Publicación Oficial de la Sociedad Ecuatoriana de Neurología, de la Liga Ecuatoriana Contra la Epilepsia y de la Sociedad Iberoamericana de Enfermedad Cerebrovascular

ataxia

 

Encefalitis autoinmune por anticuerpos anti Iglon5, reporte de caso clínico. Autoinmune encephalitis due to anti Iglon5 antibodies, clinical case report

Introduction: New clinical entities related to the discovery of autoantibodies either against intracellular antigens or against cell membrane surface receptors or synapses are being identified with increasing frequency. In the latter group, patients with apnea have been described in recent years, REM and NON-REM sleep behavior disorder, respiratory failure and presence in serum and CSF of antibodies against the LON5 antigen. These antibodies appear to alter the interaction with the cytoskeletal system of the neuron and induce accumulation of TAU, conditioning a wide clinical expression.

Clinical case: 76-year-old male patient with eight years evolution of symptoms before diagnosis. He had a mixture of respiratory symptoms, parasomnias, ataxia, movement disorder, autonomic symptoms, cognitive and mood disorder, and associated a chronic demyelinating polyneuropathy. The diagnosis was confirmed by detection of antibodies against IGLON5 in cerebrospinal fluid.

Conclusion: Anti-IGLON5 antibody encephalitis is a unique entity that challenges what we know about autoimmunity and neurodegeneration, leading to a diagnostic challenge due to the wide expression of symptoms and the possibility of improvement with early treatment.

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Palabras clave: Ataxia, encefalitis, IgLON5 proteína humana, parasomnias, polirradiculoneuropatía inflamatoria desmielinizante crónica, proteína tau,

Keywords: ataxia, chronic inflammatory demyelinating polyradiculoneuropathy, encephalitis, IgLON5 human protein, parasomnias, tau protein,

CLIPPERS (Inflamación crónica linfocítica con realce perivascular pontino y respuesta a esteroides) con compromiso longitudinalmente extenso de la médula espinal. Una presentación atípica. CLIPPERS (Chronic lymphocytic inflammation with pontine perivascular enhancement and steroid response) with longitudinally extensive spinal cord involvement. An atypical presentation

CLIPPERS (Chronic Lymphocytic Inflammation with Perivascular Pontine Enhancement and Steroid Response) is a recently described neuroinflammatory disorder in which symptoms involving the brainstem predominate, with variable pattern on neuroimaging and a perivascular infiltrate of T lymphocytes on brain biopsy. We present a case of a 50-year-old male who presented symptoms of asthenia, adynamia, weakness in lower limbs extremities, sphincter disorder and ataxia. Patient was extensively studied to rule out infectious, neoplastic, autoimmune, and demyelinating conditions. Diagnosis was made taking into account significant clinical-imaging elements, remarking the spinal cord involvement in this patient. Treatment with steroids and steroid-sparing agents, such as methotrexate, was found to be effective. Very few cases in the literature to date describe CLIPPERS-associated spinal cord involvement.

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Palabras clave: Ataxia, CLIPPERS, médula espinal, metrotexato (DeCS), tallo cerebral,

Keywords: ataxia, brainstem, CLIPPERS, methotrexate (MeSH)s, spinal cord,

Síndrome de Kinsbourne: Papel de Rituximab. Kinsbourne Syndrome: Role of Rituximab.

Kinsbourne syndrome, also known as “Opsoclonus-myoclonus syndrome” or “Dancing eyes syndrome” is a rare condition characterized by rapid, irregular, multi-directional eye movements (opsoclonus), myoclonic movements in the trunk, face and/or limbs and ataxia. It occurs in children aged between 6 and 36 months. Its etiology may be paraneoplasic (neuroblastoma), nonparaneoplasic (infectious processes) or idiopathic. Regardless of its etiology, immunosuppressive drugs have been used in order to reduce the formation of antibodies possibly involved in the pathophysiology. We report the case of a four years-old boy with this syndrome secondary to an infectious urinary illness. He had ataxia, opsoclonus, upper limbs myoclonus, irritability and altered sleep pattern. Neuroblastoma was ruled out. Initial management was done with methylprednisolone pulses, followed by oral prednisolone and intravenous gammaglobulin. He started with rituximab with a good response. He had progressive clinical improvement, and is currently asymptomatic with no sequelae. In these patients a paraneoplasic syndrome should always be ruled out.

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Palabras clave: Ataxia, infección, Kinsbourne, mioclonus, Opsoclonus, Síndrome,

Keywords: ataxia, Infection, Kinsbourne, Myoclonus, Opsoclonus, Syndrome,

Sistema de actividades para la compensación de la memoria de trabajo en adultos con ataxia por trauma craneoencefálico.

Introduction: Working memory or operational memory is considered a distinct element of the executive function. Objective: To propose a system of activities that will contribute to the compensation of working memory in adults with Ataxia by craneoencephalic trauma assisted in CIREN.

Material and methods: A pre experiment with a single group was carried out, initial tests were applied to determine the cognitive deficits in the amnesic process, before implementing the strategy, and at the end of the intervention, the results were compared to quantify evolutionary development in the work of clearing of memory in these people with special educational needs.

Results: The results of the diagnostic analysis favored the interpretation of the need to implement corrective treatments – countervailing duties taking into account the allocation of the basic mechanisms of working memory to improve the prognosis of evolution of this amnesic skill.

Discussion: A system of activities is proposed as part of considering the diagnosis as an important element in the corrective compensatory work looking for restoration to the highest possible physical, psychological functioning and social adaptation of persons towards an optimal level of social integration.

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Palabras clave: Ataxia, Compensación, Memoria de Trabajo, Sistema de Actividades, Trauma Craneoencefálico,

Keywords: ataxia, Chraneoencephalic Trauma, Compensation, system of activities, working memory,
 
 
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