Showing papers in "Neurology in 1983"

Rating neurologic impairment in multiple sclerosis An expanded disability status scale (EDSS)

Abstract: One method of evaluating the degree of neurologic impairment in MS has been the combination of grades (0 = normal to 5 or 6 = maximal impairment) within 8 Functional Systems (FS) and an overall Disability Status Scale (DSS) that had steps from 0 (normal) to 10 (death due to MS). A new Expanded Disability Status Scale (EDSS) is presented, with each of the former steps (1,2,3 . . . 9) now divided into two (1.0, 1.5, 2.0 . . . 9.5). The lower portion is obligatorily defined by Functional System grades. The FS are Pyramidal, Cerebellar, Brain Stem, Sensory, Bowel & Bladder, Visual, Cerebral, and Other; the Sensory and Bowel & Bladder Systems have been revised. Patterns of FS and relations of FS by type and grade to the DSS are demonstrated.

Alzheimer's disease: Focal cortical changes shown by positron emission tomography

Abstract: Local rates of cortical glucose metabolism were estimated by positron emission tomography in 13 right-handed patients with Alzheimer's disease. Individuals with disproportionate failure of language function had markedly diminished metabolism in the left frontal, temporal, and parietal regions. Patients with predominant visuo-constructive dysfunction evidenced a hypometabolic focus in the right parietal cortex. Patients with memory failure as the most apparent feature had no significant metabolic asymmetry in cortical regions. In all subjects, verbal competency generally correlated with metabolic activity in the left frontal and temporal areas, while visuo-constructive test performance was linked to glucose utilization in the right parietal lobe.

The anatomic basis of pure alexia

Abstract: The behavioral and anatomic correlates of pure alexia were analyzed in 16 patients. Right homonymous hemianopia failed to appear in three patients, who had right achromatopsia instead. Color anomia and unilateral optic ataxia were seen in six patients. Memory defects were found in two patients. Visual agnosia was noted in two. No patient had visual disorientation. The crucial anatomic correlate of alexia was a lesion of the paraventricular white matter of the left occipital lobe, capable of compromising both interhemispheric and intrahemispheric visual pathways. The lesion associated with color anomia was in the mesial occipitotemporal junction of the left hemisphere.

Adaptation to lateral displacement of vision in patients with lesions of the central nervous system

Abstract: The visual-motor adaptation to lateral displacement of vision by prism glasses was studied in normal individuals and patients with cerebellar dysfunction, Parkinson's disease, right or left cerebral hemisphere lesions, Alzheimer's disease, or Korsakoff's syndrome. Adaptation was analyzed in two phases, the return to normal pointing with prism glasses in place (the "error reduction portion") and the mispointing in the opposite direction after the glasses were removed (the "negative aftereffect portion"). Negative aftereffect, which seems to be the best measure of true adaptation, was significantly reduced only for the cerebellar patients. This poor performance supports the involvement of the cerebellum in motor learning.

What causes infarction in ischemic brain? The Robert Wartenberg Lecture

Abstract: Improved treatment of associated cardiovascular and hematogenous abnormalities has favorably influenced the incidence and outcome of cerebral vascular disease during the past 25 years. Strong evidence now indicates that attention to the carbohydrate content of the brain also may influence outcome from grain ischemia. With brain lactate levels above approximately 16 mmol per kilogram, ischemia produces tissue infarction; ie, the lesion includes astrocytic and endothelial necrosis as well as neuronal death. We find that equal degrees of ischemia accompanied by lower tissue lactate values produce only selective neuronal damage in predictably Vulnerable areas; astrocytes and endothelia are spared and extracellular or progressive postischemic cerebral edema fails to develop. The findings suggest that astrocytes can function to defend brain tissue against the damaging effects of acute anoxia but that during such conditions, they are potentially vulnerable to high tissue lactate levels. Initial clinical evidence suggests that scrupulous attention to blood sugar may reduce the risk of human cerebral infarction after ischemia.

Parkinson's disease in 65 pairs of twins and in a set of quadruplets.

Abstract: Among 43 monozygotic (MZ) and 19 dizygotic (DZ) pairs in which an index case had definite Parkinson9s disease (PD), only one MZ pair was definitely concordant for PD. When pairs with questionable clinical features were included, 4 of 48 MZ and 1 of 19 DZ pairs were concordant. The frequency of PD in MZ cotwins of index cases with PD was similar to that expected in an unrelated control group matched for age and sex. Although we were unable to identify a single environmental agent, we conclude that the major factors in the etiology of PD are nongenetic.

Recovery of behavioral abnormalities after right hemisphere stroke

Abstract: We studied recovery of function in 41 patients with right hemisphere stroke. Recovery was rapid for left neglect, prosopagnosia, anosognosia, and unilateral spatial neglect on drawing (USN). Recovery was slower for h mianopia, hemiparesis, motor impersistence, and extinction. Rates of recovery were intermediate for constructional apraxia and dressing apraxia. Sex had no influence on the rate of recovery. Younger patients recovered from prosopagnosia more rapidly than older patients. Patients with smaller lesions recovered more quickly from anosognosia, USN, and hemiparesis than patients with larger lesions. Patients with hemorrhages recovered more rapidly from constructional apraxia, neglect, and motor impersistence than patients with infarcts. Recovery of function and the factors influencing recovery can by studied systematically by life table methods.

The relation of cembral vasospasrn to the extent and location of subarachnoid blood visualized by CT scan A prospective study

Abstract: In 41 cases of verified ruptured saccular aneurysm, we prospectively predicted the presence or absence of delayed symptomatic cerebral vasospasm. CT criteria quantifying the extent and location of subarachnoid blood (developed in our previous retrospective study) were used in this prospective series of patients. Twenty-two patients had recognizable subarachnoid clots larger than 3 X 5 mm or layers of blood more than 1 mm thick (measured on reproduced images). In 20 of the 22 patients with severe significant clot or thick layer, severe vasospasm was correctly predicted and localized (2 false positives). In 19 patients with no blood, or diffuse blood, or blood outside the subarachnoid space, the absence of severe vasospasm was correctly predicted in 14 (5 false negatives). All of the false-positive and false-negative cases could be explained by inadequate CT technique. The data indicate that the extent and location of blood in the subarachnoid space determine the severity and location of vasospasm and that patients in jeopardy of developing symptomatic cerebral vasospasm can now be identified. Early preventive measures may now be assessed more accurately.

The human Klüver‐Bucy syndrome

Abstract: Twelve patients with the Kluver-Bucy syndrome (KBS) are described. The syndrome occurred in head trauma, Alzheimer's disease, Pick's disease, and following herpes encephalitis. KBS was transient after head trauma but was a persistent feature of the postencephalitic syndrome. In all cases KBS was combined with aphasia, amnesia, or dementia. Human KBS resembles the monkey syndrome, but in humans there is a more elaborate complex of behavioral disturbances. The behavioral manifestations are produced by bilateral temporal lobe dysfunction. Partial expression of the syndrome may have localizing validity.

Behavioral abnormalities after right hemisphere stroke

Abstract: We evaluated 41 patients with unilateral right hemisphere strokes for hemiparesis, hemianopia, constructional apraxia, neglect, extinction, unilateral spatial neglect on drawing (USND), dressing apraxia, anosognosia, prosopagnosia, and motor impersistence. Low correlations were found among USND, neglect, and extinction. Constructional apraxia showed a higher correlation with USND than with hemianopia. Motor impersistence and anosognosia correlated with the severity of the hemiplegia. Left neglect, motor impersistence, and anosognosia tended to occur only with large strokes. Injury to the right parietal lobe appears to be an important determinant of USND and constructional apraxia. In most cases of motor impersistence, left neglect, and anosognosia, there was injury to the right parietal lobe and also to structures beyond the parietal lobe. In two cases, small deep lesions produced behavioral abnormalities comparable to those of larger superficial cortical lesions.

Osteosclerotic myeloma and peripheral neuropathy

Abstract: Sixteen cases of osteosclerotic myeloma and peripheral neuropathy (SM-PN) were reviewed. The neuropathy resembled chronic inflammatory-demyelinating polyneuropathy with predominantly motor disability, high CSF protein levels, and low motor nerve conduction velocities. Twelve of the 16 patients had detectable levels of monoclonal serum proteins, all with A light chains, but results of other laboratory studies were usually normal. Most of the patients also had organomegaly, endocrine abnormalities, or both. Treatment of solitary lesions with tumoricidal irradiation usually improved the neuropathy and reversed the nonneurologic abnormalities; chemotherapy for multiple osteosclerotic lesions was less helpful.

Myasthenia gravis: Long‐term correlation of binding and bungarotoxin blocking antibodies against acetylcholine receptors with changes in disease severity

Abstract: Sixty patients with myasthenia gravis were examined prospectively by measuring serial titers of antibodies against human acetylcholine receptor, and these were correlated with a quantitative clinical score. Serial titers of antibodies detected by the standard immunoprecipitation assay (binding antibodies) correlated with the clinical score in most patients. Antibodies blocking the binding of alpha-bungarotoxin to receptors (blocking antibodies) were detected in 29 patients. Serial blocking antibody titers correlated with changes in muscle weakness less often than binding antibody titers. Titers of both classes of antibodies often followed a divergent course, suggesting that the autoimmune B-cell clones that formed these classes of antibodies may have been activated asynchronously.

Abnormal ocular motor control in Huntington's disease

Abstract: We studied eye movements in 50 patients with Huntington9s disease. Fixation was impaired in 73% of patients; such individuals had difficulty in suppressing saccades toward novel visual stimuli. Impaired initiation of saccades was manifest by increased reaction time (89%) and inability to make a saccade without head movement (89%) or blink (35%). Saccades and quick phases of nystagmus were slowed in 62%. Smooth pursuit was abnormal in 60%, and vergence in 33%. The vestibulo-ocular reflex and the ability to hold eccentric gaze were preserved even late in the disease.

Cerebrospinal fluid pressure in normal obese subjects and patients with pseudotumor cerebri

Abstract: Cerebrospinal fluid (CSF) pressure was measured in four groups of patients: 116 with acute pseudotumor cerebri, 18 with chronic pseudotumor cerebri, 41 obese and 15 nonobese normal patients undergoing spinal anesthesia. Spinal fluid pressure between 200 and 250 mm H2O was found in members of each group. This suggests that when elevated CSF pressure is suspected, confirmation requires values greater than 250 mm H2O. The CSF pressure and degree of obesity could not be correlated in any meaningful way. There was no significant statistical difference between the mean CSF pressures obtained in the obese and nonobese normal populations.

Local cerebral metabolism during partial seizures

Abstract: Interictal and ictal fluorodeoxyglucose scans were obtained with positron CT from four patients with spontaneous recurrent partial seizures, one with epilepsia partialis continua, and one with a single partial seizure induced by electrical stimulation of the hippocampus. Ictal metabolic patterns were different for each patient studied. Focal and generalized increased and decreased metabolism were observed. Ictal hypermetabolism may exceed six times the interictal rate and could represent activation of excitatory or inhibitory synapses in the epileptogenic region and its projection fields. Hypometabolism seen on ictal scans most likely reflects postictal depression and may indicate projection fields of inhibited neurons. No quantitative relationship between alterations in metabolism and EEG or behavioral measurements of ictal events could be demonstrated.

Demyeliating neuropathy and monoclonal IgM antibody to myelin‐associated glycoprotein

Abstract: We studied a patient with demyelinating neuropathy and monoclonal IgM kappa antibody to the major myelin-associated glycoprotein (MAG). Binding of this monoclonal antibody to the myelin antigen was demonstrated by imimunoelectroblot. Binding to MAG seemed to be specific, because it was completely inhibited by MAG isolated from human myelin. Immunostaining was observed with MAG from CNS and peripheral nervous system myelin.

Olfactory impairment in Parkinson's disease

Abstract: In comparison with closely matched controls, patients with Parkinson's disease had reduced scores in tests of odor detection and qualitative discrimination. Olfactory impairment was not related to age, duration of symptoms, treatment, intellectual function, or genetic factors and seemed to be a nonmotor manifestation of the disease.

The syndrome of bilateral paramedian thalamic infarction

Abstract: Bilateral anterior paramedian thalamic infarction resulting from occlusion of a bilaterally distributed thalamosubthalamic paramedian artery was demonstrated on CT in two patients. Patient 1 presented with a transient coma followed by asterixis, hypersomnia, vertical gaze disturbances, profound Korsakoff amnesic syndrome, and a subcortical dementia. Patient 2, with a predominantly right-sided thalamic infarct, showed good recovery from amnesia and vertical gaze disturbances. However, patient 1 remained with severe amnesia and mild subcortical dementia at follow-up 1 year later. These and similar reported cases constitute a lacunar syndrome with characteristic clinical and CT features.

Behavioral correlations of dopamine receptor activation

Abstract: According to the classification scheme of Kebabian and Calne, 2 there are two types of dopamine (DA) receptors: D 1 (activation of which causes increased cyclic AMP formation) and D 2 (activation of which causes no increment in cyclic AMP). It is not clear what role the different receptors play in mediating motor behavior. Using drugs that act selectively at only one receptor site, we studied the effects of D 1 and D 2 receptor activation in two different models of parkinsonism—the rotating rat and the reserpinized mouse. Neither the D 1 agonist nor the D 2 agonist, given alone, could overcome reserpine akinesia, but together they restored locomotor activity. In rats with unilateral nigrostriatal lesions, both drugs induced a rotational response, each with a distinct temporal pattern. Pretreatment with alpha-methyl-paratyrosine (an inhibitor of DA synthesis) led to decrements in the rotational response induced by D 2 agonists, but not that induced by D 1 agonists. The mechanism by which these DA agonists induce motor activity is different; activation of both types of DA receptors seems to be necessary for normal motor behavior.

Psychogenic seizures: a clinical study with follow-up data.

Abstract: We evaluated the natural history and prognosis of psychogenic or hysterical seizures in patients discharged from the Johns Hopkins Hospital in the 3 years between 1971 and 1974. Follow-up data were obtained 5 years or more after discharge. Among 41 patients with convincing evidence of psychogenic seizures, there were coexisting organic neurologic disorders in 18 (44%). Mental subnormality or retardation was present in 17% and true epileptic seizures in 37%. EEG abnormalities found in 38% of individuals with psychogenic seizures were attributed to these organic neurologic disorders or anticonvulsant drug toxicity. There was significant long-term morbidity in 56% of these individuals because of psychosocial problems rather than misdiagnosis of psychogenic seizures.

Blepharospasm associated with brainstem lesions

Abstract: We studied six patients with clinical and radiographic evidence of rostral brainstem lesion and bilateral blepharospasm. Two patients also had other facial dystonic movements. Four patients suffered rostral brainstem strokes, and two had multiple sclerosis. None had been treated with antipsychotic drugs prior to the onset of blepharospasm. Medical treatment was helpful in two patients, and bilateral selective facial nerve section was used in another patient. Possible pathogenic mechanisms are discussed.

Ocular counterrolling as an indicator of vestibular otolith function

Abstract: Sixteen normal subjects, 8 patients with unilateral vestibular nerve section, and 11 patients with acoustic neuromas underwent dynamic ocular counterrolling (OCR) testing. Both eyes were photographed at every 10 degrees as the subject was tilted about the naso-occipital axis at a constant velocity of 3 degrees/sec. Normal subjects differed from patients in four characteristics: (1) The binocular OCR patterns of normal subjects were consistent from one trial to the next. (2) The two eyes were mostly conjugate in movement. (3) The patterns were smooth. (4) OCR was approximately symmetric to right and left tilts. In the patients, patterns were abnormal when tilted to the side opposite the lesion. In quantified measures of the four characteristics, differences between the normal group and the group of patients with unilateral vestibular nerve sections were significant in consistency and total scores. In the patients with acoustic neuromas, OCR abnormalities corresponded to the size and location of the tumors and the extent to which they impinged on the utricular nerve and brainstem.

ACTH and prednisone in childhood seizure disorders

Abstract: We treated 116 children with ACTH or prednisone. Fifty-two had infantile spasms with hypsarhythmia, and 64 had other types of intractable seizures. ACTH completely controlled seizures in all patients with infantile spasms and hypsarhythmia and 74% of those with other types of seizures. Prednisone controlled 51% of patients with infantile spasms and none with other seizures. Serious side effects were minimal for both drugs, and recurrent seizures occurred in 40 to 50% of patients within 4 to 14 months after completion of therapy.

High dosage anticholinergic therapy in dystonia

Abstract: Twenty-three children and 52 adults with torsion dystonia of various etiologies and distribution patterns of the involuntary movements were treated in an open-label study with anticholinergic medication. The dosage was increased gradually until there was either benefit or intolerable adverse effects. Trihexyphenidyl was used initially, but later ethopropazine was given to adult subjects. Significant benefit occurred in 61% of children and in 38% of adults. The average daily dosages were 41 mg trihexyphenidyl for children, 24 mg trihexyphenidyl for adults, and 350 mg ethopropazine for adults. Adverse effects were the major limiting factor to high dosage in adults, but not in children.

Performance On hemispatial pointing task by patients with neglect syndrome

Abstract: Five patients with left hemispatial neglect and five aphasic control subjects were given a hemispatial pointing task. They were instructed to point to an imaginary point in space perpendicular to the midline of the chest. Right hemisphere-damaged subjects with neglect deviated more into the hemispace ipsilateral to the lesion than left hemisphere-damaged controls. This task did not require sensory input from left hemispace, and the defective performance cannot be attributed to sensory inattention or to impaired gaze or memory. The finding is compatible with hemispatial akinesia.

Cerebral blood flow and cerebrovascular CO2 reactivity in stroke-age normal controls.

Abstract: We used the noninvasive 133-xenon inhalation technique to determine cerebral hemodynamics in 55 normal volunteers aged 18 to 88. Values for cerebral blood flow and cerebrovascular CO 2 reactivity in fastclearing tissue (flow gray) and slow-clearing tissue (flow white) were examined as functions of age and in relation to hematocrit, blood pressure, and evidence of extracranial vascular disease. Flow gray declined linearly with age, but no corresponding change was found in flow white or in CO, reactivity. The data suggest that the progressive fall in flow gray is due to a physiologic aging process.

Multiple sclerosis after age 50

Abstract: When multiple sclerosis (MS) presents late in life (after the age of 50), it frequently gives diagnostic difficulty. In a large MS clinic population (N = 838), 9.4% of the patients had late onset of MS. Slow deterioration of motor function characterized the initial symptoms and subsequent course of this group of older patients. Progression of disability was more rapid than in younger patients. Evoked response studies and CSF electrophoresis were of high diagnostic yield in the older patient group. Reasons for the differing clinical features in the late-onset patients are discussed. We suggest that the age-of-onset criterion for MS be raised to 60 in adequately investigated cases.

CT and clinical correlations in recent aneurysmal subarachnoid hemorrhage: a preliminary report of the Cooperative Aneurysm Study.

Abstract: Computed tomography was performed on 1,378 patients admitted within 3 days of subarachnoid hemorrhage (SAH) at 71 centers in a cooperative study. CT on the day of SAH (day 0) was abnormal in 95.8%. Intracranial blood was detected in 95.3% of patients on day 0, 90.5% on the first day after SAH (day 1), and 73.8% on day 3. CT was normal in only 1 of 284 patients in stupor or coma but was normal in 14.6% of 638 alert patients. In the first few days after SAH, CT is highly accurate, often obviating the need of CSF examinations. The interval from SAH until CT and the seriousness of the patient's condition are two factors that influence the results of CT.

Complex partial seizures: Clinical characteristics and differential diagnosis

Abstract: Videotape analysis of 163 complex partial seizures in 40 patients showed that the mean duration of the attack was 128 seconds. Automatisms occurred in 159 seizures (97%) and involved more than the face and arms in 132 (80%). Most automatisms were simple, stereotypic, or aimless movements. Postural tone increased in 24 seizures and decreased in 62. Clonic movements of the eyelids occurred in 19 attacks, and clonic movements of the extremities in 4. Only nine patients reported auras. Distinct ictal and postictal phases could be distinguished in 132 seizures (80%); in these, the mean ictal duration was 54 seconds and the mean postictal duration 89 seconds. Videotape analysis provides objective criteria by which complex partial seizures may be differentiated from other seizure types.

Multifocal central nervous system damage caused by toluene abuse

Abstract: Four toluene abusers had evidence of severe multifocal central nervous system damage. Impairment of cognitive, cerebellar, brainstem, auditory, and pyramidal tract function, as well as CT evidence of cerebral cortical, cerebellar, and brainstem atrophy, have been noted. In addition, we found opsoclonus, ocular flutter, and ocular dysmetria. All three patients tested had abnormal brainstem auditory evoked potentials, indicative of brainstem dysfunction. The patient with opsoclonus had CT evidence of brainstem, cerebellar, and cerebral cortical atrophy.