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Biomedical subjects

U Gallenkamp

Publications and source records attributed to U Gallenkamp.

13 recordsLinked to original sources

Diagnosis and treatment in a case of juvenile subacute necrotizing encephalopathy Leigh without cytochrome c oxidase deficiency.

Subacute necrotizing encephalopathy (Leigh syndrome) is characterized by lactacidosis, seizures, ataxia, multiple cerebral hypervascularized lesions and mitochondrial oxidation defects. This is a report on a 21-year-old patient with proven Leigh syndrome, mild central and provokable peripheral lactacidosis, an extra-erythrocyte complex II defect, functionally reduced myokinase adenylate deaminase activity, but no ultrastructural mitochondrial changes. Determination of lactate, pyruvate and ammonia under ischemic conditions plus a pyruvate loading test were particularly useful. Oral flunarizine (Sibelium 30 mg/d) proved to be therapeutically effective.

Biopsy↗

Juvenile onset diabetes mellitus, central diabetes insipidus and optic atrophy (Wolfram syndrome)--neurological findings and prognostic implications.

The authors report on one case of Wolfram syndrome, a rare condition, which is characterized by juvenile onset diabetes mellitus, diabetes insipidus, optic atrophy and sensorineural deafness. The findings of this 13-year follow-up show that this patient developed typical neurological complications of long-standing diabetes mellitus as in the common type 1 variant. Moreover, some peculiar signs occurred such as anosmia, ophthalmoplegia interna, and central nystagmus. Since Wolfram syndrome is probably part of a more generalized neurodegenerative disorder, long-term prognosis will depend both upon the severity of chronic diabetic complications and upon the rapidity, by which degeneration of cerebellar, pontine and brain stem structures appear. Prognosis of the cardinal clinical signs is such that optic atrophy, though usually quite rapid in the beginning, generally does not lead to complete blindness. Sensorineural hearing loss progresses very slowly so that deafness might be expected exceptionally only. The hearing deficit in classical diabetics, however, is of retrocochlear origin. Therefore, in Wolfram syndrome, a combined inner-ear and retrocochlear hearing loss may occur.

Adolescent↗

Bradycardia due to biperiden.

In a 38-year-old male patient suffering from a severe postzosteric trigeminal neuralgia, intravenous application of 10 mg biperiden lactate led to a long-lasting paradoxical reaction characterized by considerable bradycardia, dysarthria, and dysphagia. The heart rate was back to normal within 12 hours upon administration of orciprenaline under cardiac monitoring in an intensive care unit. Bradycardia induced by biperiden is attributed to the speed of injection and to a dose-related dual effect of atropine-like drugs on muscarine receptors.

Adult↗

[Cerebral seizures, alcoholism, and deliria (author's transl)].

The occurrence of cerebral seizures in alcoholics was investigated in case histories of 84 delirious and nondelirious male patients. Eighteen patients had seizures before they became alcoholics; the frequency of the seizures increased during abuse. Twelve had no deliria at all up to the moment of this investigation; in 66 of the patients the occurrence of seizures was assumed to be caused by alcohol abuse alone. Seventy-one patients had seizures irrespective of deliria and nearly 40% of them had no deliria at all. In 21% we observed only deliria with seizures; in 16% only deliria without seizures; and 24% had deliria both with and without seizures. The remaining 13 patients of 84 had only deliria complicated by cerebral seizures; only 3 had deliria without seizures. The seizures occurred as grand mal in 94% of the alcoholics, in all patients with genuine epilepsy, and in 60% of the patients with post-traumatic epilepsy.

Adult↗

[Reversible alterations of peripheral nerve function in thyroid dysfunction (author's transl)].

In 7 patients with hyperthyroidism and 9 patients with hypothyroidism both thve before treatment and after a period of normal thyroid function of at least one year duration. As far as conduction velocity and relative refractory period are concerned there was no difference between hyperthyroid patients and normal persons, whereas in cases of hypothyroidism before treatment the conduction velocity was shown to be significantly reduced; moreover a prolonged relative refractory period and a decline in amplitude of the nerve action potential were found. These alterations of peripheral nerve function, interpreted as evidence of neuropathy, proved to be reversible when the thyroid function returned to normal.

Action Potentials↗