A retrospective analysis of 30 patients with chronic myeIomonocytic leukemia (CrMML) was performed to define the natural history of the disease and the risk of acute transformation. Our patients fulfilled the following criteria of diagnosis: blood monocytosis over 1 x lo9/], blast cell percentage in
The clinical features and prognosis of chronic posthypoxic myoclonus
β Scribed by Dr. K. J. Werhahn; Dr. P. Brown; P. D. Thompson; C. D. Marsden
- Publisher
- John Wiley and Sons
- Year
- 1997
- Tongue
- English
- Weight
- 432 KB
- Volume
- 12
- Category
- Article
- ISSN
- 0885-3185
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β¦ Synopsis
Abstract
The clinical and neurophysiologic features of 14 patients with chronic posthypoxic myoclonus are presented. Patients were first seen a mean of 2.5 years (range, 2 to 105 months) after the hypoxic event and followed up for 3.7 years (range, 7 to 84 months) thereafter. All patients had had a cardiorespiratory arrest, most caused by an acute asthmatic attack (11 cases). All patients had multifocal action myoclonus. Eleven patients had additional stimulusβsensitive myoclonus. There was late improvement in the myoclonic syndrome and the level of disability in all but one patient. Three patients were eventually able to discontinue antimyoclonic medication, and five patients were able to walk unaided. Cognitive deficits were found in seven patients and were usually mild. Other neurologic deficits were rare. Electrophysiologic investigation confirmed cortical action myoclonus in every case, although this could be combined with cortical reflex myoclonus, an exaggerated startle response, or brainstem reticular reflex myoclonus. We conclude that posthypoxic myoclonus typically consists of multifocal cortical action myoclonus that improves with time. It is only rarely associated with severe additional neurologic deficit.
π SIMILAR VOLUMES
Many cases of myoclonus-dystonia (M-D) are caused by mutations in the epsilon-sarcoglycan (SGCE) gene. We describe 3 children with a similar clinical picture of autosomal dominant M-D and an SGCE mutation in only one of them, suggesting that M-D is genetically heterogeneous.
## Abstract We report on a patient with spontaneous and stimulusβsensitive myoclonic jerks and dystonia of the right leg that had been present since infancy. Magnetic resonance imaging showed a linear area of gliosis confined to the left posterolateral putamen. This is the first report of focal myo