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Biomedical subjects

A Krumholz

Publications and source records attributed to A Krumholz.

At least 37 records · Page 2Linked to original sources

The treatment of neurosarcoidosis with cyclosporine.

Six patients with refractory neurosarcoidosis were enrolled in a 12-month open-label trial to investigate the safety and efficacy of cyclosporine therapy. Patients were stabilized on a corticosteroid dose, randomized to a low-dose or high-dose cyclosporine group (with appropriate target whole blood cyclosporine levels) for 6 months, and assessed by prospectively defined studies. The corticosteroid dose was adjusted as clinically tolerated. We found that the corticosteroid dose could be lowered to 30% to 58% of the initial stabilization dose in conjunction with cyclosporine therapy, at the time of maximal clinical and laboratory improvement. However, four patients deteriorated while using corticosteroids and cyclosporine; one of these patients died. At the time of clinical deterioration, the prednisone dose ranged from 6 to 22.5 mg daily (or the equivalent). No serious toxic effects developed from cyclosporine therapy. Cyclosporine treatment is a reasonably safe and effective adjunct to corticosteroid therapy for patients with refractory neurosarcoidosis, although clinical deterioration can occur despite combination therapy.

Adult↗

Driving and epilepsy. A review and reappraisal.

Driving and epilepsy is a problem that involves physicians as both care providers to patients and consultants to regulatory authorities. Driving restrictions for people with seizure disorders are intended to ensure the public's safety, but such restrictions may unduly harm the welfare of many people with seizures. In the United States, all states now permit some people with epilepsy to drive. In general, only people whose seizures are adequately controlled are licensed to drive. Adequate control has been judged principally by the seizure-free interval, but individual state standards widely vary. There is a trend toward greater liberalization of driving standards for people with seizure disorders, but the appropriateness and application of these standards continue to raise questions, as does the role physicians should have in the licensing process. Our responsibilities to persons with disabilities and advances in our understanding of seizures and the nature of driving risks warrant a reappraisal of the current medical, legal, and social implications of driving and epilepsy.

Accidents, Traffic↗

Clinical implications of seizures in neurosarcoidosis.

Seizures are a recognized manifestation of neurosarcoidosis, but their clinical relevance is not well established. We reviewed the characteristics, clinical correlations, and prognosis of seizures in 79 patients with neurosarcoidosis. Thirteen (15%) of the 79 patients had seizures, and in eight patients (10%) a seizure was the first manifestation of neurosarcoidosis. These seizures were generalized tonic-clonic seizures in 12 patients (92%) and partial seizures in four patients (31%). The patients with neurosarcoidosis with seizures were more likely to have a progressive or relapsing clinical course and intracranial mass lesions (four patients [31%]), encephalopathy or vasculopathy (eight patients [62%]), or hydrocephalus (five patients [38%]). These central nervous system disorders, rather than the seizures per se, were responsible for most of the serious morbidity and the two deaths (15%) among our patients with seizures. Indeed seizure control was good in 11 (85%) of 13 patients treated with combinations of steroids and antiepileptic medications. Seizures are an important sign in neurosarcoidosis because they are associated with more severe and progressive or relapsing forms of central nervous system sarcoidosis and may be an early manifestation of such disorders.

Adolescent↗

Seizures following myelography with iopamidol.

Iopamidol, a water-soluble contrast medium, has been rarely associated with seizures. We describe 3 patients (from a series of 785) who had generalized tonic-clonic seizures after iopamidol myelography. Two of the patients underwent lumbar and one cervical myelography. There was a history of seizures in 2 patients, and the dose of iopamidol used in the patients who convulsed was high, ranging from 3,000 to 4,500 mg iodine. Despite the reported low incidence of complications with iopamidol, seizures may occur, especially in patients with previous seizures and also in those receiving higher doses of iopamidol.

Female↗

Failure of high-dose intravenous magnesium sulfate to control myoclonic status epilepticus.

An unsuccessful attempt to terminate myoclonic status epilepticus with elevation of serum magnesium levels is described. During 3 days, serum magnesium was increased from 1.5 mEq/L to 14.2 mEq/L by continuous i.v. infusion of 3-6 g/h of magnesium sulfate. Other anticonvulsants were maintained at nearly constant levels. Cerebrospinal fluid magnesium was 3.5 mEq/L during the infusion. Despite magnesium-related neuromuscular blockade and accompanying cessation of visible myoclonus, the electroencephalogram revealed ongoing blunted sharp-wave activity at the baseline frequency. Transient complications of the infusion included prolongation of the PR interval on the electrocardiogram, hypomotility of the gastrointestinal tract, and peripheral muscle flaccidity, all of which resolved within 24 h of return to normal serum magnesium levels. These results suggest that the therapeutic role of magnesium in myoclonic status epilepticus is limited.

Adult↗

Outcome from coma after cardiopulmonary resuscitation: relation to seizures and myoclonus.

We studied the effect of seizures and myoclonus following cardiopulmonary resuscitation (CPR) on the outcome of all comatose adult survivors of CPR over an 8-year period. Either seizures or myoclonus occurred in 50 of 114 patients (44%): seizures in 41 patients (36%) and myoclonus in 40 (35%). Status epilepticus or status myoclonus occurred in 36 patients (32%), and 19 (17%) had myoclonic status epilepticus (MSE). Seizures and myoclonus per se were not significantly related to outcome, but status epilepticus, status myoclonus, and, particularly, MSE were predictive of poor outcome as judged by survival and recovery of consciousness.

Adult↗

Neurosarcoidosis: the long-term clinical course.

We reviewed 25 patients with neurosarcoidosis followed for at least 5 years or until death. Seventeen patients (68%) had a monophasic illness. Eight (32%) had a relapsing course, with recurrences similar to the original manifestations months later. Patients with cerebral manifestations or hydrocephalus were most likely to relapse. Seven of eight patients with relapsing disease had exacerbations while taking 10 mg prednisone or less.

Cranial Nerve Diseases↗

Neurosarcoidosis: cerebrospinal fluid lymphocyte subpopulations.

CSF lymphocyte subpopulations of eight patients with neurosarcoidosis were examined. CSF or CT was abnormal in all. The CSF T4/T8 (helper/suppressor) ratio was elevated at 6.8 and 7.6 in two patients; in one, there were only CSF T4 cells. The ratio was normal in five patients.

Adult↗

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↗

Prolonged confusion due to absence status following metrizamide myelography.

A case of absence status epilepticus resulted from metrizamide myelography. The acute confusional state following metrizamide myelography is briefly reviewed and compared with the confusional state resulting from adult-onset absence status. The similarities suggest that absence status may in fact be a relatively common cause of prolonged confusion following the use of metrizamide. Given the difficulty in clinical diagnosis and the excellent response to anticonvulsants, the possibility of absence status should be specifically excluded by EEG in any person suffering prolonged confusion following metrizamide myelography.

Aged↗

Maturation of the brain-stem auditory evoked potential in premature infants.

Patterns of development and extent of variability of the brain-stem auditory evoked potential (BAEP) are described in 52 healthy premature infants and 50 normal term newborns with reliable conceptional ages. Binaural and monaural stimulation are compared. Serial studies of individual prematures are emphasized and demonstrate that the most consistent and least variable measures are the monaurally derived interpeak intervals. All the BAEP parameters studied mature including wave form, relative amplitude, peak and interpeak intervals, but there is far more variability in preterms than in term infants or adults, even when infants are carefully matched for conceptional age. As term is approached, BAEP variability decreases substantially, suggesting that near term the BAEP becomes a more reliable indicator of neurologic function. The variability of the BAEP in the normal preterm limits its usefulness in determining neurologic dysfunction in individual high risk infants. Nevertheless, the BAEP may still prove useful for defining group differences among infants and could provide an objective measure of those factors influencing neurologic development. Serial change in the BAEP is a specific parameter which we believe merits further study in premature infants as an index of neurologic maturation.

Brain Stem↗

Hearing screening of high-risk newborns with brainstem auditory evoked potentials: a follow-up study.

Numerous techniques have been used in attempts to find a reliable and efficient screening method for determining auditory function in the newborn. The brainstem auditory evoked potential (BAEP) is the latest method advocated for that purpose. The BAEP was evaluated as a hearing screening test in 168 high-risk newborns between 35 and 45 weeks of conceptual age. Follow-up data were obtained after 1 year (mean 17.3 months) on 134 of the infants (80%). Normal hearing was defined as a reproducible response in both ears to a 25 dB normal hearing level (nHL) click stimulus; 21 infants (12.5%) failed the initial screening test. Follow-up on 19/21 infants revealed 18 infants with normal hearing and one infant with an 80 dB nHL bilateral hearing loss substantiated. One infant with an abnormal screening test died before retesting, and the other infant was lost to follow-up but had only a unilaterally abnormal BAEP. None of the infants with a normal BAEP screening study had evidence of hearing loss on retesting. Sensitivity of the BAEP was 100%, specificity was 86%, predictive value of a positive test was 5.26%, and the predictive value of a negative test was 100%. The incidence of significant hearing loss in our population was between 0.75% (1/134 infants) confirmed, and 2.24% (3/134 infants) including infants who failed screening but were lost to follow-up. The BAEP is a sensitive procedure for the early identification of hearing-impaired newborns. However, the yield of significant hearing abnormalities was less than predicted in other studies using BAEP for newborn hearing screening.

Brain Stem↗

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↗