High-dose piracetam is effective on cerebellar ataxia in patient with cerebellar cortical atrophy

2003 ◽  
Vol 18 (4) ◽  
pp. 457-459 ◽  
Author(s):  
Melih Vural ◽  
Sibel Özekmekçi ◽  
Hülya Apaydin ◽  
Attila Altinel
Author(s):  
P. Trouillas

ABSTRACT:A review concerning the characteristics of the cerebellar serotoninergic system is presented. In rat, cat and oppossum, the perikarya of origin are located in the brain stem raphe nuclei and in other brainstem structures. The projections to the cerebellar layers and deep nuclei include synaptic connections, but also non synaptic terminals, espedaily in a diffuse cortical plexus. Serotoninergic receptors have been described: 5-HT1B in the molecular layer and 5-HT2 in the inferior olive. Serotonin exerts neurophysiological effects on several target cells, directly or indirectly, presynaptically or postsynaptically. A modulatory effect on Purkinje cells is well documented. In thiamine deprived animals, a specific serotoninergic cerebellar syndrome includes a selective degeneration of the serotoninergic cerebellar system, an increase of the 5-HIAA cerebellar values and an exaggerated serotoninergic turnover. In human here-doataxias (Friedreich’s ataxia and cerebellar cortical atrophy), serotoninergic disturbances have been observed in the CSF, including low 5-HIAA values and an increased serotoninergic turnover. Therapeutic results have been obtained with L-5-HTP, a precursor of serotonin, in several conditions presenting cerebellar ataxia. L-5-HTP resistance of olivo-pontocerebellar atrophies may be explained by the destruction of serotonin-sensitive target cells, especially Purkinje cells.


2018 ◽  
Vol 141 (1) ◽  
pp. 19-22
Author(s):  
Liat Shargian-Alon ◽  
Pia Raanani ◽  
Uri Rozovski ◽  
Tali Siegal ◽  
Shlomit Yust-Katz ◽  
...  

Neurologic complications of allogeneic hematopoietic cell transplantation (allo-HCT) include infections, cerebrovascular events, therapy-induced neurotoxicity, recurrent malignancies, and neurologic manifestations of graft-versus-host disease (GVHD). Anti-glutamic acid decarboxylase (GAD) antibody-associated cerebellar ataxia is a well-established disorder of autoimmune origin, but there are no reports in the literature of its occurrence following allo-HCT. We describe a middle-aged woman with chronic GVHD after allo-HCT who presented with a rapidly progressive cerebellar syndrome. Thorough investigation revealed only cerebellar atrophy on brain imaging and positive anti-GAD65 antibodies in serum and cerebrospinal fluid suggesting the diagnosis of anti-GAD antibody-associated cerebellar ataxia. Despite prompt treatment with high-dose corticosteroids, intravenous immunoglobulins, and rituximab, the patient’s condition rapidly deteriorated, and she died 4 months later. This case suggests that anti-GAD antibody-associated cerebellar ataxia may be a rare manifestation of chronic GVHD.


2008 ◽  
Vol 46 (6) ◽  
pp. 439-440 ◽  
Author(s):  
Naomi Kondo ◽  
Nobuyuki Shimozawa ◽  
Junichi Asano ◽  
Atsushi Imamura ◽  
Tadao Orii

Author(s):  
Yuma Sonoda ◽  
Nao Yoshida ◽  
Kazunori Kawami ◽  
Akihiro Kitamura ◽  
Nobuhiro Ogawa ◽  
...  

Purpose The aim of this study was to investigate a structured approach for effective speech therapy (ST) for dysarthria and speech-related quality of life in patients with sporadic spinocerebellar degeneration (SCD), including cerebellar-type multiple-system atrophy and cerebellar cortical atrophy. Method Twenty-two patients with SCD (cerebellar-type multiple system atrophy, 15 patients; cerebellar cortical atrophy, seven patients) who underwent intensive ST were examined. Dysarthria was evaluated using the Scale for Assessment and Rating of Ataxia Speech Dysfunction, Assessment of Motor Speech for Dysarthria Articulation, oral diadochokinesis (OD), and Voice Handicap Index-10 (VHI-10). Respiratory muscle strength (inspiratory and expiratory pressure) and respiratory–phonatory coordination (maximum phonation time) were measured. Cognitive function was evaluated using the Montréal Cognitive Assessment and the word fluency test. Mood was evaluated using the Hospital Anxiety and Depression Scale. The relationships between dysarthria scales (particularly, VHI-10) and clinical data were analyzed using stepwise regression. The differences in outcomes after intensive ST were analyzed using the Wilcoxon signed-rank test. The alpha level ( p ) for statistical significance was set at .0125 by Bonferroni correction. Results For both pre- and post-ST, the patient's OD ( p = .002) and maximum phonation time ( p = .002) significantly improved, except for Speech Dysfunction scores of the Scale for Assessment and Rating of Ataxia ( p = .705) and the VHI-10 ( p = .018). The Assessment of Motor Speech for Dysarthria Articulation, OD, and inspiratory pressure were identified as independent variables of VHI-10 (adjusted R 2 = .820) for speech-related quality of life; no correlations among the Montréal Cognitive Assessment, word fluency test, and Hospital Anxiety and Depression Scale scores were observed. Conclusion OD and VHI-10 showed improvements due to changes in speech function and respiratory–phonatory coordination, justifying intensive ST treatment for dysarthria in patients with SCD.


2011 ◽  
Vol 2011 ◽  
pp. 1-4
Author(s):  
Amer Awad ◽  
Olaf Stüve ◽  
Marlyn Mayo ◽  
Rafeed Alkawadri ◽  
Bachir Estephan

Extrahepatic immunological manifestations of hepatitis C virus (HCV) are well described. In addition, antiglutamic acid decarboxylase (GAD) antibody-associated cerebellar ataxia is well-established entity. However, there have been no reports in the literature of anti-GAD antibody-associated ataxia as an extrahepatic manifestation of HCV infection. We report the case of a young woman with chronic hepatitis C virus and multiple extrahepatic autoimmune diseases including Sjögren syndrome and pernicious anemia who presented with subacute midline cerebellar syndrome and was found to have positive antiglutamic acid decarboxylase (GAD) antibody in the serum and cerebrospinal fluid. An extensive diagnostic workup to rule out neoplastic growths was negative, suggesting the diagnosis of nonparaneoplastic antiglutamic acid decarboxylase antibody-associated cerebellar ataxia as an additional extrahepatic manifestation of hepatitis C virus infection. The patient failed to respond to high-dose steroids and intravenous immunoglobulin. Treatment with the monoclonal antibody rituximab stabilized the disease. We postulate that anti-GAD associated ataxia could be an extrahepatic manifestation of HCV infection.


1999 ◽  
Vol 42 (5) ◽  
pp. 1157-1175 ◽  
Author(s):  
Geralyn M. Schulz ◽  
William O. Dingwall ◽  
Christy L. Ludlow

The purpose of this study was to determine whether cerebellar pathology interferes with motor learning for either speech or novel tasks. Practice effects were contrasted between persons with cerebellar cortical atrophy (CCA) and control participants on previously learned real speech, nonsense speech, and novel nonspeech oral-movement tasks. Studies of limb motor learning suggested that control participants would evidence reduced variability, increased speed of movement, and reduced movement amplitude with practice as compared with the CCA group. No significant differences were found between the real- and nonsense-speech tasks. For both speech tasks, although neither group reduced their movement variability with practice, both groups significantly reduced jaw closing displacement and velocity with practice. For the novel nonspeech oral-movement task, no change with practice was observed in either group in terms of variability, amplitude, or peak velocity. No effects of cerebellar pathology were seen in either the speech- or oral-movement tasks. These results demonstrated that with practice of speech tasks, a previously learned motor skill, movement speed and displacement decreased in both groups. Therefore, the effects of practice differed between previously learned speech tasks and the novel oral-movement task regardless of cerebellar pathology.


2021 ◽  
Vol 14 ◽  
pp. 175628642110628
Author(s):  
Livia Asan ◽  
Stephan Klebe ◽  
Christoph Kleinschnitz ◽  
Mark Stettner ◽  
Martin Köhrmann

We present a case of acute cerebellar ataxia and myoclonus with detected anti-GFAP-antibodies in a patient recently recovered from COVID-19. Main symptoms consisted of acute gait and limb ataxia and myoclonus. The patient improved considerably upon treatment with high-dose intravenous (IV) steroids. While cerebrospinal fluid (CSF) and magnetic resonance imaging (MRI) findings were unremarkable, anti-GFAP-antibodies were detected in the patient’s serum and disappeared upon clinical remission at a 3-month follow-up. This case suggests that anti-GFAP-antibodies might be associated with some of the increasingly observed cases of postinfectious acute cerebellar ataxias in COVID-19 patients and aid in the diagnosis of this autoimmune complication. We recommend searching for these antibodies in serum and CSF in suspected cases. Early steroid treatment may prove beneficial for these patients.


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