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)

Subacute sensory ataxic neuronopathy is a well-known form of paraneoplastic syndrome. Most sensory neuronopathies are associated with small cell lung cancer and anti-Hu antibodies, and usually show only slight improvement with immunotherapy. To date, there have been few reports of neuropathy associated with thymoma and no treatment strategy has been established for thymoma-related neuropathy. Here, we provide the first report of a case of sensory ataxic neuronopathy with thymoma that showed marked improvement after steroid therapy, even though preceding intravenous immunoglobulin treatments and tumor resection were less effective. A 57-year-old Japanese man was referred to our hospital with a 6-week history of distal paresthesia in his four limbs and an unsteady gait. He presented with left-dominant ataxia in his four limbs due to reduced sensation in his extremities. He also complained of constipation, difficulty urinating, and erectile dysfunction. Upon investigation, including electrodiagnostic studies, the patient was diagnosed as having sensory ataxic neuronopathy with invasive thymoma. A first round of intravenous immunoglobulin therapy, a following thymectomy, and a second round of intravenous immunoglobulin therapy after the surgery were not effective in treating his neurological symptoms. Subsequently, oral steroid therapy was started, which brought about a remarkable improvement; 6 weeks after the beginning of the steroid therapy, his neurological symptoms were resolved, except for slight distal paresthesia in his feet. Although rarely reported, thymoma can underlie sensory neuronopathy, and the response of thymoma-associated sensory neuronopathy to immunotherapy might be better than that of anti-Hu antibody-related neuropathies. Even if the first immunotherapy is not effective in treating neuropathy with thymoma, further immunomodulatory treatment should be tried after treating the tumor.
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PMID:Subacute Sensory Ataxic Neuronopathy With Thymoma Presenting Marked Improvement After Steroid Therapy. 3094 23

A known case of hypertension and recent onset diabetes presented to our neurological clinic with symptoms of ataxia, rigidity, and tremors. His symptoms were of relatively recent onset. He gave no history of any renal disease in past. The magnetic resonance imaging of the brain done by the neurologist was suggestive of demyelinating pathology. His renal functions showed progressive deterioration (Cr 1.4 mg/dl about 3 months back to 2.2 mg/dl at present) along with the onset of his neurological illness. An extensive work up for autoimmune encephalitis and paraneoplastic syndrome was noncontributory. A toxicology screen revealed high levels of aluminum in the blood. A renal biopsy showed features of interstitial nephritis and predominant vacuolar injury of the proximal tubule (suggestive of toxic injury.) On further questioning, the patient gave history of using an over the counter native medication. The medication was stopped and weekly desferrioxime chelation advised. A short course of steroids (0.5 mg/kg/day tapering dose for 6 weeks) was also given. The creatinine stabilized to 1.3 mg/dl on follow-up after 3 months. The neurological symptoms also resolved completely.
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PMID:Acute Interstitial Nephritis in a Patient with High Aluminum Blood Levels: A Case Report. 3201


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