Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case of a young woman with focal dystonia of the hand due to a cavernous angioma of the basal ganglia is presented. The lesion involved the anterior third of the lentiform nucleus and a large portion of white matter anterior to this nucleus and lateral to the head of the caudate, as shown by magnetic resonance imaging; it was completely removed through a computed tomography-assisted stereotactic craniotomy by microsurgical technique, resulting in the cure of the patient. These facts support the pathophysiological hypothesis of a disruption of the striatopallidothalamic projection to the premotor cortex as the cause of symptomatic dystonia. A review of the reported cases of cavernous angiomas of the deep cerebral gray nuclei shows that this is the first case of cavernous angioma associated with movement disorder.
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PMID:Focal dystonia secondary to cavernous angioma of the basal ganglia: case report and review of the literature. 832 84

On four occasions since 1978, this 53 year-old woman presented with a right hemicorporal hypotonia, symptomatic of a hemispheric cerebellar syndrome. In 1981, she experienced the progressive development of a cervical dystonia. CT scan and RM scan showed a cavernous angioma in the right cerebellar hemisphere. The 18F-2-fluoro-2-deoxy-glucose PET scan revealed a right cerebellar and a contralateral cortical and striatal hypometabolism. This crossed cerebello-cortical diaschisis can be interpreted as a functional interruption of the cerebello-cerebral pathways. This case raises the question of the role played by a cerebellar lesion in the development of a focal dystonia.
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PMID:[Cerebellar cavernous angioma, cervical dystonia and crossed cortical diaschisis]. 196 70

Secondary dystonias and experimental models of dystonia suggest that mechanisms responsible for primary dystonias may lie in the basal ganglia or brainstem. A histological study has been done in three patients with cranial dystonia (blepharospasm with oromandibular dystonia in two, blepharospasm alone in one), and one patient with craniocervical dystonia (oromandibular dystonia with retrocollis). In the patient with blepharospasm alone, an angioma, 0.5 mm in diameter, was found in the dorsal pons at the site of the central tegmental tract, confirming that some patients presenting with primary dystonias may have longstanding lesions in the brainstem. In the three other cases, the striatum, pallidum, thalamus, and brainstem were examined and cell populations in the putamen, substantia nigra, and inferior olives were compared with age-matched controls, but no significant abnormality was found.
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PMID:Pathological report of four patients presenting with cranial dystonias. 319 61

Dystonia is a neurologic syndrome whose etiology is not well recognized. We report a sixteen year old patient with difficulty in playing piano, with disability of the left hand, forearm and arm. Short after, she underwent "absence" crises and headache. Neuroradiological exams showed a deep temporal lobe cavernous angioma. It is a rare association between both diseases and there are no reports about segmental dystonia associated to pseudoabsence epilepsy and sporadic migraine secondary to cavernous angioma. Twenty five per cent of dystonias have a secondary cause and we suggest a neuroradiologic investigation.
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PMID:[Segmental dystonia as manifestation of cavernous angioma: case report]. 1158 47

We describe a 68 year-old Chinese man who presented with segmental dystonia involving the neck and right upper limb, which was exacerbated with action. EEG and MRI of the cervical spine were unremarkable, but MRI of the brain revealed a small cavernous angioma measuring one centimeter in diameter in the left centrum semiovale. The dystonia was ameliorated but not abolished with trihexyphenidyl. Arteriovenous malformations have been described to cause movement disorders, but dystonia has not previously been reported in association with cavernous angiomas. A search of the literature revealed one report of a patient with chorea arising from a cavernous angioma. This interesting case illustrates how an uncomplicated cavernous angioma can rarely cause segmental dystonia and highlights the importance of neuroimaging in patients with late-onset movement disorders.
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PMID:Symptomatic segmental dystonia from a cavernous angioma in the centrum semiovale. 1561 65

Stereotatic radiosurgery is typically not the first line of treatment for cervical dystonia. We present a patient with a rare cervical dystonia induced by a venous angioma in the right basal ganglion. The patient was successfully treated with a gamma knife thalamotomy after failed treatments of botulinum toxin injections and peripheral denervation.
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PMID:Successful treatment of cervical dystonia induced by basal ganglion venous angioma with gamma knife thalamotomy. 2227 59