremor, dystonia, muscle tone. Vague sensory abnormalities without other neurological deficits are difficult to classify; their interpretation requires a good knowledge of the underlying neuroanatomy (pp. The observation and testing of posture, station, and gait provides important information about a possible motor deficit (p.

Pts who respond to vascularization will typically have a reduction in bp of 25­30 mmHg systolic anxiety in the morning cheap tofranil 75 mg mastercard, generally within the first 48 h or so after the procedure anxiety symptoms loss of appetite generic 25 mg tofranil otc. For those with renal dysfunction anxiety and dizziness 25mg tofranil amex, only ~25% are expected to demonstrate renal improvement anxiety insomnia generic tofranil 75 mg line, with deterioration in renal function in another 25% and stable function in ~50% anxiety loss of appetite quality 25 mg tofranil. Malignant nephrosclerosis is characterized by accelerated rise in bp and the clinical features of malignant hypertension anxiety 54321 tofranil 25mg, including renal failure (Chap. Risk factors for progressive renal injury include a history of severe, longstanding hypertension; however, African Americans are at particularly high risk of progressive renal injury. Laboratory evaluation will usually reveal evidence of a microangiopathic hemolytic anemia, although this may be absent in certain causes. Stone formation begins when urine becomes supersaturated with insoluble components due to (1) low urinary volume, (2) excessive or insufficient excretion of selected compounds, or (3) other factors. Approximately 75% of stones are Ca-based (the majority Ca oxalate; also Ca phosphate and other mixed stones), 15% struvite (magnesium-ammonium-phosphate), 5% uric acid, and 1% cystine, reflecting the metabolic disturbance(s) from which they arise. Hyperoxaluria may be seen with intestinal (especially ileal) malabsorption syndromes. Struvite stones form in the collecting system when infection with urea-splitting organisms is present. Uric acid stones develop when the urine is saturated with uric acid in the presence of an acid urine pH; pts typically have underlying metabolic syndrome and insulin resistance, associated with a relative defect in ammoniagenesis and urine pH that is <5. Cystine stones are the result of a rare inherited defect in renal and intestinal transport of several dibasic amino acids; the overexcretion of cystine (cysteine disulfide), which is relatively insoluble, leads to nephrolithiasis. Stones begin in childhood and are a rare cause of staghorn calculi; they occasionally lead to endstage renal disease. Table 154-1 outlines a reasonable workup for an outpatient with an uncomplicated kidney stone. Careful medical history and physical examination, focusing on systemic diseases 3. Nephrolithiasis Treatment of renal calculi is often empirical, based on odds (Ca oxalate stones most common), clinical Hx, and/or the metabolic workup. Table 154-2 outlines stone-specific therapies for pts with complex or recurrent nephrolithiasis. It is preponderant in women (pelvic tumors), elderly men (prostatic disease), diabetic pts (papillary necrosis), pts with neurologic diseases (spinal cord injury or multiple sclerosis, with neurogenic bladder), and individuals with retroperitoneal lymphadenopathy or fibrosis, vesicoureteral reflux, nephrolithiasis, or other causes of functional urinary retention. Physical exam may reveal an enlarged bladder by percussion over the lower abdominal wall. Urinalysis is most often benign or with a small number of cells; heavy proteinuria is rare. It should be noted that unilateral obstruction may be prolonged and severe (ultimately leading to loss of renal function in the obstructed kidney), with no hint of abnormality on physical exam and laboratory survey. Fluid and electrolyte status should be carefully monitored after obstruction is relieved. It is also found in normals (increasing prevalence with age) and in those of low socioeconomic status. Duodenal Ulcer Mild gastric acid hypersecretion resulting from (1) increased release of gastrin, presumably due to (a) stimulation of antral G cells by cytokines released by inflammatory cells and (b) diminished production of somatostatin by D cells, both resulting from H. Gastric acid secretory rates are usually normal or reduced, possibly reflecting earlier age of infection by H. Clinical Features Duodenal Ulcer Burning epigastric pain 90 min to 3 h after meals, often nocturnal, relieved by food. Similar symptoms may occur in persons without demonstrated peptic ulcers ("nonulcer dyspepsia"); less responsive to standard therapy. Complications Bleeding, obstruction, penetration causing acute pancreatitis, perforation, intractability. Gastric Ulcer Upper endoscopy preferable to exclude possibility that ulcer is Detection of H. Ranitidine bismuth citrate plus Tetracycline plus Clarithromycin or metronidazole 3. Omeprazole (lansoprazole) plus Clarithromycin plus Metronidazoleb or Amoxicillinc Quadruple Therapy Omeprazole (lansoprazole) Bismuth subsalicylate Metronidazole Tetracycline Sequential Therapy Pantoprazole Amoxicillin Clarithromycin Tinidazole aAlternative: bAlternative: cUse Dose 2 tablets qid 250 mg qid 500 mg qid 400 mg bid 500 mg bid 500 mg bid 20 mg bid (30 mg bid) 250 or 500 mg bid 500 mg bid 1 g bid 20 mg (30 mg) daily 2 tablets qid 250 mg qid 500 mg qid 40 mg bid days 1­10 1 g bid days 1­5 500 mg bid days 6­10 500 mg bid days 6­10 use prepacked Helidac. Other options include trial of acid-suppressive therapy, endoscopy only in treatment failures, or initial endoscopy in all cases. Pt may be asymptomatic or experience epigastric discomfort, nausea, hematemesis, or melena. Erosive Gastropathies Removal of offending agent and maintenance of O 2 and blood volume as required. Chronic Gastritis Identified histologically by an inflammatory cell infiltrate dominated by lymphocytes and plasma cells with scant neutrophils. In its early stage, the changes are limited to the lamina propria (superficial gastritis). Differential Diagnosis Increased Gastric Acid Secretion Z-E syndrome, antral G cell hyperplasia or hyperfunction (? Normal or Decreased Gastric Acid Secretion Pernicious anemia, chronic gastritis, gastric cancer, vagotomy, pheochromocytoma. Clinical Manifestations Bloody diarrhea, mucus, fever, abdominal pain, tenesmus, weight loss; spectrum of severity (majority of cases are mild, limited to rectosigmoid). Complications Toxic megacolon, colonic perforation; cancer risk related to extent and duration of colitis; often preceded by or coincident with dysplasia, which may be detected on surveillance colonoscopic biopsies. Diagnosis Sigmoidoscopy/colonoscopy: mucosal erythema, granularity, friability, exudate, hemorrhage, ulcers, inflammatory polyps (pseudopolyps). Clinical Manifestations Fever, abdominal pain, diarrhea (often without blood), fatigue, weight loss, growth retardation in children; acute ileitis mimicking appendicitis; anorectal fissures, fistulas, abscesses. Clinical course falls into three broad patterns: (1) inflammatory, (2) stricturing, and (3) fistulizing. Others Ischemic bowel disease, appendicitis, diverticulitis, radiation enterocolitis, bile salt­induced diarrhea (ileal resection), drug-induced colitis. Toxicity (generally due to sulfapyridine component): dose-related- nausea, headache, rarely hemolytic anemia-may resolve when drug dose is lowered; idiosyncratic-fever, rash, neutropenia, pancreatitis, hepatitis, etc. Onset associated with a change in form (appearance) of stool aCriteria fulfilled for the last 3 months with symptom onset at least 6 months prior to diagnosis. Consider sigmoidoscopy and barium radiographs to exclude inflammatory bowel disease or malignancy; consider excluding giardiasis, intestinal lactase deficiency, hyperthyroidism. Irritable Bowel Syndrome (Table 158-2) Reassurance and supportive physician-pt relationship, avoidance of stress or precipitating factors, dietary bulk (fiber, psyllium extract. Selective serotonin reuptake inhibitors such as paroxetine are being evaluated in constipation-dominant pts, and serotonin receptor antagonists such as alosetron are being evaluated in diarrhea-dominant pts. Intestinal Pseudoobstruction For acute attacks: intestinal decompression with long tube. Postoperative anticoagulation indicated in mesenteric venous thrombosis, controversial in arterial occlusion. Chronic Mesenteric Insufficiency "Abdominal angina": dull, crampy periumbilical pain 15­30 min after a meal and lasting for several hours; weight loss; occasionally diarrhea. Sigmoidoscopy shows submucosal hemorrhage, friability, ulcerations; rectum often spared. For bleeding, treat by colonoscopic electro- or laser coagulation, band ligation, arteriographic embolization, or, if necessary, right hemicolectomy (Chap. Pruritus Ani Often of unclear cause; may be due to poor hygiene, fungal or parasitic infection. Treat with thorough cleansing after bowel movement, topical glucocorticoid, antifungal agent if indicated. Anal Condylomas (Genital Warts) Wartlike papillomas due to sexually transmitted papillomavirus. Epidemiology One million new cases of cholelithiasis per year in the United States. Symptoms occur when stones produce inflammation or obstruction of the cystic or common bile ducts. Laboratory Occasionally, mild and transient elevations in bilirubin [<85 mol/L (<5 mg/dL)] accompany biliary colic. The oral cholecystogram has been largely replaced by ultrasound but may be used to assess the patency of the cystic duct and gallbladder emptying function (Table 159-1). Cholelthiasis In asymptomatic patients, risk of developing complications requiring surgery is small. Patients with gallstones > 3 cm or with an anomalous gallbladder containing stones should also be considered for surgery. Laparoscopic cholecystectomy is minimally invasive and is the procedure of choice for most patients undergoing elective cholecystectomy. Oral dissolution agents (ursodeoxycholic acid) partially or completely dissolve small radiolucent stones in 50% of selected patients within 6­24 months. Because of the frequency of stone recurrence and the effectiveness of laparoscopic surgery, the role of oral dissolution therapy has been reduced to selected patients who are not candidates for elective cholecystectomy. Inflammatory response is evoked by: (1) mechanical inflammation from increased intraluminal pressure; (2) chemical inflammation from release of lysolecithin; (3) bacterial inflammation, which plays a role in 50­85% of patients with acute cholecystitis. Imaging Ultrasonography is useful for demonstrating gallstones and occasionally a phlegmonous mass surrounding the gallbladder. Differential Diagnosis Includes acute pancreatitis, appendicitis, pyelonephritis, peptic ulcer disease, hepatitis, and hepatic abscess. Complications Empyema, hydrops, gangrene, perforation, fistulization, gallstone ileus, porcelain gallbladder. Urgent cholecystectomy is appropriate in most patients with a suspected or confirmed complication. Delayed surgery is reserved for patients with high risk of emergent surgery and where the diagnosis is in doubt. Symptoms and Signs May be asymptomatic for years, may progress to symptomatic gallbladder disease or to acute cholecystitis, or present with complications. Imaging Ultrasonography preferred; usually shows gallstones within a contracted gallbladder (Table 159-1). Differential Diagnosis Peptic ulcer disease, esophagitis, irritable bowel syndrome. Laboratory Elevations in serum bilirubin, alkaline phosphatase, and aminotransferases. Leukocytosis usually accompanies cholangitis; blood cultures are frequently positive. Differential Diagnosis Acute cholecystitis, renal colic, perforated viscus, pancreatitis. Complications Cholangitis, obstructive jaundice, gallstone-induced pancreatitis, and secondary biliary cirrhosis. Laboratory Evidence of cholestasis (elevated bilirubin and alkaline phosphatase) common. Radiology/Endoscopy Transhepatic or endoscopic cholangiograms reveal stenosis and dilation of the intra- and extrahepatic bile ducts. Urodeoxycholic acid improves liver tests, but has not been shown to affect survival. Common symptoms: (1) steady, boring midepigastric pain radiating to the back that is frequently increased in the supine position; (2) nausea, vomiting. Serum amylase: Large elevations (>3 Ч normal) virtually assure the diagnosis if salivary gland disease and intestinal perforation/infarction are excluded. Urinary amylase­creatinine clearance ratio: no more sensitive or specific than blood amylase levels. Abdominal radiographs are abnormal in 30­50% of patients but are not specific for pancreatitis. Differential Diagnosis Intestinal perforation (especially peptic ulcer), cholecystitis, acute intestinal obstruction, mesenteric ischemia, renal colic, myocardial ischemia, aortic dissection, connective tissue disorders, pneumonia, and diabetic ketoacidosis. The benefit of antibiotic prophylaxis in necrotizing acute pancreatitis remains controversial. Patients with severe gallstone-induced pancreatitis often benefit from early (<3 days) papillotomy. Complications It is important to identify patients who are at risk of poor outcome. Risk factors that adversely affect survival in acute pancreatitis are listed in Table 160-2. Sterile or infected pancreatic necrosis-necrosis may become secondarily infected in 40­60% of patients, typically within 1­2 weeks after the onset of pancreatitis. Laparotomy with removal of necrotic material and adequate drainage should be considered for patients with sterile acute necrotic pancreatitis, if patient continues to deteriorate despite conventional therapy. In patients who are stable and uncomplicated, treatment is supportive; pseudocysts that are >5 cm in diameter and persist for >6 weeks should be considered for drainage. In patients with an expanding pseudocyst or one complicated by hemorrhage, rupture, or abscess, surgery should be performed. Pancreatic ascites and pleural effusions are usually due to disruption of the main pancreatic duct. Etiology Chronic alcoholism is most frequent cause of pancreatic exocrine insufficiency in U. Differential Diagnosis Important to distinguish from pancreatic carcinoma; may require radiographically guided biopsy. Patients unable to maintain adequate hydration should be hospitalized, while those with milder symptoms can be managed on an ambulatory basis. Complications Vitamin B12 malabsorption in 40% of alcohol-induced and all cystic fibrosis cases. Hepatitis B may be associated with immune-complex phenomena, including arthritis, serum sickness­like illness, glomerulonephritis, and a polyarteritis nodosa­like vasculitis.

Neuroimaging may show the intracranial lesions or evidence of basal meningitis anxiety nervousness purchase tofranil 75mg online, and lumbar puncture may reveal evidence of chronic meningitis anxiety questions purchase 25mg tofranil overnight delivery. The prognosis for patients with neuropsychiatric complications is extremely variable anxiety 7 scoring interpretation buy generic tofranil 75 mg online, but in general intracranial involvement should be viewed as a grave development anxiety symptoms for 2 weeks order tofranil 50mg line. Some show a remittent picture anxiety blog generic 75mg tofranil with amex, others slow and incomplete recovery anxiety symptoms muscle cramps tofranil 25mg mastercard, while others show progressive disability. More prolonged sequelae include cranial nerve damage with loss of taste, facial paresis and auditory and vestibular disturbances. However, the most common sequelae are peripheral nerve and spinal cord syndromes that are sometimes days or weeks in developing, perhaps as vascular changes progress. Farrell and Starr (1968) stress that a latent period of months may occasionally intervene. The pictures that have been described include delayed atrophy affecting an arm or leg, quadriparesis, or slowly progressive spasticity with sensory changes (so-called spinal atrophic paralysis). In patients such as these the current may have traversed the spinal cord directly. Prolonged neuropsychiatric aftermaths include amnesia and impaired cognitive functioning, often compounded by anxiety attaching to the shock and sometimes by compensation issues. The following patient illustrates the cognitive and personality changes that may occasionally follow. These risks increase in relation to the depth and duration of submersion and, perhaps not surprisingly, are increased in younger and less experienced divers (Newton et al. They apply equally to caisson workers who may spend several days at a time working at very considerable depths. When explosives are used divers are also exposed to increased risk of blast injury because of the enhanced transmission of pressure waves in water. Repeated exposures appear to be a special hazard in persons with a patent foramen ovale or other form of right-to-left shunt in the circulation. These matters are considered below, much deriving from accounts by Denison (1996) and Wilmshurst (1997). A 26-year-old man was rendered unconscious for several hours after sustaining a shock from bare wires which had made contact with his forehead. On recovery he had throbbing headache, was sluggish in cerebration, and complained of feeling depressed and irritable for several weeks thereafter. Two months later he was still vague and forgetful with delayed responses to questions. Five months after the accident he showed significant impairment of memory, lability of mood and psychomotor retardation. His girlfriend described a marked change of personality, with argumentativeness and occasional aggressive behaviour. Cognitive testing showed nominal and expressive dysphasia, impaired right­left discrimination, and difficulty in making simple drawings. He had lost his ability to speak German, which he had learned during the previous 3 years. Two years later his memory remained impaired and he was still mentally sluggish and rather vacuous in appearance. His mother confirmed a marked change of personality from a bright extroverted person to one who was slow, sullen and withdrawn. Though no longer depressed he was distractible, and had abandoned his reading and former hobbies. Gas toxicity the gases breathed during submersion must be delivered at the same pressure as the surrounding water. Scuba divers breathe compressed air but are restricted to relatively shallow depths (30­50 m) because of the risk of nitrogen narcosis, which is probably due to the narcotic effects of nitrogen dissolved in nerve membranes impeding neural transmission. The breathing of pure oxygen does not solve the problem, because oxygen is toxic to the lungs when alveolar pressure exceeds 50 kPa (0. Deeper dives are accomplished using a mix of oxygen and helium and this has allowed divers to reach astonishing depths (below 600 m). However, deeper than 160 m the risk of high-pressure nervous syndrome increases considerably, resulting in tremor, myoclonic jerks, nausea, vomiting, fatigue, postural instability, somnolence and cognitive dysfunction and other symptoms (Bennett & McLeod 1984). Decompression illness During submersion inert gases under pressure become dissolved in body tissues: nitrogen when breathing air, or helium when breathing oxygen­helium mixtures. In the course of the ascent such gases come out of solution as the ambient pressure falls, tending to form bubbles within the tissues and the blood (gas nucleation). Provided the ascent is sufficiently gradual the extra load of gas diffuses into the bloodstream and out of the lungs, but if it is too rapid the bubbles increase in size and number and may come to block blood vessels. Pulmonary symptoms consist of sudden chest pain, dyspnoea and cough due to bubble formation within the pulmonary circulation. Neurological symptoms, which occur in about half of cases, consist chiefly of spinal cord syndromes, visual disturbances or vertigo, although central focal deficits may occur. The range of severity is wide, from slight dysaesthesiae, ataxia and ophthalmoplegia to paraparesis, quadriparesis, dysphasia and confusion. The episodes are sometimes recurrent, in general resembling thromboembolic cerebrovascular disease except for commonly affecting the cord. The symptoms usually develop some minutes to hours after the dive is over, and must be treated immediately by recompression and the administration of oxygen. In an examination of the spinal cords of 11 divers, mostly dying from diving accidents, they found distended empty blood vessels, sometimes with perivascular haemorrhages, and minor chronic changes with foci of gliosis and hyalinisation of blood vessels. In three cases Marchi staining showed tract degeneration, variously affecting the posterior, lateral or anterior columns of the cord. Examination of the brains of 25 divers, again mostly dying from diving accidents, showed distended empty vessels in two-thirds of subjects, presumably caused by gas bubbles (Palmer et al. Perivascular lacunae were present in one-third, presumably due to bubble occlusion, along with hyalinisation of blood vessels which may have accrued from periodic rises in luminal pressure. Foci of necrosis were sometimes observed in the cerebral grey matter, and vacuolation in the white matter extending to status spongiosis. Sequelae of diving A well-known long-term effect of diving is the presence of aseptic infarcts in the long bones, evident on radiography and presumably due to gas embolism. Infarcts near the articular surfaces can be severely disabling, and crippling dysbaric osteonecrosis may occasionally ensue. At the time of examination 20% had stopped diving and six had lost their licenses because of neurological problems; 12 (8%) had had problems with vision, vertigo or reduced skin sensitivity in non-diving situations, and six had been referred to neurological clinics on account of seizures, transient cerebral ischaemia or transient amnesia attacks. On examination significantly more showed hand tremor, or signs indicative of cord damage such as reduced touch and pain sensation in the feet. In a study of construction divers matched to controls, the divers had significantly different error rates in tasks of reference memory and navigation behaviours (Leplow et al. Shallow water diving is a variant used professionally for collection of shellfish and recreationally, where instead of using scuba equipment the divers hold their breath. In a large study of professional abalone divers the incidence of deficits in visual function, psychomotor abilities and recent memory was related to individual characteristics in the divers and attributed to their diving technique (Williamson et al. Nevertheless, the possibility arises that divers with right-to-left shunts may be at particular risk of accumulating microinfarcts in the brain. The great majority of such shunts are likely to reflect a patent foramen ovale, which may well become functional only under the abnormal pressure conditions of diving. Others could be due to small atrial septal defects or pulmonary arteriovenous shunts. The radiological picture is of thickening of the inner tables of the frontal bones, with smooth rounded exostoses projecting into the cranial cavity. Part of the problem in discerning any putative clinical associations lies with the frequency of the condition and with the occurrence of minor variations. It may be found at any age from adolescence upwards, increasing markedly from the third or fourth decades onwards. While the pattern of inheritance is not understood, it does occur in families and identical twin-pairs have been reported (Koller et al. As the bone abnormalities are so easily identified in skeletal remains, the condition has frequently been diagnosed in ancient populations, medieval and prehistoric (Hershkovitz et al. In most reviews the main features have been headache, obesity, hirsutism and menstrual disorders (Capraro et al. Thirst, water retention, sleep disturbances and a variety of rather minor endocrine changes are also described. Among mental features neurotic complaints figure prominently, also disturbances of personality, memory impairment and occasionally dementia. Agenesis of the corpus callosum Absence of the corpus callosum, in whole or in part, occurs as a developmental abnormality, perhaps in as many as 1 in 4000 births (Paul et al. In complete agenesis other associated defects may be present: hydrocephalus, microgyria, heterotopias, arachnoid cysts, spina bifida or meningomyelocele. The anterior and hippocampal commissures may be intact even when the corpus callosum is entirely missing. Most cases have been reported in children, although the condition can come to light at any age. It usually presents by virtue of symptoms attributable to other cerebral malformations: seizures, mental retardation or hydrocephalus. There are some indications that it may be commoner than chance expectation in patients with organic psychosyndromes. Other Disorders of the Nervous System 893 the course of neuroradiological investigations carried out for some other purpose. The discovery of asymptomatic cases is likely to increase now that brain imaging is so frequently performed. It appears sometimes to be the result of intrauterine metabolic disturbances such as hyperglycinaemia, or intrauterine exposure to infections and toxins. Epilepsy, spasticity and other motor defects are common, likewise varying grades of mental subnormality especially in cases of partial agenesis and when associated with other conditions. However, when patients with other malformations are excluded, intelligence is usually in the normal range. There has been a renewal of interest in the psychological status of such patients in view of the abnormal functioning known to follow surgical section of the commissures and the interest in disconnection syndromes (reviewed by David et al. Various cognitive deficits have been demonstrated, in particular on tests of bimanual coordination and in crossed-responding to visual stimuli and in the matching of visual patterns between left and right visual fields. However, all such deficits are variable, and other tests of interhemispheric transfer appear often to be well performed. Compensatory mechanisms must clearly be at work, by way of bilateral speech representation, increased use of ipsilateral inflow pathways, or utilisation of such other commissural pathways as are intact. Milner (1983) provides a valuable review of neuropsychological studies in such patients, concluding that while it is likely that both cognitive and skilled performances can suffer, there are clearly great individual differences from one case to another. In particular, there is no good evidence that acallosal brains are less laterally specialised than normal brains, despite conflicting findings on the issue. These have included schizophrenia, depression and behavioural disorders of childhood including overlap with autism spectrum disorders. No conclusions could be drawn concerning the relevance of the callosal abnormalities to these clinical manifestations, not least because the prevalence of callosal anomalies in the general population is uncertain. The radiological diagnosis based on the air encephalogram was first described by Bull (1967). Marked separation is seen between the lateral ventricles; they show angular dorsal margins, concave medial borders and dilatation of the caudal portions. Characteristic findings are seen in relation to the pericallosal arteries and other vessels on angiography. Acknowledgements the editor is grateful to Dr Madhav Thambisetty for his invaluable contributions to this chapter. Value of computed tomographic scanning and magnetic resonance imaging of the brain. Professor Neurological Clinic Nordwest-Krankenhaus Sanderbusch Sande, Germany 172 illustrations by Manfred Gьther Translation revised by Ethan Taub, M. Thieme Stuttgart · New York Rohkamm, Color Atlas of Neurology © 2004 Thieme All rights reserved. Library of Congress Cataloging-in-Publication Data is available from the publisher. This book is an authorized translation of the 2nd German edition published and copyrighted 2003 by Georg Thieme Verlag, Stuttgart, Germany. Research and clinical experience are continually expanding our knowledge, in particular our knowledge of proper treatment and drug therapy. Insofar as this book mentions any dosage or application, readers may rest assured that the authors, editors, and publishers have made every effort to ensure that such references are in accordance with the state of knowledge at the time of production of the book. Nevertheless, this does not involve, imply, or express any guarantee or responsibility on the part of the publishers in respect to any dosage instructions and forms of applications stated in the book. Every user is requested to examine carefully the manufacturers` leaflets accompanying each drug and to check, if necessary in consultation with a physician or specialist, whether the dosage schedules mentioned therein or the contraindications stated by the manufacturers differ from the statements made in the present book. Such examination is particularly important with drugs that are either rarely used or have been newly released on the market. The authors and publishers request every user to report to the publishers any discrepancies or inaccuracies noticed. This applies in particular to photostat reproduction, copying, mimeographing, preparation of microfilms, and electronic data processing and storage. Preface the nervous system and the muscles are the seat of many primary diseases and are affected secondarily by many others. This pocket atlas is intended as an aid to the detection and diagnosis of the symptoms and signs of neurological disease. The text and illustrations are printed on facing pages, to facilitate learning of the points presented in each. Chapter 2 concerns the functions of the nervous system and the commonly encountered syndromes in clinical neurology.

The diverse cognitive and behavioural effects of thalamic infarcts anxiety symptoms ringing ears cheap tofranil 25 mg amex, which can partly be explained by which thalamic nuclei are involved anxiety 24 weeks pregnant tofranil 50 mg amex, have recently been reviewed (Carrera & Bogousslavsky 2006) anxiety symptoms in dogs 50 mg tofranil sale. Personality change anxiety symptoms stomach pain discount tofranil 25mg overnight delivery, particularly disinhibition and apathy anxiety symptoms urinary generic tofranil 75mg visa, executive difficulties often with perseveration anxiety symptoms vibration generic tofranil 75 mg on-line, and occasionally psychotic mood change may follow. Central pain may be more common after right-sided thalamic lesions (Nasreddine & Saver 1997). Quite often both paramedian thalamic nuclei are supplied by a single paramedian artery, which partly explains why bilateral thalamic infarcts are not all that uncommon (Schmahmann 2003). Lacunar infarcts do not result in any disturbance of higher cortical function or visual field deficit. Pure motor hemiparesis, due to lacunes in the posterior limb of the internal capsule or pons, hemisensory stroke, often with lacunes in the posterolateral thalamus, and ataxic hemiparesis (dysarthria clumsy hand syndrome or ipsilateral ataxia with crural hemiparesis) are seen. The deficits resulting from lacunar infarcts are usually slight and recover rapidly and the risk of death early after the stroke is relatively small. However, after several years 10% or more, particularly those with leucoaraiosis, will develop a vascular dementia (Norrving 2003) (described in Chapter 9), sometimes accompanied by a pseudobulbar palsy. This definition was introduced in an attempt to distinguish episodes that resulted in no permanent brain infarction from stroke. The emboli may consist of platelet aggregates, cholesterol or small fragments of thrombus. Thus a sudden fall in cardiac output, or systemic hypotension due to any cause, may compromise flow in vessels already critically narrowed by atherosclerosis. Anaemia or polycythaemia may be background factors facilitating the development of attacks. Microemboli may actually be observed in the retinal arteries on ophthalmoscopy in such cases. Motor impairments may take the form of a brief monoparesis involving only part of a limb. Mental confusion may occasionally be marked but in general non-focal symptoms are absent. Episodes of paresis or numbness may involve different sides of the body in successive attacks. Drop attacks are commonly attributable to such a cause in the elderly­the person falls abruptly to the ground without loss of consciousness, then can rise immediately ­ as a result of acute and transient failure of the antigravity muscles. Sometimes a staggering ataxia may be combined with dysarthria and drowsiness, leading to an impression of drunkenness. Visual phenomena include blurred vision, altitudinal or homonymous hemianopias, or scintillation scotomata. One-tenth of patients will have a stroke within 90 days and one-quarter within 5 years; 20% will have had a stroke or myocardial infarct or be dead by 1 year. Other high-risk factors for subsequent stroke include having a cardioembolic source, being over 60 years of age, and having diabetes mellitus or hypertension. Weakness or speech impairment during the episode also increases the risk (Shah & Edlow 2004). Once neuroimaging has excluded the possibility of an intracerebral bleed, antiplatelet agents. Anticoagulation has no value unless there is clear indication of a cardiac source of emboli, for example atrial fibrillation, prosthetic heart valve or recent myocardial infarct. Carotid endarterectomy has an important role in the prevention of later infarction in patients with greater than 70% stenosis of the carotid artery (Barnett et al. Neuroimaging of stroke the acute management of stroke, within the first few hours of onset, goes hand in hand with neuroimaging protocols, 480 Chapter 8 developed over the last few years, designed to help the clinician decide whether intravenous thrombolysis is indicated (Masdeu et al. Having confirmed that the neuroimaging findings are indeed those of a vascular lesion, the first priority is then to separate haemorrhagic stroke, in which case thrombolysis is contraindicated, from ischaemic stroke. Subsequently, assessment of the extent of ischaemic but salvageable brain (the penumbra) around the core of non-viable tissue may improve selection of those most likely to benefit from thrombolysis. Neuroimaging may also help identify the cause of the stroke, for example picking up multiple cortical infarcts suggestive of cardiac emboli. In the acute stage, infarcts of the major cerebral arteries are considered to produce three zones of hypoperfused tissue (Muir et al. The core of the infarct, where the opportunity for anastomotic perfusion is least, is defined as the zone where cells will inevitably die. Within the core there may be a zone where only neurones die, surrounding a zone where the ischaemia is so severe that no cell, including glial elements, has a chance of survival. The penumbra is the ischaemic zone surrounding the core and is defined as the zone where tissue may either die or survive in the long term. The consequent ischaemia impairs neuronal function, giving rise to neurological symptoms over and above those arising from neuronal death in the core. Finally, surrounding the penumbra is a zone of oligaemia, with blood flow greater than 20 mL/ min per 100 g but less than the normal value of about 50 mL/ min per 100 g, where oxygen extraction is increased, neuronal function is maintained and cell death in the long term is unlikely. In the acute stage the size of the core determines the extent to which there will be inevitable permanent damage, whereas the size of the penumbra indicates the potential for recovery and for thrombolysis to salvage brain tissue. Therefore identifying the extent of these zones, using neuroimaging, is clinically useful. Cells dying from ischaemia develop intracellular oedema as sodium ions enter the cell; water is thus removed from the extracellular compartment, resulting in reduced free diffusion of water. On the other hand, even as late as 3­6 hours after stroke onset, if there is a large area of mismatch (suggesting a large penumbra), then thrombolysis may improve outcome. There is uncertainty as to whether such microbleeds should be regarded as a contraindication to thrombolysis. Other neuroimaging techniques that may have a role in the investigation of stroke include cerebral angiography and Doppler studies of the cerebral vessels. This is best observed in ipsilateral cortex, particularly frontal, after thalamic infarcts, in contralateral cerebellum after large supratentorial infarcts (crossed cerebellar diaschisis) (Sobesky et al. With improvement in blood flow the penumbra shrinks as neurones that were previously unable to function return to activity. This may be followed by a period of improvement over several weeks due to the spontaneous regression of brain oedema and other acute histopathological processes in and around the stroke. Over this same period the remote effects of the stroke, particularly diaschisis, improve. Any later recovery is probably, at least in part, due to reorganisation of function (Butefisch et al. Finger movements on the unaffected side activated regional blood flow in the contralateral sensorimotor and premotor cortex and the ipsilateral cerebellar hemisphere. The same movements in the recovered hand produced more widespread activations, including significant increases bilaterally in sensorimotor and premotor cortex and both cerebellar hemispheres. Thus bilateral involvement of motor systems was seen when the recovered fingers were employed, indicating significant reorganisation and recruitment of ipsilateral motor pathways. Activations were also observed in cingulate and prefrontal areas that are not normally involved in finger movement but are known to be involved in selective attentional and intentional mechanisms, suggesting that these too may play an important part in the recovery process. This reorganisation was only seen in those who were exposed to a motor training programme, compared with those who were not, an observation that has important implications for stroke rehabilitation. Anticoagulant therapy may be indicated where embolism is suspected, or operative intervention may be needed on a stenosed or atheromatous carotid artery, but these are matters for neurological assessment. Patients with intracerebral haemorrhage are at risk of further bleeding and of developing hydrocephalus. Particularly in the younger patient or those with recurrent stroke, specific causes of stroke should be considered (Box 8. Sequelae of stroke the disablement resulting from strokes is frequently a mixture of physical and mental problems. In what follows an attempt is made to review the principal components of psychiatric disability after stroke, but first the Box 8. The general picture of the incidence of defects and quality of survival provides the framework against which to view the range and extent of the problems encountered. Overall prognosis On average, after a stroke about one-quarter of patients die within 1 month (Wolfe 2000). The wide variation in mortality rates across studies, from 18 to over 50%, presumably reflects the differing age and health status of the populations studied. The highest mortality is associated with primary intracerebral haemorrhage and subarachnoid haemorrhage; 1-year case fatality is about 34­40%. In terms of impairment it has been estimated that at 6 months after the stroke (Wolfe 2000) about half continue to have partial or complete motor loss, one-quarter are not orientated and about 15% have a significant aphasia. Presumably intervening vascular events and other effects of ageing explained some of the deterioration. Perhaps the high rates of depression also contributed to the deterioration; almost one-quarter were depressed. Early epileptic seizures, within the first week, are seen in about 3­6% of patients after ischaemic stroke (Camilo & Goldstein 2004; Ryvlin et al. Although only about 2­4% of patients will develop epilepsy in the longer term, stroke is the commonest single cause of late-onset epilepsy (Ryvlin et al. Those stroke patients who develop epilepsy probably have a worse outcome and may have greater risk of psychosis (see below). Early epileptic seizures may be a risk factor for cognitive impairment after stroke (Cordonnier et al. Cognitive impairment and dementia the mental impairment that may follow a single stroke usually proves to be focal in nature once the initial clouding of consciousness has cleared. For some time, however, global confusion and disorientation may be much in evidence, and can be slow to clear when cerebral damage has been extensive. The longer clouding of consciousness has persisted, the more likely that residual mental deficits are severe and extensive. Considerable difficulty may be encountered in assessing the extent of global intellectual impairment, particularly if the patient is dysphasic or with marked constructional difficulties. Agitation, depression or apathy in the early stages may give a false impression of dementia, as may visual disorientation or agnosic difficulties. A circumscribed amnesic syndrome due to posterior cerebral infarction may not at first be appreciated as such. Much of our understanding of the classic focal cortical syndromes (the dysphasias, apraxias and body image disturbances) has come from studies of stroke survivors. The essentials of such disorders have been outlined in Chapter 1 and are not repeated here. Disturbances of language contribute a large added handicap and source of frustration, frequently outlasting recovery of motor function. Patients with expressive loss but good comprehension will in general make much better adjustment than when understanding is faulty. Apraxic disturbances may persist as a barrier to rehabilitation when motor paralysis has cleared, particularly an apraxia of gait. Disorders of attention, including neglect, often improve spontaneously but otherwise may prove difficult to treat particularly as they are often accompanied by lack of insight. The concepts of vascular dementia and multi-infarct dementia are discussed alongside the other dementias in Chapter 9. The focus here is on poststroke dementia, a term introduced to describe those patients found to suffer global cognitive impairment after stroke (Leys et al. Thus the index event in studies of poststroke dementia is the stroke, and all types of dementia irrespective of their cause are included. In some cases the dementia will have been present before the stroke, in others the stroke will have caused the dementia, and in others the dementia will have had its onset at some time between the stroke and the assessment. Much of this variance may be because of the different classification criteria that have been used. The age of the cohort studied and the interval between assessment and stroke will affect prevalence rates. Risk factors for poststroke dementia include lower educational level, prestroke cognitive decline (but not sufficient for dementia diagnosis), and more severe stroke. Bilateral strokes involving the basal forebrain can produce a dementia as a result of severe impairment of executive function and memory. Indeed because the thalamus is supplied by four arteries, each with a very distinct origin and territory, thalamic strokes may be quite localised and offer unique insights into brain­behaviour relationships. It has been suggested that distinct patterns of behavioural change, even following unilateral lesions, can be seen depending on which of the four main thalamic arterial territories is affected (Carrera & Bogousslavsky 2006). She seemed well for a while thereafter, but gradually changed, becoming irritable, hard to please and with vague complaints of headache and giddiness. Loss of interests and slowing were accompanied by episodes of confusion and disorientation. On examination 15 months after the stroke she was not grossly demented, but showed some difficulty in understanding questions and had only a vague idea of the date. There was a residual left hemiparesis, a left homonymous hemianopia, some dyscalculia and a mild nominal dysphasia. However, her affective state was worse than her intellect: she was mostly apathetic and dull, though cheerful in a facile way at times. Poststroke dementia has a serious adverse effect on both the mortality (Desmond et al. Deficits in cognitive function are therefore among the more serious of the sequelae of stroke, delaying and often gravely compromising attempts at rehabilitation. Such elements in the clinical picture may be less immediately obvious than the hemiplegia or other physical handicap, yet often prove to be the factors responsible for failure to regain independence. Thus among patients who become long-stay invalids, permanently confined to chair or bed, paralysis by itself rather seldom accounts for their incapacity and may even contribute little towards it (Adams & Hurwitz 1963, 1974). Personality change Personality changes after stroke are among the most troublesome of the sequelae of stroke, and may overshadow the intellectual deficits. It may be difficult to determine if the change in personality is directly attributable to brain damage. When this is the case, widespread vascular changes are probably responsible and the personality change may progress even though the focal sequelae of the stroke improve.

Effective 25 mg tofranil. Think Fast Talk Smart: Communication Techniques.