Showing papers in "Neurology in 1980"

Line bisection and unilateral visual neglect in patients with neurologic impairment

Abstract: Unilateral visual neglect is a common symptom or sign in patients with lesions of the nondominant hemisphere. Several techniques have been used to demonstrate visual neglect. One such technique—asking a patient to bisect a horizontal line and expecting an estimate of center away from the side neglected—has been used for over 70 years but has not been statistically evaluated. We conducted a formal evaulation of this method and found that under special conditions, line-bisection performance can discriminate between patients with right-hemisphere lesions and patients with diffuse lesions, patients with left-hemisphere lesions, and hospital controls. When used to investigate visual neglect in an individual patient, the line-bisection test should be given in conjunction with other complementary procedures such as symmetric drawings and the Memory-for-Designs Test.

Assumptions in the radiotherapy of glioblastoma

Abstract: In the light of advances in computerized tomography (CT), we have retrospectively evaluated the assumptions that underlie the radiation therapy of glioblastoma: (1) No neuroradiologic technique provides an accurate delineation of tumor bulk and location, (2) glioblastoma is commonly multicentric, and (3) a major source of therapeutic failure is recurrence beyond radiotherapy fields. 1. CT scans, performed on glioblastoma patients within 2 months of postmortem examination, defined both gross and microscopic tumor extent (within a 2-cm margin) in all but 6 of 35 patients evaluated. The major source of error was subependymal spread (four patients). 2. Multicentricity occurred in only 4% of untreated and 6% of treated (radiotherapy with or without chemotherapy) patients. All multicentric lesions were identified on CT scans. 3. Serial CT scans on 42 patients revealed that glioblastoma recurred within a 2-cm margin of the primary site in 90%. Occurrences outside this margin were accurately delineated by CT in all instances. Because most patients show recurrence within or in close proximity to the original site, current radiation doses would appear to be inadequate for therapy of the primary tumor. CT scan accuracy may permit smaller-field and higher-dose irradiation therapy for glioblastoma.

Right hemisphere dominance for attention The mechanism underlying hemispheric asymmetries of inattention (neglect)

Abstract: Because hemiinattention is most commonly caused by right parietal lesions, it is possible that the left hemisphere attends to contralateral stimuli whereas the right attends to both contralateral and ipsilateral stimuli. We gave lateralized visual stimuli to 12 normal subjects and recorded the electroencephalograms. Desynchronization was determined by comparing the alpha power 1 second before and 1 second after a lateralized visual stimulus. Although the left parietal lobe desynchronized most after right-sided stimuli, the right parietal lobe desynchronized equally after right or left stimuli. These findings support the hypothesis that the right hemisphere is dominant for attention.

The control of muscle tone, reflexes, and movement: Robert Wartenbeg Lecture

Abstract: My pleasure at being invited to give the Wartenberg lecture this year was enhanced by the admiration I hold for the writings of Wartenberg, who pruned the eponymous tree in which so many clinicians of the past had sought to roost forever. Vast numbers of named reflexes evoked by stroking, squeezing, or tapping some bodily appendage were shown to be different ways of testing the same mechanism.’S2 Wartenberg’s analysis simplified neurology by clearing away unnecessary and confusing terminology. Robert Wartenberg was born in the Russian city of Grodno in 1887, graduated in medicine with distinction from the University of Rostock in Germany and became Physician-in-Chief of the Nerve Clinic in Freiburg before fleeing from Germany in the dark days of 193!X3 For 20 years, until his death in 1956, Wartenberg served American neurology well, becoming Clinical Professor of Neurology at the University of California School of Medicine, San Francisco, in 1952. He was a fellow of the American Academy of Neurology and an associate editor of Neurology. To those who knew him, he combined precision of thought and a sharp critical faculty with a capacity for warmth and humor. Dr. Robert Aird recalls his rejecting compromise with the zestful cry “By the great Babinski, Professor Sigvald Refsum told me of an occasion when Wartenberg was demonstrating the physical signs of a patient with double athetosis. Wartenberg commented that the patient’s facial movements resembled those of someone chewing gum. He was startled when the patient said, “But I am chewing gum,” removing the gum from his mouth and displaying it to the laughing audience. Wartenberg promptly retorted, “Then this is double double athetosis.” In honored memory of such a man, it is my privilege to dedicate this lecture. My own interest in neurology was aroused as a medical student because the explanation of many physical signs did not ring true. Was spasticity really the same as decerebrate rigidity? Was the cogwheel phenomenon in Parkinson disease caused by the combination of the classical tremor and rigidity when some patients did not have a perceptible tremor? How could degeneration of the dorsal columns abolish tendon jerks in Friedreich ataxia? Why did so many muscles contract when the radius was tapped in a spastic patient? My opportunity to study the motor system arrived when Dr. Peter Bishop, now Professor of Physiology in the Australian National University, Canberra, started a brain research unit in the University of Sydney and encouraged me to work on the pyramidal tract. The outcome of those early years was the finding of two groups of fibers with distinctive properties in the cat pyramidal tract,* the mapping of their origin from the ~ o r t e x , ~ their distribution in the cord6 and the fact that they made no attempt to regenerate after the pyramidal tract was sectioned.’ It is probable that the more slowly conducting group is related to the maintenance of posture and the rapidly conducting group to phasic movement8 but the interaction of the two groups may be comple~ .~

Seizures after head trauma A population study

Abstract: A cohort of 2747 patients with head injuries was followed for 28,176 person-years to determine the magnitude and duration of the risk of posttraumatic seizures. Injuries were classified as severe (brain contusion, intracerebral or intracranial hematoma, or 24 hours of eight unconsciousness of amnesia), moderate (skull fracture or 30 minutes to 24 hours of unconsciousness or amnesia), and mild (briefer unconsciousness or amnesia). The risk of posttraumatic seizures after severe injury was 7.1% within 1 year and 11.5% in 5 years, after moderate injury the risk was 0.7 and 1.6%, and after mild injury the risk was 0.1 and 0.6%. The incidence of seizures after mild head injuries was not significantly greater than in the general population.

“Top of the basilar” syndrome

Abstract: Infarction of rostral brainstem and cerebral hemispheral regions fed by the distal basilar artery causes a clinically recognizable syndrome characterized by visual, oculomotor, and behavioral abnormalities, often without significant motor dysfunction. Rostral brainstem infarction produces oculomotor and pupillary signs that are identical to those in thalamic hemorrhage. Somnolence, vivid hallucinations and dreamlike behavior may also accompany rostral brainstem infarction. Temporal and occipital infarctions are frequently accompanied by hemianopia with distinctive characteristics, fragments of the Balint syndrome, amnestic dysfunction, and agitated behavior. The “top of the basilar” syndrome is most often due to an embolus.

Is the mesocortical dopaminergic system involved in Parkinson disease

Abstract: Tyrosine hydroxylase was used to identify catecholaminergic neurons in the substantia nigra and ventral tegmental area of the human mesencephalon. High enzyme activity in the normal ventral tegmental area indicated the presence of numerous cathecholaminergic neurons, most likely dopamine cells. Tyrosine-hydroxylase activity was decreased in the ventral tegmental area of parkinsonian patients, implying a lesion of the mesocortical dopamine system in Parkinson disease.

The unchanging pattern of subarachnoid hemorrhage in a community

Abstract: The average annual incidence of subarachnoid hemorrhage (SAH) from aneurysm rupture in Rochester, Minnesota, has remained remarkably constant at about 11 per 100,000 population. Age-specific incidence increased with age. Survival after SAH depended on: (1) clinical grade, (2) time after onset of SAH, and (3) presence of intracerebral hematoma. Among those who survived to receive medical attention, 48% were clinical grade 1 or 2, 20% were grade 3, and 32% were grade 4 or 5. Proved rebleeding occurred within 10 days of the first SAH in 20% of patients who survived until hospital admission.

The incidence, causes, and secular trends of head trauma in Olmsted County, Minnesota, 1935–1974

Abstract: Records in the Mayo Clinic linkage system were reviewed to determine the incidence of head trauma in Olmsted County, Minnesota, from 1935-1974. Minimum criteria for inclusion--loss of consciousness, posttraumatic amnesia, or skull fracture--were met by 3587 cases. During the decade from 1965-1974, the age-adjusted incidence rates per 100,000 population were 270 in males and 116 in females. The rate was highest--658--in males aged 15 to 24, but it was at least 50 in all age and sex groups. Major causes of head injury were automobile accidents (37%) and falls (29%). The incidence of head injuries related to automobiles and recreation has been increasing, whereas most other categories have remained stable or have declined. Of all cases, 446 were fatal, the average annual incidence being 32 per 100,000 in males and 9 per 100,000 in females. Among the groups at high risk of head trauma are those who have had head trauma previously.

Selective decline in cellular immune response to varicella‐zoster in the elderly

Abstract: The incidence of herpes zoster rises markedly in the aged. We evaluated the hypothesis that cellular immunity to varicella-zoster (VZ) viral antigens may be impaired in aged subjects. We found that the lymphocyte proliferation to VZ antigen was less in older asymptomatic individuals than in normal young controls. In contrast, responses to other antigens did not differ. Antibody titers to VZ were similar in both young and old subjects. Impairment of cellular immunity to VZ, on a population basis, may contribute to the increased risk of herpes zoster in the elderly.

Epidemiologic contributions to multiple sclerosis: an overview.

Abstract: Geographically, multiple sclerosis (MS) seems to be distributed into three zones of high, medium, and low frequency. High-frequency areas, with prevalence rates over 30 per 100,000 population, include Europe between 65° and 45° north latitude, southern Canada and the northern United States, and New Zealand and southern Australia. These regions are bounded by areas of medium frequency with prevalence rates of 5 to 25 per 100,000, which include southern Europe, the southern United States, and most of Australia. Known areas of Asia and Africa (save for one white group in South Africa) are all low, with prevalence rates under 5 per 100,000 population. All high- and medium-risk areas are among predominantly white populations: In America, blacks and Orientals have much lower rates of MS than whites but still demonstrate the geographic gradients found for whites. Migration studies indicate that on the whole, migrants retain much of the risk of their birthplace. However, this risk is clearly not defined at birth: MS death rates for migrants born in one risk area and dying in another are intermediate between the rates characteristic of their birthplaces. Prevalence studies for migrants from high- to low-risk areas indicate the age of adolescence to be critical for risk retention; those migrating under age 15 years acquire the lower risk of their new residence. Furthermore, several low-to-high studies show that those migrating in childhood or adolescence increase the risk of MS. The migrant data, plus the geographic distributions, serve to define MS as an acquired, exogenous (environmental) disease whose acquisition in ordinary circumstances takes place years before clinical onset. The data fit best the “simple” or “prevalence” hypothesis: that the cause of MS will be found where the clinical disease is common. Further evidence for this viewpoint is provided by the occurrence of two epidemics of MS: one (definite) in the Faroe Islands, the other (probable) in Iceland. Both followed the occupation of those lands by British forces during World War II. If this relation is causal, MS is not only acquired but also transmittable.

Interictal memory and language impairment in temporal lobe epilepsy.

Abstract: Memory and language were evaluated in patients with temporal lobe epilepsy and generalized epilepsy. Subjects were matched for age, duration of illness, and seizure frequency, and grouped according to the electroencephalographic results and seizure type into right temporal, left temporal, and generalized. In formal tests of intelligence, auditory and visual memory, and language, a significant difference was noted only on a confrontation naming test. The mean score on this test was considerably lower in the left temporal group; right temporal and generalized groups scored in the normal range. This correlated with impairment on many verbal subtests of intelligence and memory. These results suggest that the interictal memory impairment of temporal lobe epilepsy may be an anomia and that the anomia may contribute to impairment of verbal learning and memory; both circumlocution and circumstantiality may compensate for anomia.

Central achromatopsia Behavioral, anatomic, and physiologic aspects

Abstract: The neuropsychologic, neuroanatomic, and neurophysiologic correlates of achromatopsia were studied in two patients. Prosopagnosia accompanied the color perception defect in the bilateral case but not in the unilateral one. No other neuropsychologic disturbance was present in either case. The lesions compromised the ventromedial sector of the occipital lobe in both cases. Cerebral evoked responses produced by pattern shift stimulation were normal for black and white but abnormal for red and green, when stimulation was given in the achromatopsic field.

Infectious agents in spinal epidural abscesses

Abstract: Of 27 cases of spinal epidural abscess, 19 were caused by bacteria, 7 by Mycobacterium tuberculosis, and 1 by Echinococcus granulosus. Blunt trauma and cutaneous infections were the most frequent preceding events in bacterial cases. Tuberculous abscess was usually the sole manifestation of reactivation of dormant tuberculosis. Drug addiction, the most common cause in young adults, was associated with gram-negative infections. Whatever the infectious agent, paraparesis for longer than 4 days led to a poor outcome. Myelography was the best diagnostic test, whereas lumbar puncture and percutaneous bone biopsy offered little specific information.

Central neurofibromatosis with bilateral acoustic neuroma Genetic, clinical and biochemical distinctions from peripheral neurofibromatosis

Abstract: Neurofibromatosis includes the common “peripheral” form and a recently documented “central” form. We describe the central form in 130 cases from 9 kindreds personally studied and 15 reported kindreds. Central neurofibromatosis with bilateral acoustic neuroma is an autosomal dominant disorder beginning about 20 years of age, accompanied by mild skin changes. In three kindreds with central neurofibromatosis, we measured nerve growth factor in serum by radioimmunoassay and radioreceptor assay. Only the antigenic activity of nerve growth factor was increased. In contrast, in peripheral neurofibromatosis, only the functional activity of nerve growth factor has been reported increased. Central and peripheral forms of neurofibromatosis are closely related but discrete diseases which appear to have separate alterations in nerve growth factor activity.

Fatal infhntile mitochondrial myopathy and renal dysfunction due to cytochrome‐c‐oxidase deficiency

Abstract: A 1-month-old boy was admitted because of failure to thrive. He was floppy and had bilateral ptosis, diminished reflexes, and poor suck. He had aspiration pneumonia, developed seizures, and died at age 3 1 / 2 months. Laboratory data showed lactic acidosis, proteinuria, glycosuria and generalized aminoaciduria. He was an only child, and family history was negative. Muscle biopsy showed large clumps of granules positive with oxidative enzyme stains and increased lipid droplets. Ultrastructural studies showed large aggregates of mitochondria, many of which were greatly enlarged and contained disoriented or concentric whorls of cristae and paracrystalline inclusions. Cytochrome c oxidase was absent in fresh frozen sections by histochemical staining. By biochemical assay, cytochrome c oxidase (cytochrome aa3) was 6% of normal in muscle biopsy and undectectable in autopsy muscle; spectra and content of cytochromes showed lack of cytochrome aa3, decreased cytochrome b and normal cytochrome cc1. In kidney, cytochrome-c-oxidase activity was 38% of normal and spectra showed decreased cytochromes aa3 and b. The association of fatal infantile mitochondrial myopathy, lactic acidosis and renal dysfunction was previously reported by Van Biervliet et al and appears to be a distinct nosologic entity, one of the few biochemically defined mitochondrial myopathies.

Relationship between plaques, tangles, and dementia in Down syndrome

Abstract: In patients with Down syndrome, senile plaques and neurofibrillary tangles accumulate in the cortex at an earlier age than in persons of normal karyotype. We studied 20 Down syndrome patients dying after age 30 (average age, 49); all had neocortical plaques and tangles, but only 3 of 20 had been demented. In 12 cases (average age, 53), tissue was available for quantitative study of plaque and tangle densities and estimation of cell loss in the hippocampus. Although at least 8 of these 12 cases had plaque and tangle densities comparable to those previously reported in demented old people, only 1 had dementia. The regional distribution of plaques and tangles in the hippocampus of these Down cases differed from the pattern in senile dementia. Although Alzheimer-like dementia occurs in Down disease, it is less prevalent than the plethoric plaques and tangles in the cortex.

Glial fibrillary acidic protein and Alzheimer‐type senile dementia

Abstract: Glial fibrillary acidic protein (GFAP), a protein that is associated with 9-nm filaments of astrocytes, was observed to be increased in the astrocytes surrounding senile plaques in patients with Alzheimer dementia and in aged subjects without dementia. A few GFAP-positive fibers were seen in the centers of plaques. These results emphasized the selectivity of senile changes; whereas some cells seemed to undergo degeneration or dysfunction, other cells--astrocytes--maintain their capacity for reaction and may increase the formation of at least one protein, GFAP.

Aphasia after left hemispheric intracerebral hemorrhage

Abstract: The function of subcortical nuclear structures in language is uncertain, and language disorders after injury to these structures are described incompletely. We report 15 patients with left putaminal or thalamic hemorrhage, describe the range of language and behavioral disorders produced, and review the potential mechanisms of these disorders. Clinicoanatomic correlations revealed no definite differences between aphasia after hemorrhage in putamen or in thalamus.

Multiple sclerosis Measurement and validation of central nervous system IgG synthesis rate

Abstract: An empirical formula has been developed to calculate the de novo rate of synthesis of IgG in the central nervous system (CNS), based on physiologic principles that govern the passage of albumin and IgG across the blood-brain barrier (BBB). To validate the formula, radiolabeled IgG and albumin from pooled normal sera were followed from the blood to the cerebrospinal fluid (CSF) over 21 days in nine patients with definite multiple sclerosis (MS). IgG synthesis rates were calculated by the isotope exchange method and compared to values obtained with the empirical formula. There was excellent concordance, from a low rate of synthesis of 5 mg per day to a high rate of 120 mg per day. A double radiolabeled IgG experiment in two patients showed that the MS BBB processed normal serum IgG in the same way as IgG derived from autologous MS serum. Accordingly, the empirical formula, which requires only one sample of CSF and matched serum, can reliably and validly estimate the de novo rate of IgG synthesis in CNS of patients with MS.

Clinical criteria for diagnosis of Machado‐Joseph disease: Report of a non‐Azorean Portuguese family

Abstract: A Portuguese family of non-Azorean origin is described as affected by an autosomal dominant inherited ataxia resembling Machado-Joseph disease. Clinical criteria for diagnosis are proposed, based on a complex clinical picture extending from extrapyramidal signs to peripheral amyotrophy associated with secondary, but more specific, minor features such as progressive external ophthalmoplegia, dystonia, intention fasciculation-like movements of facial and lingual muscles, and bulging eyes. Machado-Joseph disease may be more widespread than previously believed.

Pathogenesis of Binswanger chronic progressive subcortical encephalopathy.

Abstract: We studied the clinicopathologic findings in four hypertensive patients with multiple leukomalacia, demyelinated lesions, and lacunar state. Only one patient had clinical evidence of dementia. The periventricular watershed infarcts were attributed to transient episodes of cardiac failure in brains with a compromised circulation in the territory of the deep perforating branches. These observations suggest that Binswanger encephalopathy does not differ from multi-infarct dementia.

Evidence for and against the transmissibility of Alzheimer disease

Abstract: Nonhuman primates were inoculated intracerebrally with brain tissue from 52 patients with confirmed Alzheimer disease (AD) in order to investigate the possibility of an infectious etiology. Animals inoculated with brain tissue from two patients with familial AD developed a spongiform encephalopathy that was indistinguishable from Creutzfeldt-Jakob disease (CJD). Seventeen other cases of AD on test for more than 50 months failed to produce similar changes, and 33 cases have not been incubating for a sufficient period of time to ascertain the presence of a transmissible agent. The initial transmission of spongiform encephalopathy with brain tissue from the two familial cases of AD has not been reproduced and the association between AD and an infectious agent has not yet been demonstrated with any reasonable degree of certainty. The frequent overlap of clinical symptoms of AD and CJD, and the occurrence of cases of CJD and AD in the same families indicate the need for continuing research on the relationship between the two diseases.

Acquired aphasia with convuIsive disorder Course and prognosis

Abstract: Acquired aphasia with convulsive disorder is an unusual condition in childhood, characterized by loss of language function associated with a paroxysmal electroencephalogram. To determine the course and outcome of this disorder, we evaluated nine patients 10 to 28 years after the onset of aphasia. Four patients had recovered fully, one had mild language dysfunction, and four had moderate language disability. Four of the five patients with the best outcome had decreased visuoperceptive function as measured by the Revised Benton Visual Retention Test (RBVRT), whereas the three tested patients with moderate language dysfunction had normal RBVRT scores.

Pattern shift visual, brainstem auditory, and short-latency somatosensory evoked potentials in multiple sclerosis.

Abstract: Pattern shift visual (PSVER), brainstem auditory (BAER), and short-latency somatosensory (SER) evoked potentials are practical and reliable clinical tests that provide objective measures of impulse conduction in the respective central nervous system (CNS) white matter tracts. In large groups of patients with multiple sclerosis (MS), the abnormality rates were 56, 32, and 60% (PSVER, BAER, and SER, respectively). Evidence of clinically unsuspected lesions was found in 42, 21, and 51%, respectively. Abnormality rates for a definite MS group alone were higher. The tests provide confirmatory and additional evidence for multiple lesions in CNS white matter. They may also provide an objective assessment of the efficacy of therapeutic regimens.

Correlation of arteriographic findings and symptoms in cerebrovascular disease.

Abstract: The arteriograms of 109 patients with symptomatic cerebral ischemia were analyzed to determine the distribution of extracranial and intracranial vascular abnormalities. In the 66 patients with transient hemispheric or ocular ischemia, potentially embolic lesions were more common than hemodynamically significant lesions (84% versus 50%). In the 29 patients with fixed neurologic deficits, 25% had occlusion of the internal carotid artery on the appropriate side; ulcerated lesions were again more common in the remaining patent arteries than were hemodynamically significant lesions. The major difference between the transient group and the fixed group was the 29% incidence of intracerebral disease or anomaly in the transient group, with similar lesions in 90% of the group with fixed neurologic deficit. Symptoms of cerebral ischemia are more likely to be related to ulceration than to hemodynamically significant lesions. The risk of stroke seems greatest when there is also intracerebral siphon disease or anatomic anomaly of the circle of Willis.

Neuropsychologic impairment in astxocytoma survivors

Abstract: Thirteen patients selected for long-term survival with primary astrocytic tumor (who failed to return to premorbid educational or vocational levels) were examined by neuropsychologic tests of specific and generalized higher cortical functions. In the absence of tumor regrowth or other neurologic disorders, each demonstrated difficulty in problem solving or coping with novel situations when previously acquired abilities, overlearned material, and psychometric intelligence appeared consistent with their premorbid level. The diffuse difficulties were unrelated to tumor type or location, and were not explicable by existing focal deficits, psychotic or depressive thought disorders, metabolic difficulties, or hydrocephalus. These examinations explained in part why these patients failed to resume active social lives or premorbid employment. The diffuse cortical dysfunction was most notable on the Category Test, Trails B, and Localization component of the Tactual Performance Test.

Intracranial hemorrhage with amphetamine abuse

Abstract: Intracranial hemorrhage (ICH) occurred in a drug abuser soon after self-administration of amphetamine. Other reported cases indicate a consistent clinical picture, sometimes fatal or causing permanent neurologic disability.

Amphetamine induces depletion of dopamine and loss of dopamine uptake sites in caudate.

Abstract: Long-lasting depletion of dopamine and concomitant loss of dopamine uptake sites follow repeated administration of methylamphetamine to rats We found similar effects after similar treatment with d-amphetamine, but not after treatment with methylphenidate Methylphenidate also failed to produce long-term depletion of regional catecholamine levels in rhesus monkeys These long-lasting alterations of the dopaminergic system suggest that amphetamines or their metabolites have toxic interactions with dopaminergic neurons, which do not occur with methylphenidate

ALS in Rochester, Minnesota, 1925–1977

Abstract: The incidence, trend, and survivorship of ALS in the population of Rochester, Minnesota, was determined for the years 1925 through 1977. The average annual incidence was 1.76 per 100,000 population. There was a small but nonsignificant increase in the rate during the 53-year period. The male:female ratio was 1.1, and the male:female ratio of incidence rates was 1.6. The median age at onset was 66 years; the incidence rates increased with increasing age. Median survivorship was 22.5 months, and was longer for younger patients than for patients with an advanced age at onset. The demographic characteristics in this total community experience are believed to reflect the pattern of ALS more accurately than previously reported clinical series.