Biomaterial Database

Cerebellar degeneration and Meige's syndrome. 1991

J S Khara, and V P Calabrese

Department of Neurology, Medical College of Virginia, Richmond.

We have reported a case of Meige's syndrome in a middle-aged man who eventually had a cerebellar degeneration syndrome. The extrapyramidal symptoms preceded cerebellar signs and symptoms by 5 years. Most patients with idiopathic Meige's syndrome show some improvement with high-dose anticholinergic therapy. Our patient's lack of response to such agents and his subsequent cerebellar symptoms are reminiscent of the situation seen with parkinsonian patients who do not respond to medications, indicating a more widespread degenerative disease. The association of extrapyramidal symptoms with some spinocerebellar disorders, and the pathologic changes seen in the one reported autopsy case, should place the group of spinocerebellar disorders high on the differential list.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008538	Meige Syndrome	A syndrome characterized by orofacial DYSTONIA; including BLEPHAROSPASM; forceful jaw opening; lip retraction; platysma muscle spasm; and tongue protrusion. It primarily affects older adults, with an incidence peak in the seventh decade of life. (From Adams et al., Principles of Neurology, 6th ed, p108)	Blepharospasm-Oromandibular Dystonia,Brueghel Syndrome,Idiopathic Orofacial Dyskinesia,Blepharospasm-Oromandibular Dyskinesia,Blepharospasm-Oromandibular Dystonia Syndrome,Blepharospasm-Oromandibular Dystonia Syndrome, Idiopathic,Idiopathic Blepharospasm-Oromandibular Dystonia Syndrome,Syndrome, Blepharospasm-Oromandibular Dystonia,Blepharospasm Oromandibular Dyskinesia,Blepharospasm Oromandibular Dystonia,Blepharospasm Oromandibular Dystonia Syndrome,Blepharospasm Oromandibular Dystonia Syndrome, Idiopathic,Blepharospasm-Oromandibular Dyskinesias,Blepharospasm-Oromandibular Dystonia Syndromes,Blepharospasm-Oromandibular Dystonias,Dyskinesia, Blepharospasm-Oromandibular,Dyskinesia, Idiopathic Orofacial,Dyskinesias, Blepharospasm-Oromandibular,Dyskinesias, Idiopathic Orofacial,Dystonia Syndrome, Blepharospasm-Oromandibular,Dystonia Syndromes, Blepharospasm-Oromandibular,Dystonia, Blepharospasm-Oromandibular,Dystonias, Blepharospasm-Oromandibular,Idiopathic Blepharospasm Oromandibular Dystonia Syndrome,Idiopathic Orofacial Dyskinesias,Orofacial Dyskinesia, Idiopathic,Orofacial Dyskinesias, Idiopathic
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D011859	Radiography	Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film).	Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013132	Spinocerebellar Degenerations	A heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. Sporadic and inherited subtypes occur. Inheritance patterns include autosomal dominant, autosomal recessive, and X-linked.	Ataxias, Hereditary,Cerebellar Ataxia, Early Onset,Cerebellar Ataxia, Late Onset,Cerebellar Degenerations, Primary,Corticostriatal-Spinal Degeneration,Marie Cerebellar Ataxia,Marinesco-Sjogren Syndrome,Spinocerebellar Degeneration,Early Onset Cerebellar Ataxia,Familial Spinocerebellar Degenerations,Garland-Moorhouse Syndrome,Hereditary Oligophrenic Cerebello-Lental Degeneration,Hereditary Spinocerebellar Degenerations,Inherited Spinocerebellar Degenerations,Late Onset Cerebellar Ataxia,Marie's Cerebellar Ataxia,Marinesco-Garland Syndrome,Marinesco-Sjogren Syndrome-Hypergonadotrophic Hypogonadism,Marinesco-Sjogren Syndrome-Myopathy,Marinesco-Sjogren-Garland Syndrome,Marinesco-Sjögren Syndrome,Spino Cerebellar Degenerations,Spino-Cerebellar Degenerations,Spinocerebellar Diseases,Ataxia, Hereditary,Cerebellar Ataxia, Marie,Cerebellar Ataxia, Marie's,Cerebellar Degeneration, Primary,Corticostriatal Spinal Degeneration,Corticostriatal-Spinal Degenerations,Degeneration, Corticostriatal-Spinal,Degeneration, Familial Spinocerebellar,Degeneration, Hereditary Spinocerebellar,Degeneration, Inherited Spinocerebellar,Degeneration, Primary Cerebellar,Degeneration, Spino Cerebellar,Degeneration, Spino-Cerebellar,Degeneration, Spinocerebellar,Degenerations, Corticostriatal-Spinal,Degenerations, Familial Spinocerebellar,Degenerations, Hereditary Spinocerebellar,Degenerations, Inherited Spinocerebellar,Degenerations, Primary Cerebellar,Degenerations, Spino Cerebellar,Degenerations, Spinocerebellar,Familial Spinocerebellar Degeneration,Garland Moorhouse Syndrome,Hereditary Ataxia,Hereditary Ataxias,Hereditary Oligophrenic Cerebello Lental Degeneration,Hereditary Spinocerebellar Degeneration,Hypogonadism, Marinesco-Sjogren Syndrome-Hypergonadotrophic,Inherited Spinocerebellar Degeneration,Marinesco Garland Syndrome,Marinesco Sjogren Garland Syndrome,Marinesco Sjogren Syndrome,Marinesco Sjogren Syndrome Hypergonadotrophic Hypogonadism,Marinesco Sjogren Syndrome Myopathy,Marinesco Sjögren Syndrome,Primary Cerebellar Degeneration,Primary Cerebellar Degenerations,Spino Cerebellar Degeneration,Spino-Cerebellar Degeneration,Spinocerebellar Degeneration, Familial,Spinocerebellar Degeneration, Hereditary,Spinocerebellar Degeneration, Inherited,Spinocerebellar Degenerations, Familial,Spinocerebellar Degenerations, Hereditary,Spinocerebellar Degenerations, Inherited,Spinocerebellar Disease,Syndrome, Garland-Moorhouse,Syndrome, Marinesco-Garland,Syndrome, Marinesco-Sjogren,Syndrome, Marinesco-Sjogren-Garland,Syndrome, Marinesco-Sjögren,Syndrome-Hypergonadotrophic Hypogonadism, Marinesco-Sjogren,Syndrome-Myopathy, Marinesco-Sjogren