Showing papers in "Neurology in 1984"
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TL;DR: The criteria proposed are intended to serve as a guide for the diagnosis of probable, possible, and definite Alzheimer's disease; these criteria will be revised as more definitive information becomes available.
Abstract: Clinical criteria for the diagnosis of Alzheimer's disease include insidious onset and progressive impairment of memory and other cognitive functions. There are no motor, sensory, or coordination deficits early in the disease. The diagnosis cannot be determined by laboratory tests. These tests are important primarily in identifying other possible causes of dementia that must be excluded before the diagnosis of Alzheimer's disease may be made with confidence. Neuropsychological tests provide confirmatory evidence of the diagnosis of dementia and help to assess the course and response to therapy. The criteria proposed are intended to serve as a guide for the diagnosis of probable, possible, and definite Alzheimer's disease; these criteria will be revised as more definitive information become available.
26,847 citations
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TL;DR: Your contributions to the investigation of this tragic disorder are presented and a review of current ideas about the disease is reviewed.
Abstract: Huntington9s disease (HD) is an autosomal dominant disorder of midlife onset, characterized by progressive involuntary choreiform movements, psychologic change, and dementia. In 1980, funded by the NINCDS, we established a center for the study of HD. This report presents a summary of our contributions to the investigation of this tragic disorder and a review of current ideas about the disease.
1,400 citations
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TL;DR: There was no significant difference in incidence of the major clinical manifestations between the two groups with and without myeloma, and they had a common characteristic histologic finding of the lymph node resembling that of Castleman's disease.
Abstract: Clinical manifestations of 102 cases with the Crow- Fukase syndrome (the syndrome of polyneuropathy, anasarca, skin changes, endocrinopathy, dysglobulinemia, and organomegaly), with or without myeloma, were reviewed. Fifty-six cases with myeloma consisted of 31 with osteosclerotic, 17 with mixed osteosclerotic and osteolytic, and 8 with osteolytic. Forty-six cases without myeloma consisted of 2 with extramedullary plasmacytoma, 33 with M protein alone, and 11 with polyclonal protein alone. There was no significant difference in incidence of the major clinical manifestations between the two groups with and without myeloma. They had a common characteristic histologic finding of the lymph node resembling that of Castleman's disease.
500 citations
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TL;DR: When the cholinergic cell count in the basal forebrain drops below about 100,000 cells, the level of cortical ChAT may be so low that clinical dementia appears, and giant neurons of the medial basal fore brain stained for choline acetyltransferase (ChAT) are stained.
Abstract: All giant neurons of the medial basal forebrain stained for choline acetyltransferase (ChAT). Cell numbers declined from 400,000 to 475,000 in young controls to approximately 140,000 in elderly controls. Five senile dementia cases had counts ranging from 45,000 to 100,000 cells. ChAT levels in control frontal cortex decreased from 1.2 μmol/hr/100 mg protein at age 40 to 0.5 at age 95. Five senile dementia cases had levels ranging from 0.04 to 0.30. When the cholinergic cell count in the basal forebrain drops below about 100,000 cells, the level of cortical ChAT may be so low that clinical dementia appears.
467 citations
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TL;DR: 43 patients, aged 15 to 69 years, whose convulsive seizures were uncontrolled because the syndrome of juvenile myoclonic epilepsy was not recognized, had diffuse 3 1/2- to 6-Hz multispike-wave complexes and valproic acid stopped convulsions.
Abstract: We studied 43 patients, aged 15 to 69 years, whose convulsive seizures were uncontrolled because the syndrome of juvenile myoclonic epilepsy was not recognized. Awakening myoclonic jerks appeared with tonic-clonic (18 patients), clonic-tonic-clonic (24 patients), and absence seizures (17 patients), with a mean age at onset of 13.6 years. Generalized seizures were present in relatives of 17 patients. All patients had diffuse 3 1/2- to 6-Hz multispike-wave complexes. Valproic acid stopped convulsions in 86% of patients. After being free of seizures for 2 years, withdrawal of valproic acid was followed by relapse of convulsions in 12 patients.
393 citations
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TL;DR: No uniformly accepted hypothesis explains the genesis and rupture of intracranial aneurysms, but smoking, particularly heavy smoking, was also more frequent among cases.
Abstract: No uniformly accepted hypothesis explains the genesis and rupture of intracranial aneurysms. We followed 5,184 men and women prospectively for 26 years; 36 cases of aneurysmal subarachnoid hemorrhage (SAH) accounted for 62% of all intracranial hemorrhages. Blood pressure before SAH was higher in these patients than in controls. Definite hypertension (greater than or equal to 160 mm Hg and/or greater than or equal to 95 mm Hg) at entry to the study or at closest exam before SAH was more frequent than in controls. Cigarette smoking, particularly heavy smoking, was also more frequent among cases.
381 citations
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TL;DR: The data suggest that oxygen-derived free radicals damage endothelial cells of the blood-brain barrier; the brain injury is characterized by edema and by structural damage of neurons and giia.
Abstract: We studied the cerebral effects of oxygen-derived free radicals generated from the xanthine oxidase/hypoxanthine/ADP-Fe3+ system. Xanthine oxidase/hypoxanthine/ADP-Fe3+ solution (0.1 ml) was infused into caudate putamen, and brain was frozen rapidly in situ. Brain water and sodium content increased concomitant with decreased potassium content at 24 hours and 48 hours after the infusion. The degree of brain edema and injury depended on the dose of xanthine oxidase. Spongy neuropil and neuronal cytoplasmic vacuoles were seen at 2 hours, with an infiltration by polymorphonuclear leukocytes at 24 hours, followed by lipid-laden macrophages and reactive astrocytes. Leakage of fluorescent dye into neuropil was seen at 2 hours, but not later. These data suggest that oxygen-derived free radicals damage endothelial cells of the blood-brain barrier; the brain injury is characterized by edema and by structural damage of neurons and glia.
378 citations
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TL;DR: Signs and symptoms of a predominantly sensory peripheral neuropathy developed in 92% of the patients given cisplatin plus adriamycin by monthly intravenous injections, and sural nerve biopsies showed loss of large-diameter nerve fibers, with axonal and myelin degeneration.
Abstract: Patients with advanced cancer, previously untreated, were given 60 mg/m2 cisplatin plus 60 mg/m2 adriamycin by monthly intravenous injections. Signs and symptoms of a predominantly sensory peripheral neuropathy developed in 92% of the patients. Patients complained of dysesthesias and paresthesias in hands and feet. Clinically, there was progressive decrease or loss of tendon reflexes, decreased vibratory sense, and mild decrease in light touch and pin sensation. Distal sensory latencies became prolonged or dropped out completely, but there was little change in motor nerve conduction velocities or motor unit action potentials. Sural nerve biopsies showed loss of large-diameter nerve fibers, with axonal and myelin degeneration.
359 citations
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TL;DR: It was found that the CSF content of the major metabolite of serotonin, 5-hydroxyindoleacetic acid, was lower in depressed than in nondepressed parkinsonians, suggesting that the alterations in serotonin metabolism in Parkinson's disease identify a subgroup of patients who are prone to depression.
Abstract: Depression is a common symptom in patients with Parkinson's disease. It is not related to the severity of the motor symptoms or changes in dopamine metabolism and does not improve on treatment with dopamine agonists. Alterations in serotonin metabolism are found in primary (endogenous) depression. The brain content of serotonin in Parkinson's disease is also reduced, but this has not been related to any manifestation of the disorder. We found that the CSF content of the major metabolite of serotonin, 5-hydroxyindoleacetic acid, was lower in depressed than in nondepressed parkinsonians. The data suggest that the alterations in serotonin metabolism in Parkinson's disease identify a subgroup of patients who are prone to depression.
338 citations
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TL;DR: In this paper, a cross-sectional analysis of CBF values was carried out among 668 volunteers and patients and subjects were subdivided according to age, gender, and degree of cerebrovascular disease, ranging from healthy volunteers with or without risk factors for stroke to patients with multi-infarct dementia.
Abstract: Cross-sectional analysis of CBF values was carried out among 668 volunteers and patients. Subjects were subdivided according to age, gender, and degree of cerebrovascular disease, ranging from healthy volunteers with or without risk factors for stroke to patients with multi-infarct dementia. Four-year longitudinal analysis was also carried out on 230 individuals from the original sample. Decrements in CBF values were evidenced by both cross-sectional and longitudinal analysis in relation to advancing age, progressive cerebrovascular disease, and dementia. Regional, age-related CBF declines in healthy volunteers were heterogeneous, possibly related to changes in levels of functional activity within different brain regions.
324 citations
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TL;DR: The zygomatic and mandibular branches of the facial nerve were stimulated separately, recording simultaneously from the orbicularis oculi and mental muscles, suggesting ectopic excitation caused by hypocalcemia.
Abstract: We studied 62 patients with hemifacial spasm to test the presence of ephaptic transmission and ectopic excitation. The zygomatic and mandibular branches of the facial nerve were stimulated separately, recording simultaneously from the orbicularis oculi and mental muscles. Antidromic impulses were transmitted bidirectionally between the two branches. Transmission took place in a fraction of slow conducting motor nerve fibers. After-activity and late-activity were recorded as single potentials or trains, suggesting autoexcitation of fibers. The interspike frequency was 250 to 350 Hz. Hyperventilation produced synchronous clonic-tonic activity, suggesting ectopic excitation caused by hypocalcemia. Ectopic excitation and ephaptic transmission are important pathophysiologic factors in hemifacial spasm.
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TL;DR: The right hemisphere seems to be dominant for comprehending emotional prosody but not propositional prosody, and both LHD and RHD groups had more impaired comprehension of propositional Prosody than controls.
Abstract: We studied patients with damage of either the right (RHD) or left hemisphere (LHD) and control subjects to determine whether the RHD patients had a global or limited prosodic defect. Compared with LHD patients and controls, RHD subjects had decreased comprehension of emotional prosody. Both LHD and RHD groups had more impaired comprehension of propositional prosody than controls, but the RHD and LHD groups did not differ. The right hemisphere, therefore, seems to be dominant for comprehending emotional prosody but not propositional prosody.
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TL;DR: In this article, the authors studied 11 patients with amnesia and personality change after surgical repair of a ruptured anterior communicating artery (ACoA) aneurysm, and clinical evidence suggested that infarction in the territory of the ACoA was responsible for amnesia.
Abstract: We studied 11 patients with amnesia and personality change after surgical repair of ruptured anterior communicating artery ( ACoA ) aneurysm. CT and clinical evidence suggested that infarction in the territory of the ACoA was responsible for amnesia and personality change. The medial septal nuclei, the paraventricular nucleus of the anterior hypothalamus, and the medial forebrain bundle are the primary areas of potential interest in these cases. Gross infarction in the frontal lobes is not a requirement for the syndrome.
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TL;DR: The NRS is a simple, reliable, and sensitive scale that can be used with other objective measurements of neurologic function, such as neurophysiologic studies, in the clinical assessment of MS patients.
Abstract: A neurologic rating scale (NRS) has been developed for clinical assessment of MS patients. The scale has been tested on 250 MS patients. Assignment of the NRS score is based on assessment of each component of the neurologic examination and accurately reflects overall neurologic function. Clinical exacerbations are evident as significant deviations from baseline scores. There was close interexaminer correlation, with the range of variability no greater than 2.6%. The NRS is a simple, reliable, and sensitive scale that can be used with other objective measurements of neurologic function, such as neurophysiologic studies, in the clinical assessment of MS patients.
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TL;DR: If a soluble nonacidic dopamine replacement drug can be developed for continuous subcutaneous infusion, “brittle” parkinsonians may be chronically controlled by portable, minipump technology.
Abstract: In 10 parkinsonian patients with severe, chaotic clinical response fluctuations, oral levodopa treatment was replaced by continuous intravenous infusion of levodopa (with orally administered benserazide). Four patients were also given levodopa infusions in addition to their usual oral treatment. All patients remained continuously mobile and ambulant during the infusions. Side effects were minimal, except in two patients with diphasic dyskinesias whose abnormal movements were consistently suppressed by a further increase in the rate of levodopa administration, only to return after an interval. If a soluble nonacidic dopamine replacement drug can be developed for continuous subcutaneous infusion, "brittle" parkinsonians may be chronically controlled by portable, minipump technology.
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TL;DR: On pense que, sur des bases cliniques, la douleur apparaissant dans les neuropathies peripheriques peut provenir des terminaisons normales du tronc du nerf ou de cylindraxes de nocicepteurs leses anormalement excitables et regenerant (douleur dysesthesique).
Abstract: On pense que, sur des bases cliniques, la douleur apparaissant dans les neuropathies peripheriques peut provenir des terminaisons normales du tronc du nerf (douleur tronculaire) ou de cylindraxes de nocicepteurs leses anormalement excitables et regenerant (douleur dysesthesique)
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TL;DR: Differences in clinical and angiographic features of 26 white and 45 black patients with symptomatic occlusive cerebrovascular disease were compared, finding that Blacks had more severe disease of the middle cerebral artery stem and supraclinoid internal carotid arteries.
Abstract: We compared clinical and angiographic features of 26 white and 45 black patients with symptomatic occlusive cerebrovascular disease. White patients had more transient ischemic attacks, carotid bruits, and more severe occlusive disease of the internal carotid artery origin. Blacks had more severe disease of the middle cerebral artery stem and supraclinoid internal carotid arteries. Differences were not explained by racial differences in the prevalence of hypertension, diabetes, hypercholesterolemia, or ischemic heart disease. Since the middle cerebral artery lesions in blacks do not correlate with other accepted epidemiologic, clinical, and laboratory markers of atherosclerosis, the lesions may arise from a disorder that differs from atherosclerosis.
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TL;DR: Nineteen of 50 Parkinson patients had sensory complaints of numbness, coldness, burning, or pain, but there was no objective sensory loss, and sensory symptoms did not correlate with specific motor or autonomic signs.
Abstract: Nineteen of 50 Parkinson patients had sensory complaints of numbness, coldness, burning, or pain. There was no objective sensory loss, and sensory symptoms did not correlate with specific motor or autonomic signs. Symptoms were frequently restricted to the hemiparkinson side and sometimes preceded motor symptoms. Nerve conduction and somatosensory evoked potential studies were normal.
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TL;DR: Clinical and pathologic findings suggest that lesions limited to the hippocampus can cause profound amnesia.
Abstract: A patient developed an amnesic syndrome after cardiopulmonary arrest Neuropathologic examination revealed pronounced anoxic injury to both hippocampi with little damage to other hemisphere structures The clinical and pathologic findings suggest that lesions limited to the hippocampus can cause profound amnesia
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TL;DR: Medroxyprogesterone acetate, a synthetic progesterone, was added to the antiepileptic drug regimen of 14 women who had uncontrolled seizures to suggest further evaluation of MPA for catamenial seizures.
Abstract: Medroxyprogesterone acetate (MPA), a synthetic progesterone, was added to the antiepileptic drug regimen of 14 women who had uncontrolled seizures. Of the 11 women who developed amenorrhea, 7 reported fewer seizures during MPA therapy. Overall reductions in seizure frequency averaged 30% (n = 11), declining from a baseline 8.3 +/- 5.8 seizures per month to 5.1 +/- 4.1 seizures per month (p = 0.02). No serious side effects were encountered, but spotting was common. These preliminary data suggest further evaluation of MPA for catamenial seizures.
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TL;DR: IS and PE may benefit LEMS patients, but caution is required when carcinoma risk is high, and three of six noncarcinoma patients developed sustained clinical and MAP remission after 0.5 to 1 year.
Abstract: We undertook serial clinical and electromyographic muscle action potential (MAP) amplitude assessments in nine patients with the Lambert-Eaton myasthenic syndrome (LEMS) over 0.5 to 2.5 years who received plasma exchange (PE; 5 to 15 exchanges over 4 to 19 days) and immunosuppressive drug (IS) treatment (prednisolone 60 to 100 mg on alternate days, azathioprine 2.5 mg/kg for 0.5 to 2.5 years), and who had no signs of carcinoma at entry. Eight patients responded to PE. The peak MAP response occurred 10 to 15 days post-PE, representing a 2.5-fold mean increase and was significant (p less than 0.01). Two of three patients who developed carcinoma responded initially to IS. Three of six noncarcinoma patients developed sustained clinical and MAP remission after 0.5 to 1 year; the three others, of whom two were intolerant of azathioprine, improved. IS and PE may benefit LEMS patients, but caution is required when carcinoma risk is high.
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TL;DR: Five patients with nonhemorrhagic thalamic infarction with neuropsychological tests, CT, and somatosensory evoked responses (SERs) had abnormalities of language, memory, visuospatial processing, intellect, and personality—changes compatible with dementia.
Abstract: We studied five patients with nonhemorrhagic thalamic infarction with neuropsychological tests, CT, and somatosensory evoked responses (SERs). The three patients with left thalamic lesions had abnormalities of language, memory, visuospatial processing, intellect, and personality—changes compatible with dementia. The two patients with right thalamic lesions were not aphasic and did not have verbal memory defects, but were otherwise comparable. Four lesions occurred in the tuberothalamic artery territory and one in the deep interpeduncular artery territory. SERs revealed a delay in the first negative peak after P 14 in the tuberothalamic patients, and a delay in the third negative peak (N 60 in all patients.
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TL;DR: Analysis of language profiles and CT anatomy in transcortical motor aphasia (TCMA) suggests that the essential lesion is disruption of connections at sites between the supplementary motor area and the frontal perisylvian speech zone.
Abstract: Analysis of language profiles and CT anatomy in transcortical motor aphasia (TCMA) suggests that the essential lesion is disruption of connections at sites between the supplementary motor area and the frontal perisylvian speech zone. If the lesion is extended, there may also be poor articulation (lesion deep to motor strip for face), impaired auditory comprehension (lesion in anterior head of caudate, anterior limb internal capsule, anterior putamen, and anterior portion of external capsule, claustrum, extreme capsule, and insula), or stuttering (lesion in pars opercularis and lower third of premotor region). This concept unifies disparate anatomic and psychophysiologic observations about three syndromes: classical TCMA, aphasia after left medial frontal infarction, and TCMA during recovery from Broca's aphasia.
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TL;DR: A striking feature of the brain morphology in DS may be the curtailment of a specific cell type, most likely the aspinous stellate.
Abstract: Although gross abnormalities have been described in the brains of patients with Down's syndrome (DS), microscopic studies have revealed only minor and inconsistent findings. We compared two DS brains, in whole-brain serial sections, with similarly prepared age- and sex-matched normal controls. Architectonic abnormalities were noted, and cell counts revealed a significant poverty of granular cells in the DS brains, particularly in granular fields such as areas 3, 17, and 41. Golgi studies demonstrated all major cell types. A striking feature of the brain morphology in DS may be the curtailment of a specific cell type, most likely the aspinous stellate.
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TL;DR: The induction of demyelination by MHV-A59 provides a suitable system to study virus-induced demyElination further and is suitable system for studying virus- induced demYelination further.
Abstract: Intracerebral inoculation of 4- to 6-week-old C57BL/6 mice with the A59 strain of mouse hepatitis virus (MHV), a murine coronavirus, produced biphasic disease. Acute hepatitis and mild meningoencephalitis were followed by subacute spastic paralysis with demyelinating lesions in the brain and spinal cord as determined by Eponembedded toluidine-blue-stained sections and by electronmicroscopy. MHV-A59 was cultured by plaque assay from the blood, brain, spinal cord, and liver of infected mice during the acute phase, but not in the chronic stage. MHV-A59 antigen was detected by immunofluorescence (IF) until 3 months postinfection (PI). Serum anti-MHV-A59 antibodies were detected from 7 days to 5 months PI. The induction of demyelination by MHV-A59 provides a suitable system to study virus-induced demyelination further.
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TL;DR: Correlations among prevalence of febrile convulsion, epilepsy, and epileptic EEG abnormality in healthy children were inconsistent in geographically isolated villages identified in Miyake Island.
Abstract: Prevalence rates in all 3-year-old children in Fuchu/Tokyo, for a 6-year survey from 1974 to 1980 (total number examined, 17,044), were 8.3% for febrile convulsion and 0.9% for afebrile convulsion. The figures in boys were higher than in girls for febrile convulsion (9.0%:7.5%), but for afebrile convulsion (0.9%:0.9%). Prevalence in a Miyake Island survey for 10 years, from 1973 to 1982 (total number examined, 543) was 9.9% for febrile and 0.4% for afebrile convulsion. Correlations among prevalence of febrile convulsion, epilepsy, and epileptic EEG abnormality in healthy children were inconsistent in geographically isolated villages identified in Miyake Island.
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TL;DR: Clinical and pathologic data suggested an embolic etiology for most strokes that complicate acute myocardial infarction, including peak creatine kinase and ventricular arrhythmia.
Abstract: We used a computer data bank to evaluate 740 consecutive patients admitted to a cardiac care unit with myocardial infarction. Stroke occurred in 18 (2.4%) patients in the hospital; the anterior circulation was involved in 76% of strokes. Hospital mortality was 61% in patients with stroke and 13% in patients without stroke. Atrial arrhythmia was a significant (p less than or equal to 0.03) risk factor for stroke, but peak creatine kinase and ventricular arrhythmia were not. Cardiac pump failure, apical or anterior-lateral myocardial infarction, and history of previous stroke were associated with an increased risk of stroke. Clinical and pathologic data suggested an embolic etiology for most strokes that complicate acute myocardial infarction.
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TL;DR: Although any form of treatment gave better seizure control, patients undergoing subtotal lobectomy had more than 95% reduction in seizures.
Abstract: One hundred ninety patients were considered for surgical treatment of uncontrolled partial seizures. Twenty-seven (15%) had intracranial mass lesions detected during preoperative evaluation. Seizures were present for a mean of 10 years. Sixty percent of the patients had prior negative radiologic studies. The lesions included 19 neoplasms and 8 non-neoplastic structural (non-atrophic) lesions. Refractory simple partial seizures and a changing neurologic examination were more commonly associated with neoplasms. Treatment included biopsy and radiation, mass resection, or subtotal lobectomy. Although any form of treatment gave better seizure control, patients undergoing subtotal lobectomy had more than 95% reduction in seizures.
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TL;DR: The human M-proteins probably bind to a carbohydrate moiety in MAG that is also present in other PNS myelin proteins, which may explain the observed differences in immunostaining and the sparing of the CNS in patients with anti-MAG M- Proteins.
Abstract: Some patients with neuropathy have IgM M-proteins that bind to myelin and to myelin-associated glycoprotein (MAG). We compared the binding properties of a human anti-MAG M-protein with three mouse monoclonal anti-MAG antibodies (GEN-S1, GEN-S3, GEN-S8) and with a mouse monoclonal antibody (HNK-1) that binds to both MAG and to human natural killer cells. The antibodies GEN-S1, GEN-S3, and GEN-S8 bound to different epitopes in the polypeptide portion of MAG as shown by peptide mapping, deglycosylation and competitive binding studies. The M-protein and HNK-1 bound to both CNS and PNS MAG and to several additional protein bands of 70K, 30K, 26K, and 23K daltons in peripheral, but not in central myelin; they did not bind to deglycosylated MAG. The M-protein and HNK-1 immunostained myelin diffusely, whereas GEN-S8 immunostained only the periaxonal and outer regions of myelin sheath, and there was no staining with GEN-S1 or GEN-S3. The human M-proteins probably bind to a carbohydrate moiety in MAG that is also present in other PNS myelin proteins. This may explain the observed differences in immunostaining and the sparing of the CNS in patients with anti-MAG M-proteins.