Biomedical subjects
J C Brust
Publications and source records attributed to J C Brust.
Hypoglycemia: causes, neurological manifestations, and outcome.
During a 12-month prospective study there were 125 visits to the Harlem Hospital Emergency Room for symptomatic hypoglycemia. Sixty-five patients had obtundation, stupor, or coma; 38 had confusion or bizarre behavior; 10 were dizzy or tremulous; 9 had had seizures; and 3 had suffered sudden hemiparesis. Diabetes mellitus, alcoholism, and sepsis, alone or in combination, accounted for 90% of predisposing conditions; others included fasting, terminal cancer, gastroenteritis, insulin abuse, and myxedema. Average blood glucose levels were lower among comatose than among obtunded patients, but overlap was considerable, and overall there was little correlation among cause, blood glucose levels, and symptoms. Although mortality was 11%, only one death was attributable to hypoglycemia per se, and only four survivors had focal neurological residua.
Peripheral nerve involvement in sarcoidosis: an electrodiagnostic study.
The frequency of peripheral nerve involvement in sarcoidosis is uncertain. To determine how often peripheral nerves are affected in the absence of symptoms, electromyography was performed on 29 sarcoid patients and 29 age-matched controls. Nineteen sarcoid patients, compared to two controls, had abnormally low sensory amplitudes in one or more nerves, and the mean sensory amplitudes for median, ulnar, and sural nerves were lower in the patient than in the controls. Subclinical mononeuropathy multiplex appears to be common in sarcoidosis.
Nonspecificity of ring enhancement in "medically cured" brain abscess.
Since the introduction of computed tomography (CT), there have been numerous reports of brain abscess treated successfully without surgery. Because pathologic confirmation was lacking in these patients, diagnosis was based on CT abnormalities, usually ring enhancement. However, our recent clinical experience and the experimental work of others indicate that the "ring sign" on contrast-enhanced CT is not absolutely diagnostic of encapsulated brain abscess. Moreover, in the reported cases of alleged brain abscess cured medically, atypical clinical features suggest that some patients may have had cerebral infarction or cerebritis. Stricter clinical and radiologic criteria are needed before concluding that encapsulated brain abscess can be cured by medical therapy alone.
Anton's syndrome accompanying withdrawal hallucinosis in a blind alcoholic.
During alcohol withdrawal, a man totally blind following ocular trauma experienced formed visual hallucinations ("alcoholic hallucinosis"), the unreality of which he recognized. During these same periods, however, he believed that his vision had been restored, and confabulated descriptions of his surroundings (Anton's syndrome). He was otherwise mentally normal. Anosognosia for blindness can occur episodically or chronically, and with either peripheral or central visual loss; dementia nearly always coexists. The basis of the syndrome is obscure, and its occurrence during alcohol withdrawal, in association with readily acknowledged visual hallucinations and otherwise preserved mentation, has not been previously reported.
Delayed onset of post-rabies vaccination encephalitis.
A 31-year-old veterinarian developed seizures, left hemiparesis, loss of memory, and behavioral disorders 5 months after intensive antirabies vaccination, the longest incubation period yet recorded. Computed tomographic scan revealed a right frontal contrastenhanced mass that extended to the left frontal lobe through the corpus callosum. Brain biopsy showed foci of primary demyelination largely confined to the white matter. The lesions were characteristic of the demyelinating encephalomyelitis that follows treatment with certain vaccines against rabies. The hemiplegia improved, but seizures, memory impairment, and abnormal behavior persisted.
The nonimpact of opiate research on opiate abuse.
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Dementia and cerebrovascular disease.
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Subarachnoid hemorrhage: early detection and diagnosis.
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Hypertensive encephalopathy and the neurologic manifestations of malignant hypertension.
In a prospective study, 34 patients with 41 admissions for malignant hypertension were examined by a neurologist before and after antihypertensive treatment. Neurologic examination was normal in 17 cases, and neurologic diagnoses in 19 others included acute cerebral infarction (10), uremic encephalopathy (4), dementia of uncertain etiology (3), multiple infarct dementia (1), and chronic paranoid schizophrenia (1). Five patients had a generalized neurologic syndrome consistent with hypertensive encephalopathy, characterized by altered mental function with or without generalized convulsions. In these patients, neurologic examination was normal 1 to 7 days after lowering of blood pressure, while renal function remained unchanged. Neurologic disease is therefore common in malignant hypertension, and hypertensive encephalopathy may be more frequent than previously described.
Language disorder in a right-hander after occlusion of the right anterior cerebral artery.
A right-handed woman developed left hemiparesis and a language disturbance. At autopsy, there was infarction in the territory of the right anterior cerebral artery, involving, among other structures, the supplementary motor area. This brain region has been considered to play a role in speech, but whether the language disorder that follows its destruction is truly aphasic is controversial. Our patient does not answer that question, but if her disturbed language is viewed as aphasic, she represents the fourth autopsy case of "crossed aphasia in a dextral" and the first, with or without autopsy, after right anterior cerebral artery occlusion.
Subcortical neglect.
Unilateral neglect is usually caused by lesions of the right cerebral cortex, especially parietal. Neglect after subcortical lesions has been reported rarely in thalamic or striatal hemorrhage, and after infarction of thalamus or striatum and internal capsule shown by CT. In these patients, there may have been compression or ischemia of overlying cerebral cortex and white matter. We report a patient with sensory hemineglect, asomatognosia, and anosognosia caused by infarction of striatum and deep white matter, with involvement of neither cortex nor thalamus and without mass effect. This is the first autopsy-confirmed example of human neglect with involvement of only the striatum and deep white matter.
Intracranial subdural sarcoid granuloma. Case report.
A patient is reported in whom a subdural sarcoid granuloma caused symptoms of an intracranial mass lesion, and disappeared following steroid therapy. Twenty-three previous cases with large intracranial sarcoid granulomas have been documented, a review of these 24 cases leads the authors to conclude that: 1) neither symptoms nor nonhistological diagnostic studies, including computerized tomography, differentiate sarcoid from cerebral neoplasms or other central nervous granulomas; and 2) because sarcoid mass lesions frequently respond well to corticosteroid therapy, surgery should probably be reserved for cases in which there is diagnostic uncertainty, a need for emergency decompression, or lack of response to steroids.
Failure of CT sharing in a large municipal hospital.
Harlem Hospital Center lacks computed tomography (CT) facilities. During 1979 it attempted to share the CT facilities of two other institutions. Retrospective analysis reveals that under this arrangement only a small percentage of Harlem patients needing CT obtained it. Among patients recommended for CT, 252 of 1528 without trauma and six of 342 with trauma received it. In some instances there were difficulties in moving patients between hospitals. If transportation had been optimal, however, the other hospitals, because of their own needs, would have been unable to provide Harlem Hospital with more than a third of the CT scans requested. Furthermore, even if unlimited opportunities for scanning had been available, patients whose need for CT seemed most urgent--the very ill--would have been unable to receive it; of 163 patients who died without receiving CT as recommended, 93 were too sick or deteriorating too rapidly to be moved to the CT facilities. We conclude that CT cannot be effectively shared by large acute-care hospitals.
Sarcoidosis presenting as senile dementia.
Cerebral sarcoidosis was found at autography in a 68-year-old woman with progressive dementia. Of 35 previously reported cases of central nervous system sarcoidosis with dementia, only 1 was over 65 years old, and in only 2 was the presenting clinical syndrome that of a degenerative dementia. Other unusual features of the index case include the restriction of the initial cognitive deficit to memory loss and mild anomia, the scarcity of antemortem evidence for systemic sarcoidosis, a positive tuberculin test, a cerebrospinal fluid (CSF) protein concentration as high as 2028 mg per deciliter. sarcoidosis is a rare but potentially treatable cause of dementia. Consistently normal CSF probably excludes the diagnosis.
Spastic paraplegia-paraparesis. A reappraisal.
During a 16-year period 672 patients were admitted to the Columbia Presbyterian Medical Center with spastic paraplegia-paraparesis (SPP) as a prominent finding. Group I. 520 patients (77.3%) had either a familial disorder, sensory findings, other neurological signs, or a diagnosis obvious on admission by history, examination or plain spine roentgenograms. Group II. In 108 patients (16.2%) diagnosis was reached after more extensive investigation. In 78 of these a cause other than multiple sclerosis was found including spinal cord tumor, arteriovenous malformation and cervical spondylotic myelopathy. Thirteen patients had probable multiple sclerosis on the basis of additional signs emerging, and 17 were considered to have possible multiple sclerosis on the basis of elevated CSF gamma-globulin. Group III. In 44 patients (6.5%) no cause for SPP could be found. Diagnostic considerations for this group included amyotrophic lateral sclerosis, multiple sclerosis, unappreciated structural lesions, hereditary disease, and "primary lateral sclerosis".
Music and language: musical alexia and agraphia.
Two aphasic right-handed professional musicians with left hemispheric lesions had disturbed musical function, especially musical alexia and agraphia. In Case 1 aphasia was of transcortical sensory type, with severe agraphia and decreased comprehension of written words, although she could match them with pictures. Except for reading and writing, musical ability was normal; she could sing in five languages. Musical alexia and agraphia affected pitch symbols more than rhythm. Case 2 had conduction aphasia and severe expressive amusia, especially for rhythm. Although his language alexia and agraphia were milder than Case 1's, his musical alexia and agraphia were more severe, affecting rhythm as much as pitch. In neither patient were those aspects of musical notation either closest to verbal language or most dependent upon temporal (sequential) processing maximally impaired. These cases are consistent with the literature in suggesting that the presence or absence of aphasia or of right or left hemispheric damage fails to predict the presence, type, or severity of amusia, including musical alexia and agraphia. The popular notion that receptive amusia follows lesions of the language-dominant temporal lobe, whereas expressive amusia follows non-dominant frontal lobe damage, is an over-simplification, as is the view that increasing musical sophistication causes a shift of musical processing from the right hemisphere to the left.
Presumably Azorean disease in a presumably non-Portuguese family.
Autosomal dominant motor system degeneration (ADMSD) is a hereditary ataxia that has been reported previously only in Portuguese families from the Azores Islands. Cerebellar ataxia, pyramidal and extrapyramidal signs, amyotrophy, dystonia, abnormal eye movements, and prominent eyes are variably present. Four members of a family had cerebellar ataxia, dystonic posturing, a variety of abnormal eye movements, and prominent eyes resulting from lid retraction. Eight other family members had a similar disease. The combination of cerebellar ataxia, dystonia, abnormal eye movements, and prominent eyes has been reported only in ADMSD. The family reported here may be the first example of ADMSD in a non-Portuguese family.