These are all in distinction to Bassen-Kornzweig disease that is caused by an inherent defect in the lipid layer of the red cell mem brane (see further on). Evidently, the responsiveness of the autonomic nervous system in these patients remains heightened and a number of stimuli (cold, pain, muscular effort) may pro duce abnormal responses in pulse, respiration, oxygen consumption, and work performance. Myelin may also degenerate from axonal disease in a general process that may occur either proximal or distal to the site of axonal interruption. Although trawna may involve the spinal cord alone, the vertebral column is almost invariably injured at the same time. Inarnasu J, Hori S, Ohsuga F, et al: Selective paralysis of the upper extremities after odontoid fracture: Acute central cord syn drome or cruciate paralysis Jonesco-Sisesti N: Syringobulbia: A Contribution to the Pathophysiology of the Brainstem. Of course, paranoid delusions may be part of a depressive psychosis and of other dementias, but most of the elderly patients in whom paranoia is the presenting problem, seem not to be depressed, and their cognitive functions are for a time relatively well preserved. In patients with very large muscles, one must con sider not only myotonia congenita but also familial hyperdevelopment, hypothyroid myopathy, the Bruck-de Lange syndrome (congenital hypertrophy of muscles, mental retardation, and extrapyramidal movement dis order), Becker myotonia (see later), Duchenne dystrophy, and most of all, hypertrophic myopathy (hypertrophia musculorum vera); this last disease is of interest because the aberrant protein (myostatin) and gene defect have been characterized. Genetic Aspects of HereditanJ Spastic Paraplegia Numerous genetic mutations have given rise to this dis ease. The motor fibers of the vagus are derived from two nuclei in the medulla-the nucleus ambiguus and the dorsal motor nucleus. Other abnormalities may be discovered by observing the speed and efficiency of contraction and relaxation during one or a series of maximal actions of a group of muscles. They may become disinclined to participate in social activities and become withdrawn or quieter than usual. We have seen several patients with Sjogren disease in whom the trigeminal neuropathy and the associated antibodies or infl ammation of the minor salivary glands were evident well before the char acteristic sicca syndrome or other systemic manifestations of the disease. At the tum of the century, Koch introduced the term psychopathic inferiorih; implying that it was a constitutionally determined devia tion in personality. Characteristically, the angiomas cause partial syndromes and are followed by considerable recovery of function just as when they occur in the brain. Haloperidol may also help alleviate abnormalities of behavior or emo tional lability, but it does not alter the progress of the disease. A clonic or tonic contraction of one side of the face may be the sole manifestation of a cerebral cortical seizure. The finding of some degree of sensorimotor or reflex change cor responding only to the level of the spinal abnormalities is a point that always favors spondylotic myelopathy. Excessive sweating of the soles and dorsal aspects of the feet and of the volar surfaces of the hands and fingers is a common manifestation of alcohol-induced nutritional neuropathy. These individuals have fewer facial tics and orofacial features than those with neuro acanthocytosis. It is subject to modulation by different medications than those used for the alternating Parkinson tremor. In the Western world, a visual disorder indis tinguishable clinically and pathologically from that observed in prisoners of war is observed infrequently, mainly among undernourished alcoholics. How this relates to the entity of arteriosclerotic, multiinfarct, or vascular dementia is entirely clear. In such cases, the blink responses are delayed ipsilaterally and contralaterally as a result of conduction block in the proximal facial nerve. However, with complete destruction of the muscle fiber, this regen erative capacity is greatly impaired. Diaphragmatic paralysis occurs with lesions of the upper three cervical segments (transient arrest of breathing from brainstem paralysis is common in severe head injury). The frequency of these illnesses is greatly increased in the Several lines of study, including those employing functional imaging, indicate that a few select regions of the brain are implicated in the pathogenesis of the complex symptomatology of depression (Drevets). The cen tral processes of these two ganglia terminate in relation to the nucleus of the spinal trigeminal tract and the tractus solitarius, respectively. A number of mitochondrial disor ders have been associated with deafness alone as well as with a number of the better-characterized mitochondrial syndromes (see Chap. Very symmetrical findings, particularly tremor, suggest an alternative to idiopathic Parkinson disease. Trazodone has been helpful in treating depression and insomnia, the latter also being a major problem in some patients. Several pieces of contro versial evidence suggested that maternal depression may affect fetal growth and infant temperament.
When the depolarized zone moves under the recording electrode, it becomes relatively negative and the beam is deflected upward (at B). Diphtheritic polyneuropathy is typified by the demyelin ative character of the nerve fiber change, the location of this change in and around the roots and sensory ganglia, the subacute course, and the lack of inflammatory reac tion. The similarity between beriberi and alcoholic neuropathy was commented upon by several authors, but it was Shattuck, in the relationship of 1928, who first seriously discussed the 2 disorders. Bone age, head size, and height are normal and there are none of the coarse facial features of the myxedematous form. As with Huntington chorea (where the expanded poly glutamine tract is in the protein huntingtin), this disease is inherited as an autosomal dominant trait and shows an inverse correlation between the age of onset and the size of the gene expansion (anticipation). This form of immobilization may be sufficient to reduce discomfort in the neck and arms; only exceptionally in our experience, however, has arm and shoulder pain alone been sufficiently severe and persistent to require surgical decompression unless there is in addition a laterally protruded disc or osteophytic constriction of a root foramen. Males and females are equally affected; the pattern of inheritance is autosomal dominant in some and recessive or uncertain in others. Possibly the individuals with Lewy bodies would have developed Parkinson disease had they lived a few more years. The machine itself has as the essential element a large capacitor that is dis charged to produce an electrographic seizure. Creutzfeldt-Jakob subacute spongiform encephalopathy may cause difficulty in diagnosis initially but the course of illness clarifies the situation rapidly. Many of their seminal ideas, including those of Schneider discussed below, have been retained but oth ers have been discarded in various diagnostic criteria. Farcas P, Avnum L, Frisher S, et al: Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barre syn drome. Other important benzodiazepine drugs are lorazepam (Ativan), flurazepam (Dalmane), triazolam (Halcion), Immediately following withdrawal, the patient seem ingly improves over a period of 8 to 12 h, as the symp toms of intoxication diminish. The details of the process by which acetaldehyde is metabolized are still not settled. In every series of severely undernourished infants and young children who have been observed for a period of many years, a variable proportion has been developmentally delayed to a modest degree; the majority recovers, however (Galler). A predominantly bibrachial paralysis is an unusual presentation of neuropathic disease but may occur in the inflammatory-demyelinating polyneuropathies, as well as in Sjogren syndrome, chronic immune or paraneoplas tic neuropathies, lead neuropathy, Tangier disease, and in a familial type of brachial neuritis. If medication is successful in preventing progressive decompensation, the patient can many times return to the family and com munity. The diagnostic tests for plumbism in children are generally applicable to adults, with the exception of bone films, which are of no value in the latter. As Wilson stated, the relation of the choreic to the mental symptoms "abides by no general rule. Intermittent low-grade fever, substernal and abdominal pain (like that of peptic ulcer), melena, and hematemesis from bowel infarction may occur, the result of an accompanying systemic vasculitis. A proportion of young women with myasthenia have moderately elevated titers of antinuclear antibody without the clinical manifestations of systemic lupus. Although still valid, it was replaced by nerve conduction studies and by the needle electrode examination. Lesser degrees are characterized by a wide-based stance and a slow, uncertain, short-stepped gait; the mildest degrees are apparent only in tandem walking. When historical data are insufficient, it is often necessary to examine siblings and parents of the proband. A diagnostic problem arises in that orthostatic hypotension is also observed in up to 15 percent of patients with Parkinson disease, a feature that may be exaggerated by medications, but the degree of drop in blood pressure is far greater and more frequent in patients with this form of multiple system atrophy. The delayed pain, swelling, and tenderness that occur after sustained exer cise of unconditioned muscles are evidently a result of fiber necrosis (Armstrong). Furthermore, it is not certain that continued administration of anticonvul sants dependably prevents abstinence seizures. The mutilating effects are the result of repeated injury to analgesic parts and to a lack of auto nomic vascular reflexes. There are also cases on record in which an epidural or similar cath eter has accidentally penetrated the cord and caused a traumatic partial myelopathy as mentioned earlier.
Syndromes
These types of personality disorders and social maladjustments tum out not to be schizophrenia. Some observers have noted changes in the globus pallidus, subthalamic nucleus, red nucleus, cerebellum, and in the pars reticu lata of the substantia nigra. It is the lobar atrophy and marked changes in the underlying white matter that provide the unifying ele ments of this group of diseases. Some of the larger fibers have a target appear ance and may show degenerative changes. The diagnosis may be suspected if the systemic disease is apparent at the time but usually, only the histology of a surgical specimen reveals the underlying process. This change renders the sarcolemma susceptible to breaks and tears during muscle contraction, a hypothesis proposed first by Mokri and Engel and entirely consistent with the ultrastructural abnormalities that character ize Duchenne dystrophy. Freud viewed schizophrenia as a manifestation of a " weak ego" and an inability to control anxiety and instinctual forces-the result of a fixation of libido at an early ("nar cissistic") stage of psychosexual development. Such cases appear in the literature, and all are considered to have a humoral immune basis. Quite often one observes an acute or subacute form of multiple cranial neuropathy of undetermined cause. Ataxia Essentially the ataxia is one of stance and gait; in the acute stage of the disease it may be so severe that the patient canno t stand or walk without support. The physiologic defect is similar to the one that characterizes the myasthenic syndrome of Lambert-Eaton (see Chap. Undoubtedly, further studies of genes and gene products will continue to advance our understanding of the inherited neuropathies. In most cases, the encephalopathy is preceded for several days to a week by fever, symptoms of upper respiratory infection, and protracted vomiting. The ocular, facial, and bulbar muscles are involved, just as in native myasthenia. The muscles most likely to escape are those of the eyes, face, tongue, phar ynx, larynx, diaphragm, and sphincters, but on occasion even these may be involved. The most common is the "jaw winking" phenomenon (also called Wartenberg or inverse Marcus-Gunn sign), in which jaw movements, especially lateral movements (engaging the pterygoid muscle), cause an involuntary closure of the eyelid ipsilateral to the move ment. Pathologic studies in a limited number of cases have disclosed a relatively stereotyped pattern of reduced numbers of Betz cells in the frontal and prefrontal motor cortex, degeneration of the corticospinal tracts, and preservation of motor neurons in the spinal cord and brainstem (Beal and Richardson; Fisher; Pringle et al). There is weak ness and wasting of the small muscles of the hand and a characteristic clawhand deformity. Of more theoretical importance, the investigation of the acquired metabolic diseases provides new insights into the chemistry and pathology of the brain. All of these newer medications produce the "metabolic syndrome" of weight gain, adverse lipid changes, and glucose intolerance. Dexamethasone and acetazolamide prevent and counteract mountain sickness to some extent. In addition, there are reports of several sibships in which multiple members had a sensory ties are impaired (touch-pressure somewhat more than trunk (mal perforant du pied). Most of these disorders exhibit the characteristic and striking features of fluctu ating weakness and fatigability of muscle. Janet postulated a dissociative state of mind to account for certain features, such as trances and fugue states, a term that has reappeared in modem psychiatry. The most important aspect of treatment is the intravenous administration of large amounts of sodium bicarbonate to reverse acidosis. There are in addition examples of spinal cord compres sion that result from prolonged static hyperextension of the cervical spine during a protracted period of stupor. These include the polyneuropathy of critical illness (see further on in the chapter); an accel erated neuropathy of renal failure that is seen mainly in diabetic patients receiving peritoneal dialysis (both discussed further on); acute hypophosphatemia induced by hyperalimentation; polymyopathy produced by the administration of high-dose corticosteroids; and the pro longed effects of neuromuscular blocking drugs, result ing in the accumulation of their metabolites in patients under conditions of renal failure and acidosis. There may be telangiectasias over the bridge of the nose, on the lips, and fingers.
The parkinsonian features can respond favorably to L-dopa, but only for a limited time and sometimes at the expense of causing an agitated delirium or hallucinations that would be uncharacteristic of early Parkinson disease (Hely et al); in others, the response to L-dopa is inconsis tent or inapparent. A series of animal experiments that demonstrated the possibility of removal of plaques by immunization against amyloid has led to human studies with a similar vaccination. Among patients with primary depres sive disorders, life events of a stressful nature were found to have occurred more frequently in the months preceding the onset of depression than in matched con trol groups. These are the typ ical changes in idiopathic polymyositis (in combina tion with infiltrates of inflammatory cells), and infec tive polymyositis. The symptomatic course of this illness is quite vari able but usually extends over a period of 5 or more years, but judging from pathology studies, the pathologic course has a much longer asymptomatic duration. In both alike we are confronted with the same discrepancy between fact and statement, objective sign and subjective symp tom-the outward aspect of health seemingly giving the lie to all the alleged functional disabilities. Ultimately, the patient loses the ability to stand and walk, being forced to lie inert in bed and having to be fed and bathed, the legs curled into a fixed posture of paraplegia in flexion (in essence, a persistent vegetative state). Cramps of all types need to be distinguished from sen sations of cramp without muscle spasm. Most reports attribute this syndrome to infil In yet other instances, the neuropa tration of neural by malignant plasma cells rather than to hyperviscosity. The use of hypothermia with cooling blankets or the infusion of cooled saline is under investigation to protect spinal tissue but has not been validated. Any intercurrent illness may result in a further mobilization of lead from bones and soft tissues and an exacerbation of symptoms of lead intoxication. The the spinal cord, brain, optic nerves, and peripheral spinal cord is usually affected first and often exclusively. The electrical activity of various muscles is recorded both at rest and during active contraction by the patient. Diagnostic criteria have been offered by a working group, requiring 2 of 3 of the following: a parkinsonian syndrome (usually symmetric), fluctuations in behavior and cognition, and recurrent hallucinations (McKeith et al). An incomplete and revers ible myelopathy is referable to the site and level of the injury. Insight into the disease has been gained from a breed of pigs, inbred for muscular development, in which mus cle spasm (true contracture) and hyperthermia follow the administration of anesthetic agents. Some of these patients quickly pass through this acute post-hypoxic phase and proceed to make a full recov ery; others are left with varying degrees of permanent disability. Following nerve stimulation, an action potential is transmitted by the sarcolemma from the motor endplate region to both ends of the muscle fiber. The symptoms usually develop suddenly, often after an altercation or other emotionally charged event. A small, almost insignificant proportion of addicts are introduced to drugs by physicians in the course of an illne ss. The ulnar and median nerves were involved in their patients and there was motor conduction block and sensory slowing in affected nerves. According to Mesulam (2003), who has studied the condition extensively, 60 percent of these cases show no characteristic pathologic change, 20 percent have Pick bodies, and a similar proportion show the typical changes of Alzheimer disease in the affected cortical region. A syndrome of persistent fatigue, lack of stamina, inability to concentrate, poor memory, and irritability has also been attributed to chronic exposure to solvents, but these symptoms are quite nonspecific, and evidence for such a syndrome is unsupported by convincing experimental or epidemiologic studies. The mechanism is uncertain but there is, so far, no evidence of direct spirochetal infection of the nerve. A small number of patients respond so well to plasma exchange and find the side effects of steroids so intoler able that they choose to be maintained with two to three exchanges every several weeks or months. These and other concepts of the treatment of anxiety are discussed by Goodwin and Guze. Reduction in motor and sensory amplitudes is a more specific and sensitive indicator of axonal loss than is slowing of conduction velocity or prolongation of distal latencies. There may be enhancement of the margins of the purulent collection after several days. The therapeutic value of this measure has since been questioned after reanalysis of the data (Nesathurai; Hurlbert) and it is no longer considered essential.
More often than not, the delusional hallucinatory content is persecutory, but it may also be religious, depressive, grandiose, or bizarrely hypochondriacal in nature. There were complications of this operative procedure, and the results were no better than those obtained from simple decompression. This procedure has reportedly caused a localized rhabdomyolysis (Meinck et al), for which reason Griggs and associates recommend that the test be carried out without a blood pressure cuff. Then a new group of symptoms develops, consisting of nervousness, tremor, insomnia, postural hypotension, and weakness. These changes always begin and are most severe in the geomet ric center of the pons, where they may proceed to frank necrosis of tissue. Sympathomimetic effects-pupillary dilatation, piloerection, hyperthermia, and tachycardia are prominent, and the user may also show hyperreflexia, incoordination of the limbs, and ataxia. The symptoms then become augmented, reaching their peak intensity 24 to 36 h after the complete cessation of drinking. Nerve conduction velocities and distal motor latencies are normal unless there is a coincident polyneuropathy. The ones in clinical use for the treatment of myasthenia gravis are the carbamates neostigmine, physostigmine, and pyridostigmine, the effects of which are reversible. Approximately 30 percent of patients have no family history of the disease and these represent spontaneous mutations. In this way a new pharmacogenically induced need is developed, and the use of opioids becomes self-perpetuating. Since 1903, when Morax and Marie proposed their theory of centripetal migration of the tetanus toxin, it has been taught that spread to the nervous system occurs via the peripheral nerves, the toxin ascending in the axis cylinders or the perineural sheaths. It usually appears over a period of days to weeks and may terminate fatally; or, with appropriate treatment, the symptoms may regress and then fluctuate in severity for several weeks or months. Some reports have included a parkinsonian syndrome and more consistently, a mild frontal dementia, making the distinction from frontotemporal dementia difficult. Ciliary body paralysis with loss of accommodation and blurring of vision but with preserved light reaction usually appears in the second or third week (the opposite of the Argyll Robertson reaction). The symptoms are malaise, fatigue, nervousness, headache and tremor, which may be difficult to distinguish from anxious depression. Here we mention mainly that penicillin and its derivatives such as imipenem, and to a lesser degree, the cephalosporins, are capable of causing seizures when high serum concentrations are attained. If the histologic examination is performed early in the course of the neuropathy, sparse infiltrates of lymphocytes are observed distributed in foci around blood vessels. Testicular biopsy shows atrophy and hyalinization of tubular cells and hyperplasia of Leydig cells. To overcome this, Schneider proposed that the distinction between primary and accessory manifestations be abandoned. Haloperidol (Haldol), or one of the newer anti psychosis agents discussed in Chap. Local differences in the natural concentration of vitamin E in various parts of the nervous system and musculature are believed to account for the distribution of the lesions. Depolarization spreads quickly to the interior of the fiber along the walls of the transverse tubules, probably by a conducted action potential. The pathologic changes that underlie the syndrome have been studied in a small number of cases and no uniform change has emerged. The motor axons produce fasciculation potentials, myokymic discharges, neuromyotonia, and cramp syndromes; and the central nervous system is the source of complex ensembles of continuous motor activity such as occur in the stiff man syndrome. Alertness is not impaired, but the ability to maintain attention, as measured by continuous performance tasks, is reduced (Seidman). Multiple mononeuropathies have also been our experience has been a progressive or relapsing dis ease that cannot be distinguished clinically from chronic reported, as has involvement of the autonomic nervous some instances suggests nerve root involvement. Thyrotoxicosis with periodic paralysis (5 percent of myasthenic patients; see further on and Chap.
The optic nerve lesions appear first in the papillomacular bundles, in the retrobulbar portions of the nerves; it sub sequently spreads beyond the confines of this bundle and caudally in the optic nerves, chiasm, and tracts. Abrupt worsening, even paraplegia or quadriple gia, may follow forceful traumatic flexion or extension injuries of the neck, as indicated later. Leprous neuritis is the one infectious member of this group and also the one exception to the rule that all chronic neuropathies are more or less sym metrical in pattern. The pattern of inheritance is most often autosomal dominant, but autosomal recessive and X-linked patterns also have been described. During and after the procedure, hypotension, hypoprothrombinemia with bleeding and cardiac arrhythmias may occur. Goldflam (in 1895) first called atten tion to the remarkable vacuolization of the muscle fibers that is characteristic of the process. In severe forms of the disease, many months may pass before the patient is able to walk unaided. In approximately 15 percent of cases, however, there appears to be no associated illness (p ri mary hypochondriasis). Scattered throughout the nervous system of their case were unusual glycogen-containing bodies, similar but not identical to corpora amylacea. In the common early adult form of the disease, the small muscles of the hands along with the extensor muscles of the forearms are often the first to become atro phied. Or, following a prolonged drinking binge, the patient may have experienced several days of tremulousness and hallucinosis or one or more seizures and may even be recovering from these symptoms when delirium tremens develops, rather abruptly as a rule. Neural complications are infrequent and usually take the form of somnolence, confusion, agita tion, and depression. The familial cases do not diffe r fundamentally in their symptoms and clinical course from nonfamilial ones, although as a group the former have an earlier age of onset, an equal distribution in men and women, and a slightly shorter survival. Multiinfarct dementia is usually not difficult to separate from Alzheimer demen tia, as discussed further on. Hysteria may include anxiety symptoms, though they are seldom prominent, and phobic and obsessive-compulsive neuro ses constantly create an anxious state in affected patients, but each has distinguishing attributes. Presumably axis cyl inders and myelin degenerate together, as would be expected from the loss of nerve cells in the superficial layer of the retina. Despite ataxia and complete areflexia, muscular power remained normal and pain was not a problem. Testing for jitter is carried out by having the patient voluntarily contract a muscle to the slightest degree possible so as to activate only one motor unit (requiring a great deal of cooperation by the patient) or by stimulating until two muscle fibers from the same motor unit are recorded. Kraepelin remarked on the changeable, fantastic, and bizarre character of the delusions. Others have emphasized a deficiency of aldose reductase and an elevation of polyols (particularly sorbitol) as being causally important. The origin of these discharges is probably in the distal peripheral nerve, where activity of afferent fibers, possibly via ephaptic transmission, irregularly excites distal motor terminals. A small proportion of cases are bilateral at the outset, or the opposite side is affected weeks later but most cases remain one-sided. Extended-action forms of both drugs are available but are given at bedtime mainly to patients who complain of weakness during the night or early morning hours. If abruptly discontinued, the patient may become totally immobilized by a sudden and severe increase of tremor and rigidity; rarely, a neuroleptic syndrome, sometimes fatal, has been induced by such withdrawal. Extremely high levels cause impairment of consciousness with asterixis, myoclonus, seizures, and choreiform movements. Lower plexus lesions weaken the posterior thigh, leg, and foot muscles and abolish sensation over the first and second sacral segments (sometimes the lower sacral segments also). Irrespective of its mode of origin, the syrinx first occupies the central gray matter of the cervical portion of the spinal cord, usually independent of the central canal but sometimes extending into it. This state is of special importance because it complicates neurologic diseases of my types: head trauma, bacterial meningitis and encephalitiS, cerebral infarction, subarachnoid hemorrhage, cerebral and systemic neoplasm, Guillain-Barre syndrome and the effects of certain medications.
Rhamnus zizyphus (Jujube). Super Avana.
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Implementing and sustaining hypothermia, either by external cooling, infusion of cooled normal saline, or intravenous cooling devices is difficult, and the iatrogenic problems of hypotension, bleeding, ventricular ectopy and infection have some times arisen, although this mild degree of temperature reduction is usually well tolerated. We have seen other types of cutaneous diseases with vasculitic mononeuritis, the most impressive being a massive leukocytoclastic vas culitis of the skin (necrotic polymorphonuclear cells surrounding venules) resulting in large confluent hem orrhagic lesions. Moreover, the few postmortem studies of myelopathy as a consequence of electrical injury have disclosed a widespread demyelination of long tracts, to the point of tissue necrosis in some segments, and relative sparing of the gray matter, but no abnormalities of the blood vessels. From the observations of Brun and Passant and of Neary and associates, pure tau-reactive cases outnumber Pick dis ease when the latter is strictly defined by the cortical white matter degeneration and Pick inclusions. In older patients, a stellate cataract slowly forms in the posterior cortex of the lens. Moreover, this dietary regimen may permit the patient to reduce slightly the total daily dose of L-dopa. The attack terminates with deep sleep, which occurs spontaneously or in response to parenteral sedation; on awakening, the patient has no memory of the episode. This is a complex area that at one time, considered the 2 processes to be intimately related and later, was rejected, only to now be resurrected with clearer focus, as discussed Chap 34. As oro pharyngeal palsy progresses, food should be cut into small pieces and dry foods, such as toast should be avoided; milk shakes and preparations of the same consis tency are ideal at this stage. One group seems not to mind and to be relieved by the expression of concern and reassurance that there is no dangerous disease at the root and may do well in the short run. Relaxation occurs as a result of active (energy-dependent) Ca reuptake by the sarcoplasmic reticulum. These are probably secondary effects of immobilization, as pointed out long ago by Lewis and Pickering. Although highly speculative, perhaps some of these changes explain the delay in improvement after the administration of antidepression drugs. Breathlessness, vague chest or upper abdomi nal discomfort, a palpitating sensation as if the heart were beating too hard, and a generalized "washed out" feeling (asthenia) are other common symptoms. However, low levels of this enzyme are not specific, occurring in such unrelated disorders as hypokalemic periodic paralysis and spinal muscular atrophy (see Layzer for details). Characteristically in these patients, an extensive portal-systemic collateral circula tion can be demonstrated (hence the term portal-systemic encephalopathy) and an association established between the as 37) In all these states, it is common for an excess of protein derived from the diet or from gastrointestinal hemorrhage to induce or worsen the encephalopathy. Some of the recent findings using functional imaging in hysterical states are reviewed by Carson and colleagues. Sometimes, the electrophysiologic and biopsy tests may have to be repeated after several weeks or more to give a definitive diagnosis. On occasion, weakness of facial muscles may be combined with myasthenic weakness of the masseters and other bulbar muscles without involvement of ocular muscles. Brandt, in his study of 112 Danish patients, found that in about one-third the weakness was present at birth, and in 97 the onset was in the first year of life; in 9 patients, the disease was not recognized until after the first year of life. The number of fibers composing a motor unit may also be of significance; in the ocular muscles, a motor unit contains only 6 to 10 muscle fibers (some even fewer), but a motor unit of the gastrocnemius contains as many as 1,800 fibers. Suicide is the eighth leading cause of death among adults in the United States and the second leading cause among persons between the ages of 15 and 24 years, figures that emphasize the importance of recognizing depression that has a high potential for self-destruction. In many of the fatal cases there is an associated infectious illness or injury, but in others, no complicating illness is discernible. It is hoped that the genetic mutations that give rise to Parkinson disease will expose the molecular pathophysi ology of the disease. Visser and colleagues reported similar findings in a series of acute motor polyneuropathies. After 7 to 10 days or more, as the process of wallerian degeneration pro ceeds, sensory potentials are progressively lost and the amplitudes of compound muscle action potentials are variably reduced. In the sense that gaps in memory exist and that whatever the patient supplies in place of the correct answers fills these gaps, the statement is incontrovertible. The affected parts may ache and feel cold, but true paresthesias, except from poor positioning and pressure on nerves, do not occur or are minor. There are several other less common chronic myopa thies in which external ophthalmoplegia is associated with involvement of other muscles or organs, namely, the congenital ophthalmoplegia of the Goldenhar-Gorlin syndrome (see Aleksic et al); the Keams-Sayre syn drome (retinitis pigmentosa, heart block, short stature, generalized weakness, and ovarian hypoplasia); other congenital myotubular and mitochondrial myopathies; and nuclear ophthalmoplegia with bifacial weakness (Mobius syndrome). It will be interesting to learn how such a deficiency might impair brain development and whether there is any connection to some cases of mental deficiency without muscular dystrophy. The nature of this disorder has been a matter of controversy, some writers claiming it to be a progressive muscular dystrophy and others, a muscular atrophy of spinal or neuropathic type. Perhaps sprue is able to cause a neuropathy with minimal gastrointestinal symptoms.
Presumably, the immunocompromised patient is unable to respond to protozoan infections, allowing latent infec tions to be reactivated. Atrophy proceeds slowly over several weeks and months, the degree being proportional to the number of damaged motor nerve fibers. Thymectomy is best performed in a hospital where there is close collaboration between the thoracic surgeon and the neurologist. Electrophysiologic test ing confirms the diagnosis by demonstrating prolonged sensory conduction across the wrist and explains cases in which operation has failed (see the review by Stevens). There may be diagnostic difficulty in the case of an acute lesion of the cord in which tendon reflexes are initially lost (spinal shock), or with necrotizing myelopathy, where a permanent loss of tendon reflexes follows extensive destruction of spinal gray matter. Microscopically, the crystalline material (probably lipids and cholesterol, which cause the iridescence) lies in vacuoles and lacunae among the lens fibers. It consists of attacks of mild to severe weakness of the muscles of the trunk and limbs; usually the cranial muscles are spared. Plott D: Congenital laryngeal-abductor paralysis due to nucleus ambiguus dysgenesis in three brothers. There is also a group of patients who have idiopathic leg pain during rest after activity. Some patients complain of numbness or paresthesias, most often in one or two digits, a part of the palm, or a longitudinal band along the forearm. Fracture of the temporal bone (usu ally with damage to the middle or internal ear), otitis media, and middle ear surgery are uncommon causes. Whether a peripheral neuropathy is a pri mary component of the disease or is secondary to dam age of the posterior root fibers of entry in the dorsal cord has been debated, but the available pathologic evidence favors the latter, except perhaps for a few advanced cases, in which other nutritional deficiencies could have been responsible. Additional insight into obsessive-compulsive dis order may be obtained from the many cases in which acquired striatal damage may be linked to obsessional behavior. The relation between the increased phytanic acid and the polyneuropathy is uncertain. Early Machado-Joseph disease characteristi cally demonstrates the finding of dysmetric horizontal and vertical saccades, even before the ataxia is obvious (Hotson et al). In our clinical material, symptoms of dementia or psychosis have not been frequent and always followed the spinal cord disorder. The exophthalmos varies in degree, sometimes being absent at an early stage of the disease, and it is not in itself respon sible for the muscle weakness. Inflammatory myopathy coexists with numerous systemic diseases as discussed, and some authors con sider it to be a syndrome rather than a disease. Although the nerves may not be palpably enlarged, "hypertrophic" changes with onion-bulb formation are invariable pathologic features. The adverse effects of platinum are discussed later, with the antineoplastic agents. However, we have encountered cases of asym metric and profound atrophic weakness of the arms that began almost two decades after the shock and progressed over many years without long tract signs, both with a previously presumed diagnosis of amyotrophic lateral sclerosis. Although population studies allow only limited conclusions about clinical correlations, the glucocerebrosidase mutation is present more often in patients with a family history of the disease, have an earlier onset that in patients with a normal gene and a lower incidence of resting tremor. This gives rise to osteomyelitis with extension of the purulent process to the epidural space. Little is known about the pathologic basis of the muscular pain in these diseases; it is not a result of muscle inflammation and is probably produced by circu lating cytokines that are common to most systemic infec tions. After fusion of the myoblasts, a series of cellular events including the sequential activation of myogenic transcription factors leads to myofibril formation. Sensorimotor polyneuropathy, polyradiculoneuropathy; autonomic neuropathy, or mononeuropathy (most often of the trigeminal nerve, as described by Kaltrieder and Talal) are less common. Unlike typical delirium tremens, the atypical states usually present as a single circumscribed episode without recurrences, are only rarely preceded by seizures, and do not end fatally. We comment here that the Chvostek sign is found in some normal individuals, without obvious explanation.
Treatment is usually ineffec tive unless radiation therapy is begun before paraplegia supervenes (Winkelman et al). Nielsen and Jannetta have shown that ephaptic transmission disappears after the nerve is decompressed. Other patients receiving high-dose ara-C have developed a mild, reversible cerebellar syn drome with the same clinical features. Sophisticated psychometric testing has disclosed abnormalities not so much in intelligence and memory (which are slightly reduced in 20 to 30 percent of cases) as in other psychologic functions. This theory and others have been largely discounted; they are the subject of reviews by Butterworth and coworkers, by Zieve, by Rothstein and Herlong, and by Jones and Basile, to which the reader is referred for detailed information. The differential diagnosis of this imaging appearance is from orbital pseudotumor, a usually painful condition, which is discussed in Chap. The importance of the alcohol-induced deficiency diseases relates to the fact that they are preventable and, if neglected, may lead to permanent disability. The disorder tended to occur in outbreaks (286 cases were reported to the Centers for Disease Control during a 4-month period in 1974). In the nutritionally deficient patient, Korsakoff amne sia is usually associated with and immediately follows the occurrence of Wernicke disease. He called it dementia praecox (adopting the term introduced earlier by Morel) to refer to a deterioration of mental function at an early age, from a previous level of nor malcy. More often, there is no associated obstruc tion at the upper cord and no hydrocephalus for which reason it is our impression that most cases are benign and relatively nonprogressive. As these conditions were more carefully docu mented in the early part of the last century, they came to be called psychoneuroses, and later, neuroses, and those that created societal difficulties were called psychopathies, and more recently, personality disorders and sociopathies. Approximately Most of the syndromes listed here are likely to be a result of ischemia or infarction of nerves or nerve fas cicles, because of a diabetic microvasculopathy. The extent of recovery is roughly proportional to the acuteness and severity of the disease and the duration of symptoms prior to institution of therapy. Bicrural Palsy Presenting as Lower Leg Weakness With Inability to Walk on the Heels and Toes, or as Paralysis of All Leg Muscles With the exception of certain distinctive distal types of muscular dystrophies, this pat 26 and 46 in relation to its most dramatic tern, usually due to weakness of peroneal, anterior tibial, and thigh muscles, is usually not a result of myopathy. Less often, a localized granuloma gives rise to a cord syndrome and, rarely, the disease takes the form of an acute transverse myelitis with massive necrosis of cord tissue (Queiroz et al). Exaggerated responses to the usual dose of meperidine (Demerol) and other narcotic drugs have also been observed sporadically; in these cases, respiratory function may be depressed to a serious degree, and hyperpyrexia, agitation, and pronounced hypotension may occur as well, sometimes with fatal issue. Trauma from sudden extreme extension, as in a fall, severe whiplash injury, or chiropractic manipulation, or from a lesser degree of retraction of the head during myelography, tooth extraction, or a tonsillectomy may be operative in individual cases, particularly in patients with congenitally narrow canals. In fatal cases there were zones of fiber loss, with fibrosis in skeletal and cardiac muscles, and a greater than normal variation in fiber size. Certain early or mild forms of dystrophy may selectively involve only the peroneal and scapular muscles. The goiter usually requires no treatment but it is possible to administer thyroid hormone so as to cause the thyroid enlargement to regress. In many of these patients the response to analgesic drugs has been unusual or excessive, and some of them are addicted. Death from poisoning is because of respiratory depression and hypotension; patients who survive may show signs of liver and kidney disease. It also provides the sensory innervation of the dura in the anterior and middle cranial fossae. In the case reported by Newcomer and associates, a rapid reversal of thyrotoxic coma (and corticospinal signs) was effected by plasma exchange, in parallel with a reduction in T4 and T3 levels, and similar results were reported by Boers and Colebatch. In thyrotoxic and steroid myopathies, the major effects are on the quadriceps muscles. The patient may suspect his elderly wife of having an illicit relationship or his children of stealing his pos sessions. On leaving the skull, the ninth, tenth, and eleventh nerves lie adjacent to the cervical internal carotid artery, where they can be damaged (presumably made ischemic) by a dissection of that vessel. In the least severe form, the onset may be in late infancy (with episodic meta bolic disturbances) or in childhood or adult life (with a lipid storage myopathy and a deficiency of serum and muscle carnitine). Sagittal (left) and axial (right) T1 gadolinium enhanced images show the peripherally enhancing pyogenic collection (arrows) wruch extends over several vertebral segments. Note the long extent of the lesion and thinning of the cord as the acute illness subsides. The astrocytoma is the most common intra medullary tumor if one excludes tumors arising in the filum terminate.
These myotonic discharges wax and wane in amplitude and frequency, producing a "dive-bomber" sound on the audio monitor. After a period of several days there is a rapid development of muscular weakness and thereafter, sensory and reflex impairment. Interestingly, the multiple skeletal and developmental abnormalities that characterize both pseudohypoparathyroidism (a failure of sensitivity to the hormone) and pseudopseudohypo parathyroidism (short stature, round face, short neck, stocky body build, shortening of metacarpal and meta tarsal bones and phalanges from premature epiphyseal closure) are rarely seen in pure hypoparathyroidism. A deficiency of vitamin B12 is not causative, but may coexist in some cases, probably on the basis of a shared inadequacy of dietary intake. Progressive supranuclear palsy (discussed in a sec tion further on) is characterized by rigidity and dystonic postures of the neck and shoulders, a staring and immobile countenance, and a tendency to topple when walking-all of which are vaguely suggestive of Parkinson disease. However, a fairly severe impairment of ventilation may occur before the first sign of dyspnea appears and before there is elevation of arterial carbon dioxide content. Severe exophthalmos, marked by periorbital and conjunctival edema, and the extraocular muscle weakness may be partially controlled by high doses of corticosteroids (about 80 mg/ d predni sone). Autonomic failure was another feature in a few and one of our patients became deaf. Examination discloses a reduction in visual acuity because of the presence of central or centrocecal scotomata, which are larger for colored than for white test objects. During the 1960s, more than 10,000 cases of this disease were collected in Japan by Tsubaki and colleagues. Among 5,362 infants who were followed prospectively since their birth in 1946 by Jones and col leagues, the 30 individuals who later developed schizo phrenia had been delayed in the attainment of motor milestones and speech and exhibited greater social with drawal and schoolroom anxiety as well as lower scho lastic achievement. Perhexiline maleate for the treatment of angina pectoris may also cause a generalized, predomi nantly sensory polyneuropathy in a small proportion of patients. Certain of the newer antipsychotic drugs, discussed in the next chapter, have overtaken lithium for the treatment of bipolar disease, with little evidence that they are superior. For example, sustained upgaze for 30 or may uncover myasthenic ocular motor weakness. Each myelin segment and Schwann cell has a symbiotic relationship to the axon but is morphologically inde pendent. One example of a biologic and genetic basis of a human personality trait, albeit to a limited degree, has been found in the expression of thrill seeking, exploration, and excitability. Gaist D, Jeppesen U, Andersen M, et al: Statins and risk of poly neuropathy: A case control study: Neurologtj 58:1333, 2002. Also, in recent years, effective forms of treatment for several peripheral neuropathies have been introduced, making accurate diagnosis imperative. The term is misleading in that in most cases the amyloid is derived from a circulating para protein, but the proportion of "benign" and malignant plasma cell sources of the protein varies from one report to another. The early symptoms of childhood clumsiness and athletic imprecision are listed previously, to which are added foot deformities of high arches and hammer toes. Cortical nerve cells degenerate and are replaced by microglia cells and astrocytes. These patients are, of course, younger and have had a shorter duration of disease. Organic compounds of combinations of headache, restlessness, drowsiness, confusion, delirium, coma, and convulsions, which, as a rule, occur late in the illness or preterminally. Additional myo toxic agents that can be expected to appear as new drugs are introduced. This may be indicative of a tumor (cholesteatoma), an aneurysmal dilatation of the basilar artery or one of its branches, or an arteriovenous malformation that compresses both the trigeminal and facial nerves. Similarly, contractures occur in the hip flexors because of the relatively greater weakness of hip extensors and abdominal muscles. In identifying a process as caused by single or mul tiple mononeuropathies, the reader can refer to Table 46-1, which lists the roots, nerves, and muscles that are involved in particular movements, and to Table 46-4, which gives the main etiologies of mononeuropathy multiplex. The corticospinal tract degeneration is most evident in the lower parts of the spinal cord, but it can be traced up through the brainstem to the posterior limb of the internal capsule and corona radiata by means of fat stains, which show the macrophages that accumulate in response to chronic myelin degeneration.
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