Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Vitamin E is one of the most important lipid-soluble antioxidant nutrient. Severe vitamin E deficiency (VED) can have a profound effect on the central nervous system. VED causes ataxia and peripheral neuropathy that resembles Friedreich's ataxia. We report here a patient presenting this syndrome, but also a prolactin and FSH adenoma. Both the neurological syndromes and the adenoma regressed after treatment with alpha-tocopherol. Although, the presence of the prolactinoma in this patient may not be related to his vitamin E deficiency, alpha-tocopherol treatment seems to be beneficial and might usefully be tested in patients with hypophyseal secreting other forms of adenoma.
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PMID:Vitamin E deficiency ataxia associated with adenoma. 1006 78

The authors report the clinical features of and imaging studies on a rare case of pediatric malignant prolactinoma. A 12-year-old boy presented with ataxia, blurred vision, and consciousness disturbance. He had received transcranial surgery and adjuvant radiotherapy. However, the tumor regrew with craniospinal metastasis 16 months after the operation. The relevant literature was reviewed regarding the clinical presentation, pathogenesis, treatment approaches, and prognosis of malignant prolactinoma.
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PMID:Malignant prolactinoma with craniospinal metastasis in a 12-year-old boy. 1862 81

Superficial siderosis of the central nervous system (CNS) is a rare disorder caused by deposition of hemosiderin in neuronal tissue in the subpial layer of the CNS due to slow subarachnoid or intraventricular hemorrhage. The most common neurologic manifestations include progressive gait ataxia, sensorineural hearing loss, and corticospinal tract signs. We present a case of superficial siderosis in a 43-year-old man who presented to the Emergency Department with sudden onset bilateral visual deterioration and a loss of consciousness. A hemorrhagic giant prolactinoma was diagnosed based on brain CT scan, T1-weighted MRI, and an endocrine blood examination. Susceptibility-weighted non-contrast MRI showed pathognomonic signs of superficial siderosis in the form of a hypointensity rim surrounding the brainstem, cerebellar fissures, and cranial nerves VII and VIII. This report demonstrates that superficial siderosis can be caused by pituitary apoplexy.
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PMID:Superficial siderosis of the central nervous system due to chronic hemorrhage from a giant invasive prolactinoma. 2366 7