Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System

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iSS can be associated with significant neurotologic and cerebellar morbidity; the recurrent SAH variant is frequently clinically symptomatic, has a shorter latency and greater neurotologic disability. In these cases, a thorough search and management of a covert source of bleeding may stop clinical progression. The frequency and clinical course of radiographic iSS after traumatic and post-aneurysmal SAH is largely unknown. Detection of radiographic iSS after trauma or aneurysm bleeding suggests that the slower clinical course could benefit from an effective intervention if it became available. The use of cochlear implants is a valid alternative with advanced hearing impairment.

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Effects of Acetyl-DL-Leucine on Cerebellar Ataxia (Study Protocol)

Cranial Nerves

Effects of acetyl-DL-leucine on cerebellar ataxia (ALCAT trial): study protocol for a multicenter, multinational, randomized, double-blind, placebo-controlled, crossover phase III trial Katharina Feil1,2,11* , Christine Adrion3, Julian Teufel1,2, Sylvia Bösch8, Jens Claassen5, Ilaria Giordano7, Holger Hengel6, Heike Jacobi7, Thomas Klockgether7, Thomas Klopstock1,4,12, Wolfgang Nachbauer8, Ludger Schöls6, Claudia Stendel1,4, Ellen Uslar5, Bart van de Warrenburg9, Ingrid Berger2, Ivonne Naumann2, Otmar Bayer2, Hans-Helge Müller10, Ulrich Mansmann3 and Michael Strupp1,2 Background: Cerebellar ataxia (CA) is a frequent and often disabling condition that impairs motor functioning and impacts on quality of life (QoL). No medication has yet been proven effective for the symptomatic or even causative treatment of hereditary or non-hereditary, non-acquired CA. So far, the only treatment recommendation is physiotherapy. Therefore, new therapeutic options are needed. Based on three observational studies, the primary objective of the acetyl-DL-leucine on ataxia (ALCAT) trial is to examine the efficacy and tolerability of a symptomatic therapy with

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Effects of acetyl-DL-leucine in patients with cerebellar ataxia

No existing medication has yet been shown to
convincingly improve cerebellar ataxia. Therefore, the
identification of new drugs for its symptomatic treatment is
desirable. The objective of this case series was to evaluate
the efficacy of treatment of cerebellar ataxia with the
amino acid acetyl-DL-leucine (Tanganil). Thirteen patients
(eight males, median age 51 years) with degenerative
cerebellar ataxia of different etiologies (SCA1/2, ADCA,
AOA, SAOA) were treated with acetyl-DL-leucine (5 g/
day) without titration for 1 week.

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