Neurosarcoidosis. Presentation and management.
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Adult↗
Biomedical subjects
B J Stern
Publications and source records attributed to B J Stern.
Sarcoidosis and its neurological manifestations.
Neurosarcoidosis is a disorder that is difficult to diagnose and manage. We assessed its neurological manifestations in 649 patients seen at The Johns Hopkins Hospital, Baltimore, from 1975 through 1980. Neurological problems could be attributed to neurosarcoidosis in 33 patients (5.1%). The presenting manifestation of sarcoidosis was neurological in 16 (48%) of them. Cranial neuropathy was the most frequent problem, and a peripheral facial nerve palsy was the single most common abnormality. Other manifestations were aseptic meningitis, hydrocephalus, parenchymatous disease of the central nervous system, peripheral neuropathy, and myopathy. Three-quarters of the patients were treated with steroids. The outcome was good in 27 (82%) of 33 episodes of neurological dysfunction in 25 patients with a well-documented clinical course. A thorough investigation of patients with suspected neurosarcoidosis is recommended to establish the diagnosis, delineate the extent of disease, and guide therapy.
Adult↗
Isolated cerebral phycomycosis presenting as focal encephalitis.
A patient with features of a focal encephalitis was found to have isolated cerebral phycomycosis. No risk factors for phycomycosis were present, and the diagnosis was made by brain biopsy.
Adult↗
Recurrent thyrotoxicosis and papilledema in a patient with communicating hydrocephalus.
A patient with communicating hydrocephalus had recurrent papilledema during episodes of thyrotoxicosis. Increased intracranial pressure was documented and, as reflected by the severity of the papilledema, fluctuated in proportion to the thyroid hormone levels.
Adult↗
Chronic demyelinating polyneuropathy in systemic lupus erythematosus.
Mononeuritis multiplex and acute demyelinating polyneuropathy (Guillain-Barré syndrome) are well known in systemic lupus erythematosus (SLE). Chronic acquired demyelinating polyneuropathy has been reported rarely in SLE. Two young women had monophasic progressive weakness, areflexia, elevated CSF protein, and slow nerve conduction velocities as the first manifestation of SLE. Sural nerve biopsy in one patient revealed mild loss of myelinated fibers and an increased number of thinly myelinated fibers. Steroid therapy led to improvement in both patients. These patients demonstrate that chronic demyelinating polyneuropathy can be an unusual presentation of SLE, even preceding characteristic systemic disease by months.
Adult↗
Regional sharing of CT scanners: experience in a community hospital.
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Aged↗
Neuroleptic malignant syndrome: successful treatment with dantrolene and bromocriptine.
A patient with chronic schizophrenia treated with fluphenazine developed neuroleptic malignant syndrome, characterized by fever, obtundation, rigidity, and tremulousness. Hyperthermia and elevated serum creatine kinase were successfully corrected by parenteral treatment with dantrolene. Obtundation, rigidity, and tremulousness responded to high doses of bromocriptine.
Adult↗
Seizures and seizure care in an emergency department.
We evaluated the nature and significance of seizure problems in an emergency department (ED) by studying seizures in an urban community hospital. In 6 months, there were 29,131 ED visits; of these, 200 (0.7%) were for diagnosed seizures. Among these 200 seizure visits, were 69 (34.5%) new-onset seizures, 30 (15%) febrile seizures, and 92 (46%) seizures in epilepsy patients with prescribed antiepileptic drugs (AEDs). These seizures were often serious and complicated by medical and psychosocial problems; e.g., 37 patients (18.5%) had multiple seizures, 14 (7%) had status epilepticus, and 63 (31.5%) required hospitalization. Associated psychosocial problems included 61 patients (31%) who had no medical insurance, 62 others (31%) who were judged indigent, and 60 (30%) who abused alcohol. Of 92 epilepsy patients receiving AEDs, 52 (56.5%) had subtherapeutic blood levels and were noncompliant with AED prescription patients. Problems with continuity of care were demonstrated by the failure of the ED to communicate with primary care providers about drug levels, noncompliance, and changes in therapy in greater than 85% of patients. A hospital ED is a major source for epilepsy and seizure care, but this care is not always optimum. EDs need to be prepared to manage common acute seizure problems. However, EDs must also place greater emphasis on significant nonemergency aspects of seizure care such as AED compliance, associated psychosocial problems, and effective communication with primary care providers.
Adolescent↗
Evaluation of brainstem stroke using brainstem auditory evoked responses.
Brainstem stroke syndromes are primarily determined by clinical criteria. There are few diagnostic procedures which are of benefit for the evaluation of brainstem ischemic events. Brainstem auditory evoked responses (BAERs) are a new electrophysiologic technique for assessing brainstem function. To evaluate the use of BAERs in patients with brainstem ischemic events, 35 individuals with recent brainstem strokes, selected by strict clinical criteria, were evaluated with BAERs. The initial BAER was abnormal in 22 of 35 patients (63%). When the clinical course and site of the lesion are correlated with the BAER results, several trends emerge. An unstable course, characterized by progression or remission and relapse, was present in 19/35 (54%) of patients, and 15/19 (79%) of these individuals had an initially abnormal BAER. The other 16 brainstem stroke patients with a stable clinical course had an initially abnormal BAER in 7 instances (44%). This difference is statistically significant at the p = 0.04 level. The principal sites of ischemia were mesencephalic in 11/35, pontine in 13/35, and medullary in 11/35. The association of an abnormal BAER with an unstable clinical course seemed independent of the site of the lesion, However, of the 9 deaths that occurred, all were in patients with mesencephalic or pontine lesions, and 8 of these individuals had an initially abnormal BAER. Abnormal BAERs in patients with brainstem ischemic lesions correlate with an unstable clinical course. Furthermore, individuals with pontomesencephalic infarction and abnormal BAERs have an especially poor prognosis. The BAER may be of prognostic value in the early evaluation of patients with brainstem ischemic strokes.
Brain Ischemia↗
Sarcoidosis of the CNS: comparison of unenhanced and enhanced MR images.
Sarcoidosis involving the CNS has a predilection for the leptomeninges, although parenchymal involvement occurs. We retrospectively evaluated the appearance of CNS sarcoidosis on unenhanced and enhanced MR images. MR studies were abnormal in 17 of 20 patients with CNS sarcoidosis. In all 17 patients, meningeal disease was detected on the gadopentetate-dimeglumine-enhanced T1-weighted images; the disease was detected on unenhanced images in three patients. Well-defined leptomeningeal patterns of enhancement were present in 15 patients. Other areas of involvement were the dura; brain parenchyma including hypothalamus, periventricular white matter, and ventricular ependyma; optic chiasm; and pituitary gland. Gadopentetate dimeglumine optimally evaluates meningeal disease and highlights the importance of the leptomeninges and Virchow-Robin spaces in the pathogenesis of CNS sarcoidosis. Enhanced MR is the preferred imaging technique for the evaluation of CNS sarcoidosis.
Adult↗