Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Guillain-Mollaret triangle comprises the ipsilateral red nucleus in the midbrain, the inferior olive in the medulla and the contralateral dentate nucleus in the cerebellum: together, these form the dentato-rubro-olivary pathway. Pathology in this triangle disinhibits (and so activates) the inferior olivary nucleus. The olivary nucleus then hypertrophies and its rhythmical discharges may manifest clinically as oculopalatal tremor. We describe three cases with either oculopalatal tremor or MRI evidence of olivary hypertrophy caused by vascular insults to this triangle. It is not clear why only some patients have the oculopalatal tremor. Olivary hypertrophy can be confused with demyelination if the imaging is not put into clinical context. Oculopalatal tremor may occur without olivary hypertrophy since the nucleus atrophies with time. Oculopalatal tremor does not respond to medical treatment. A better understanding of the mechanism of the discharge at a cellular level may lead to more targeted medical treatments.
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PMID:The Guillain-Mollaret triangle in action. 2674 Mar 79

Olivary hypertrophy (OH) is the secondary degeneration of the inferior olivary nucleus (ION). It is observed one month after the onset of a primary lesion within the dento-rubro-olivary pathway and is usually associated with oculopalatal tremors. Here, we report two unique cases with rare autoimmune diseases leading to OH development with progressive cerebellar ataxia, both of which improved with steroid treatment. The first patient was a 59-year-old man with slowly progressive dysarthria and ataxic gait without palatal tremor. Anti-N-methyl-d-aspartate (NMDA) receptor antibody was positive in the CSF, supporting a diagnosis of anti-NMDA receptor encephalitis. The second patient was a 56-year-old man who developed dysarthria, ataxia, gait disturbance, and palatal tremor. He was diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), based on presence of a punctate contrast-enhancing lesion in the middle cerebellar peduncle, pons, and cerebellum on magnetic resonance imaging (MRI). Brain MRI in both patients demonstrated high signal intensity regions in the bilateral IONs. Semi-quantitative volume analysis of MRI revealed significant reduction in ION volume after steroid treatment and accordingly cerebellar ataxia was improved in both cases. Clinical and radiological features of the two cases were unique, indicating potential novel etiologies in the pathophysiology of OH associated with cerebellar ataxia.
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PMID:Olivary hypertrophy improved by steroid treatment: Two case reports with unique presentations. 3130 54