PubMed Health⌕ Search

Biomedical subjects

A Michotte

Publications and source records attributed to A Michotte.

32 records · Page 2Linked to original sources
PubMed1992

[Cerebrotendinous xanthomatosis. 2 cases with magnetic resonance imaging].

A 40-year-old woman presented with bilateral juvenile cataract, tendinous xanthomas, intellectual deterioration, spastic tetraparesis, proprioceptive deficit and parkinsonian syndrome. A younger sister's clinical picture differed by the absence of xanthomas and the presence of a cerebellar syndrome. The diagnosis of cerebrotendinous xanthomatosis was confirmed by a high concentration of plasma cholestanol and by urinary chromatography. Magnetic resonance imaging displayed some abnormalities in the hemispheric and cerebellar white matter. Under chenodesoxycholic therapy the biological abnormalities decreased while the clinical disturbances were unchanged.

Achilles Tendon↗
PubMed1991

Frontal impairment and hypoperfusion in neuroacanthocytosis.

Cerebral blood flow tomography, by xenon 133 inhalation or HMPAO (99mTc-d, l-hexamethyl-propylene amine oxime) technetium Tc 99m injection, revealed a severe hypoperfusion in both frontal lobes of a 40-year-old woman with confirmed neuroacanthocytosis. This finding occurred in conjunction with neuropsychological deficits consistent with selective frontal lobe dysfunction. This observation is the first documentation of this type of dementia in neuroacanthocytosis.

Acanthocytes↗
PubMed1991

Focal neurological deficit with sudden onset as the first manifestation of sarcoidosis: a case report with MRI follow-up.

Stroke as a presenting manifestation of sarcoidosis has rarely been reported. This contrasts with the frequent anatomopathological findings of cerebrovascular involvement in neurosarcoidosis. We present a patient who developed acutely a right brachiofacial weakness and dysarthria. Pulmonary sarcoidosis was found. A brain CT and magnetic resonance imaging (MRI) scan disclosed multiple bilateral ischemic, mainly subcortical lesions. Despite a favorable clinical evolution under adequate corticotherapy, an MRI performed 3 months later showed an increased number of the previously observed lesions. This observation suggests that in some cases the evolution of central nervous system sarcoid lesions occurs independently from corticotherapy, and that MRI, in spite of its known great sensitivity in detecting those lesions, may not play a role in the follow-up of some patients with neurosarcoidosis.

Adult↗
PubMed1990

Recurrent brachial plexus neuropathy and giant cell arteritis.

A 73-year-old women presented with a recurrent form of sporadic brachial plexus neuropathy, the so-called Parsonage and Turner syndrome. This diagnosis is based on clinical and electromyographic findings. Interestingly a biopsy of the temporal artery demonstrated a giant cell arteritis. The clinical picture started 2 weeks after an upper respiratory tract illness. The possible viral etiology of giant cell arteritis is considered. We think an immunological rather than ischemic disturbance may have caused the recurrent brachial plexus neuropathy. This case report suggests that giant cell arteritis be considered in the investigation of the Parsonage and Turner syndrome.

Aged↗
PubMed1989

Ataxia as the only delayed neurotoxic manifestation of organophosphate insecticide poisoning.

A patient is reported presenting a cerebellar disorder developing about 5 weeks after acute exposure to an organophosphate insecticide. Initially no major cholinergic features were observed. The ataxia of delayed onset was not accompanied by clinical or electrophysiological signs of polyneuropathy. The possible pathogenetic mechanisms are reviewed and discussed. This case illustrates the need to closely monitor all patients intoxicated with such chemicals for at least 5 weeks. One should not only keep in mind the well-known late-onset polyneuropathy, but also the less frequent delayed central nervous system effects, even in the milder cases where initial signs of acetylcholine excess are only minimally present or lacking.

Ataxia↗
PubMed1988

Recurrent forms of sporadic brachial plexus neuropathy. A report of two cases.

Two patients presenting a relapsing form of sporadic brachial plexus neuropathy, the so-called Parsonage Turner syndrome, are reported. The diagnosis is based on clinical and electromyographic features. Recurrent attacks, although infrequently encountered, have been well described in the past. Sporadic cases of this syndrome must be differentiated from the familial varieties of neuralgic amyotrophy in which, two main subgroups of patients are distinguished: those showing facial dysmorphic features and those with findings of a tomaculous neuropathy predisposing them to pressure palsies. Apart from the obvious difference as regards familial occurrence, the familial and non-familial varieties of neuralgic amyotrophy differ in a number of respects: associated congenital defects, early age of onset and high rate of recurrence in the former. Finally some possible pathogenetic mechanisms of the syndrome are briefly reviewed.

Adult↗
PubMed1986

Brain stem infarction as a complication of giant-cell arteritis.

Two cases of brain stem infarction as an early and fatal complication of giant-cell arteritis are reported. These complications occurred despite adequate treatment with corticosteroids. The findings at autopsy are compared with those of the literature. The possible pathogenetic mechanisms of vertebro basilar occlusion and the therapeutical implications are discussed.

Aged↗
PubMed1985

Unilateral seizures in a patient with hairy cell leukemia treated with interferon.

A patient is described who developed unilateral seizures whilst being treated with recombinant interferon for hairy cell leukemia. Special features included the relatively low dose of interferon, the focal aspect of the epilepsy and the high resistance to anticonvulsants. Oligoclonal banding of cerebrospinal fluid proteins may have resulted from polyclonal activation of bone marrow plasma cells during interferon treatment. Disturbances of consciousness, dysphasia, visual hallucinations, upper motor neuron deficit, tremor, dizziness, numbness, myalgia and headache, all of them neurological complications of interferon treatment, are discussed.

Adult↗
PubMed1977

Oxygen consumption by frog skin and its isolated epithelial layers as a function of their sodium-transporting activity.

The metabolic cost (in terms of oxygen consumption) of transcellular sodium transport was assessed on ventral frog skin and its isolated epithelial layers, by measuring the decrease in oxygen consumption by the tissue upon transient withdrawal of sodium from the outside solution. The same number of sodium ions was transported per molecule oxygen consumed whole skin (17.4 +/- 2.3) and its isolated epithelium (17.3 +/- 2.4). The metabolic cost of sodium transport could not be estimated properly when this process was blocked by amiloride or ouabain, as these drugs were found to bring about an increase in oxygen consumpton by the tissue when no sodium was available for transport.

Amiloride↗
PubMedDate not supplied

Pyruvate dehydrogenase deficiency: clinical and biochemical diagnosis.

A female neonate with pyruvate dehydrogenase (PDH) deficiency is presented with clinical, radiologic, biochemical, neuropathologic, and molecular genetic data. She was dysmorphic, with a high forehead, lowset ears, thin upper lip, upturned nose, and rhizomelic limbs. Cranial MRI revealed severe cortical atrophy, ventricular dilatation, and corpus callosum agenesis. Pyruvate and lactate levels were increased in CSF and blood. Urinary organic acid profile was compatible with PDH deficiency. PDH activity was normal in fibroblasts, lymphocytes, and muscle. The PDH E1-alpha gene was sequenced and a single base mutation was found within the regulatory phosphorylation site in exon 10. It is postulated that this mutation causes a cerebral form of PDH deficiency. Tissue-specific expression of the disease could be explained by differential X chromosome inactivation because the PDH E1-alpha gene is located on this chromosome. Dysmorphism with severe cerebral malformations in female patients merits a metabolic evaluation, including determination of lactate and pyruvate levels in CSF.

Brain↗