Myopathy as possible side-effect of cyclosporin.
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Adult↗
Biomedical subjects
T Kuntzer
Publications and source records attributed to T Kuntzer.
Reversible emetine-induced myopathy with ECG abnormalities: a toxic myopathy.
A young anorexic woman is described with a history of progressive muscle weakness following chronic ingestion of syrup of ipecac that was used in an attempt at weight control. Electrocardiogram (ECG) showed T-wave inversion in all leads and prolongation of the Q-T interval. Electromyography was abnormal. Muscle biopsy revealed a randomized, generalized, predominantly type-2 fibre atrophy and structural alterations in oxidative enzyme stains, such as targetoid and moth-eaten fibres. A few necrotic cells were actively phagocytosed. Electron microscopy revealed severe sarcomeric abnormalities with Z-line streaming, myofibrillar disorganization and increased lysosomal activity. After discontinuation of ipecac syrup, the patient noted a gradual improvement in her condition. The ECG became normal. Emetine and related drug-induced myopathies are uncommon but are of experimental interest because of their effects on mitochondria and the light they shed on autophagic mechanisms in muscle.
Adult↗
[Lipid myopathy: a heterogenic familial case].
A case is reported of lipid storage myopathy in a 24-year-old patient and her family. In the patient and an aunt, muscle biopsy disclosed intrafibrillar lipid depositions, and electron microscopy revealed lipid vesicles in the sarcolemma border. In the father, no lipid depositions were observed but electron microscopy showed alterations to mitochondria compatible with a mitochondrial myopathy. In the patient muscular biochemistry revealed a major reduction in NADH oxydase activity and in the aunt a diminished level of carnitin compatible with carnitin deficiency. The heterogeneity of these lipidic myopathies is discussed.
Adult↗
[Benign encephalitis of the brain stem].
Seven cases of an encephalitic brainstem syndrome are reported. All had an initial flu-like phase, with a febrile state. Then, all had eye movements disturbances and ataxia. Six had a variable dysfunction of other cranial nerves, five had dysmetria of limbs and three had pyramidal signs. In all, the topographic diagnosis was based on clinical, neurophysiological (electromyography, evoked potentials) and neuroradiological (swelled brainstem on CT scan) findings. Analysis of cerebro-spinal fluid revealed pleocytosis (mean; 165 cells/ml), without profile indicative of multiple sclerosis. Serologic tests for Adenovirus or Echovirus were positive in three cases. All recovered spontaneously. With a mean follow-up of three years there was no relapse and the clinical examination remained normal. These seven cases had, after exclusion of other brainstem syndromes, a Bickerstaff's encephalitis. Previous reports are discussed and a post-infectious origin is proposed to this benign syndrome.
Adult↗
[Supratentorial intracranial arachnoid cysts].
20 cases of supratentorial arachnoid cysts are reported. Their classification according to the clinical symptomatology allows to separate them in 3 categories: those presenting with intracranial hypertension and/or a focal neurological deficit (7 cases), those presenting with headache (4 cases) or epilepsy (4 cases), those which were found incidentally and where asymptomatic (5 cases). The neuropsychological examination showed that 5 cysts of the left sylvian fissure had some memory deficit in the verbal modality, but remarkably none was found to have signs of aphasia on detailed testing. Indications for surgical treatment are discussed considering the various clinical groups, the results of neuropsychological evaluation and the neuroradiological findings.
Adolescent↗
[Conduction blocks and peripheral neuropathies].
The conduction block (CB) of the nerve impulses in the peripheral nervous system induces a partial or complete loss of the nerve function, usually more marked on the motor fibers. The CB is due to a focal loss of the electrical properties of the nerve fibers. The underlying mechanisms include paranodal and segmental demyelination, and physical or chemical dysfunction of the ionic channels. The electrophysiological study allows distinction of axonal lesion from dysfunction due to CB. Recognition and quantification of the CB requires criteria that are not yet widely accepted. The different modalities of the examination are detailed and the criteria are discussed. The peripheral neuropathies with CB have different aetiologies; physical (electrical, thermal, percussion, compression or constriction injuries, radiation-induced), toxic (acting either on the axon or the myelin), more rarely ischaemic (arteriopathy or vasculitis), but frequently inflammatory (acute or chronic, primary or secondary) or related to dysimmune states (multifocal sensory-motor and motor neuropathies with persistent CB). Their review allows a discussion of their differential diagnosis in relation to the underlying mechanisms, site and duration (transient to persistent) of the CB.
Animals↗