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Biomedical subjects

A Krumholz

Publications and source records attributed to A Krumholz.

52 records · Page 3Linked to original sources
PubMed1983

Herpes zoster ophthalmicus and delayed contralateral hemiparesis caused by cerebral angiitis: diagnosis and management approaches.

Four patients with herpes zoster ophthalmicus and delayed contralateral hemiparesis are described, and their findings are compared with those in patients previously reported in the English language literature. The current patients evidenced multifocal ipsilateral cerebral angiitis by angiography and multifocal infarcts in the distribution of the ipsilateral middle cerebral artery by computed tomographic scanning. Cerebrospinal fluid showed mononuclear pleocytosis, positive oligoclonal bands, and an elevated immunoglobulin G index. Two patients were treated with corticosteroids and acyclovir, and 1 with corticosteroids alone, all without apparent response. Necrotizing angiitis ipsilateral to the herpes zoster ophthalmicus was demonstrated postmortem in 1 patient with multifocal cerebral infarction and progressive leukoencephalopathy. Neither herpes varicella zoster immunocytochemical reactivity nor viral inclusions were seen. The leukoencephalopathy associated with herpes varicella zoster either may be caused by cerebral angiitis or, as previously reported, may be a temporally remote manifestation of persistent herpes varicella zoster infection. The cerebral angiitis associated with herpes varicella zoster is histologically similar to granulomatous angiitis, and both may be related to herpes varicella zoster infection of the cerebral vasculature.

Acyclovir↗
PubMed1983

Electrophysiological studies in Tourette's syndrome.

The value of standard electrophysiological studies using electroencephalography and evoked responses was evaluated in patients with Tourette's syndrome. Sixteen-channel electroencephalograms were obtained in 40 patients (36 males, 4 females) awake and asleep, and evoked responses were obtained in a subgroup of 17 patients. Evoked response variables evaluated included latencies and amplitudes of visual evoked responses, brainstem auditory evoked responses, and somatosensory evoked responses to median and peroneal nerve stimulation. Only 5 of the 40 patients (12.5%) demonstrated electroencephalographic abnormalities, which included central spikes, generalized and paroxysmal slow activity, and slowing of the normal basic frequency. Evoked response studies demonstrated no consistent differences between the patients with Tourette's syndrome and age- and sex-matched controls. The data demonstrate no notable diagnostic or therapeutic value for routine electroencephalographic or evoked response studies in Tourette's syndrome.

Adolescent↗
PubMed1983

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

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.

Adolescent↗
PubMed1982

Solitary intracranial plasmacytoma: two patients with extended follow-up.

Solitary neoplastic proliferation of plasma cells (plasmacytoma) rarely occurs in the central nervous system. The longest follow-Up in nine previously reported patients was 5 years. We have followed two patients with solitary intracranial plasmacytoma for 8 and 13 years, respectively. At the time of biopsy diagnosis, neither showed involvement of the adjacent calvarium or evidence of a systemic plasma cell dyscrasia. The first patient underwent partial excision of a mass in the right occipital lobe that showed no gross or microscopic involvement of the dura. The second patient had partial resection of a mass arising from dura and invading the left temporal lobe. Postoperative irradiation was given to both patients. No clinical or radiological sign of recurrent brain tumor has developed in either patient during the extended follow-up period. Neither patient has had laboratory or clinical evidence of a systemic plasma cell dyscrasia. Solitary intracranial plasmacytoma may be a "curable" brain tumor.

Adult↗
PubMed1981

Evoked responses in vitamin B12 deficiency.

Abnormalities of visual, brainstem auditory, and somatosensory evoked responses were demonstrated in two of seven individuals with vitamin B12 deficiency. The evoked response delays correlated directly with the degree of neurological dysfunction. Abnormalities were present in sensory systems without clinical evidence of involvement and were similar to those found in individuals with multiple sclerosis.

Adult↗
PubMed1981

Subacute encephalopathy with seizures in alcoholics: a clinical-electroencephalographic study.

A subacute form of encephalopathy was observed in 7 patients with a history of severe chronic alcoholism. These episodes lasted about 1 to 6 weeks and were characterized by epileptic seizures (grand mal, focal motor), prominent EEG abnormalities with focal features (slowing, spiking, "PLEDs") and neurological deficits such as hemianopsia or hemiparesis. These changes resolved with symptomatic (mainly anticonvulsive) treatment but recurrences occurred in 3 cases. The clinical picture does not fit any of the known CNS complications of chronic alcoholism and there seems to be no relationship to alcohol withdrawal. Impressive EEG abnormalities strongly differ from the mild abnormalities or normal tracings found in other CNS complications of alcoholism. The pathogenetic mechanisms are essentially obscure; vascular changes might play a contributory role in some of the cases.

Adult↗
PubMed1978

Seizures of axial structures. Presumptive evidence for brain stem origin.

Scattered reports, both clinical and experimental, have been accumulating in the past 20 years indicating that true seizures may, indeed, originate from the brain stem and its immediate connections. Four cases are reported that give further strong presumptive evidence that this is so. All the seizures were confined to axial structures (face, tongue, palate, pharynx, diaphragm, and abdomen), and in one case each seizure had an identical "Jacksonian march." The phenomena were documented by 16-mm motion pictures, brain stem signs, and electroencephalography. The EEG consisted of periods of burst activity followed by relative interictal electrocerebral silence.

Abdominal Muscles↗
PubMed1978

A disorder of biogenic amines in dihydropteridine reductase deficiency.

A severe deficiency of dihydropteridine reductase (DHPR) in liver, brain, and cultured skin fibroblasts was demonstrated in a child with hyperphenylalaninemia and an atypical form of phenylketonuria. DHPR is required for regeneration of the cofactor, tetrahydrobiopterin. The cofactor is essential in hydroxylation of aromatic amino acid precursors in the biosynthesis of neurotransmitters, serotonin, dopamine, and norepinephrine. In gray tissue at brain biopsy, dopamine was low at 3 ng per gram of tissue, serotonin was barely detected, and norepinephrine appeared high at 1600 ng per gram. In cerebrospinal fluid, homovanillic acid (HVA) was low normal at 33 ng/ml, 5-hydroxyindoleacetic acid (5-HIAA) was low at 4.2 ng/ml, and after a high dose of oral probenecid there was impaired accumulation of HVA to 128 ng/ml and 5-HIAA to 22.4 ng/ml. When the patient was 22 months of age, treatment with hydroxylated aromatic amino acid precursors was initiated, and after three months HVA and 5-HIAA levels were increased in CSF. The apparent restoration of biogenic amines in brain appears to have delayed the rate of neurological deterioration. DHPR activity in cultured skin fibroblasts of children with persistent hyperphenylalaninemia should permit early diagnosis and early treatment of this disorder.

5-Hydroxytryptophan↗
PubMed1978

Neuroradiological manifestations of intracranial sarcoidosis.

Six cases of sarcoidosis involving the central nervous system are reported with the neuroradiological findings and appearance on computed tomography. Communicating hydrocephalus with sarcoid arachnoiditis is the most common finding, but arteritis and masses have also been reported. Two rare cases of intracerebral masses are included. The radiological findings and clinical histories are reviewed with emphasis on the use of CT and complete neuroradiological studies for patients with possible central nervous system sarcoidosis.

Adult↗
PubMed1975

Phenylketonuria due to a deficiency of dihydropteridine reductase.

The onset of neurologic symptoms in a child who had markedly elevated blood phenylalanine levels during the first two weeks of life and who was promptly treated with a low phenylalanine diet, with excellent control of serum phenylalanine levels, suggested that this child had an unusual form of phenylketonuria. In assays of the components of the phenylalanine hydroxylating system (open liver biopsy at 14 months), the activity of phenylalanine hydroxylase was 20 per cent of the average normal adult value. By contrast, no dihydropteridine reductase activity was detected in the patient's liver, brain or cultured skin fibroblasts. Since dihydropteridine reductase is also essential for the biosynthesis of dopamine, norepinephrine, and serotonin, disturbed neurotransmitter function may be responsible for the patient's neurologic deterioration. On the basis of these results, assay of reductase in cultured skin fibroblasts may be advisable in the initial diagnosis of phenylketonuria.

Biopterins↗
PubMedDate not supplied

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↗
PubMedDate not supplied

Driving and epilepsy: a historical perspective and review of current regulations.

Historically, persons with seizures or epilepsy have been prohibited from driving automobiles or motor vehicles because of concern for public safety. Seizures have a tendency to recur and pose risks of traffic accidents, property damage, and personal injury. In our modern world, however, driving an automobile is such an economic and social necessity that a conflict results between our need to protect public safety and our responsibility to provide reasonable opportunities to drive for persons handicapped by seizures and epilepsy. Currently, there is a trend toward liberalization of driving standards for persons with epilepsy, but there is still considerable controversy regarding the specific driving restrictions necessary for persons with seizures, the way in which such policies should be administered, and the role physicians should have in the process.

Accidents, Traffic↗
PubMedDate not supplied

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↗