Neurology
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Comparative Study Clinical Trial Controlled Clinical Trial
Initial chemotherapy in gliomatosis cerebri.
Because of the diffuse nature of gliomatosis cerebri (GC), surgery is not suitable, and large field radiotherapy carries the risk of severe toxicity. In this setting, initial chemotherapy warrants further investigation. ⋯ Initial chemotherapy is useful for some patients with gliomatosis cerebri. Temozolomide is well tolerated and appears to be a valuable alternative to procarbazine-CCNU-vincristine, especially for those with slow-growing, low-grade GC.
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The optimal therapy for gliomatosis cerebri is unclear, and the rate of response to chemotherapy is not known. Eleven radiotherapy-naive patients received a median number of 10 treatment cycles of temozolomide. An objective response was documented in 45%, and the median time to tumor progression was 13 months with a progression-free survival of 55% at 12 months. These results indicate that radiotherapy to extensive brain regions can be deferred until progressive disease is observed.
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Biography Historical Article
Americo Negrette (1924 to 2003): diagnosing Huntington disease in Venezuela.
To elucidate the role of Dr. Americo Negrette in diagnosing and reclassifying the dancing mania in Maracaibo, Venezuela as Huntington disease (HD). ⋯ The "dancing mania" of Maracaibo, because of the work of Americo Negrette, has been reclassified as Huntington disease.
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Multicenter Study
Development and initial validation of a screening tool for Parkinson disease surgical candidates.
As there is currently no standardized assessment tool for evaluating Parkinson disease (PD) patients for deep brain stimulation (DBS), the authors developed the Florida Surgical Questionnaire for Parkinson Disease (FLASQ-PD). Part I of the study was a retrospective analysis of 174 patients presenting for a surgical screening. Part II was a multicenter study to assess the correlation of FLASQ-PD scores. The results of this study suggest that the FLASQ-PD may be a useful triage tool for screening PD patients for DBS surgery.
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Review
Copper deficiency myelopathy produces a clinical picture like subacute combined degeneration.
Copper deficiency in ruminants is known to cause an ataxic myelopathy. Copper deficiency as a cause of progressive myelopathy in adults is underrecognized. ⋯ Unrecognized copper deficiency appears to be a common cause of idiopathic myelopathy in adults. The clinical picture bears striking similarities to the syndrome of subacute combined degeneration associated with vitamin B12 deficiency. Early recognition and copper supplementation may prevent neurologic deterioration.