Cerebellar ataxia as presenting feature of hypothyroidism

SUMMARY Symptoms and signs of the hypothyroidism vary in relation to the magnitude and acuteness of the thyroid hormone deficiency. The usual clinical features are constipation, fatigue, cold intolerance and weight gain. Rarely it can present with neurologic problems like reversible cerebellar ataxia, dementia, peripheral neuropathy, psychosis and coma. Hypothyroidism should be suspected in all cases of ataxia, as it is easily treatable. A 40 year-old male presented with the history facial puffiness, hoarseness of voice and gait-ataxia. Investigations revealed frank primary hypothyroidism. Anti-TPO antibody was positive. Thyroxine was started and patient improved completely within eight weeks. Hypothyroidism can present with ataxia as presenting feature. Hypothyroidism should be considered in all cases of cerebellar ataxia as it is a reversible cause of ataxia.

Mujeres, adicción y rehabilitación: Reflexiones desde la frontera noroeste de México / Women, addiction and rehabilitation: Reflections from the northwestern border of Mexico

Desde que la adicción adquirió reconocimiento como enfermedad mental, los estudios relativos al tratamiento y modelos terapéuticos han tendido a centrarse en sus dimensiones psiquiátricas y psicológicas. El objetivo de este artículo es destacar la centralidad de la dimensión social, no solo como potencial detonante de comportamientos adictivos y estigmas sobre ellos, sino como variable que permea también su diagnóstico y tratamiento. Las reflexiones parten del trabajo de campo realizado en centros de rehabilitación para mujeres consumidoras de drogas, en la ciudad fronteriza de Tijuana, al noroeste de México. Los resultados arrojan que los modelos terapéuticos predominantes en la ciudad se basan en el rol tradicional de la mujer y lo reproducen. Se analizan las implicaciones sociales de este hecho, y finalmente se sugiere que esos centros podrían ser igualmente aprovechados en el proceso de construcción de una sociedad con mayor equidad de género.

Since the recognition of addiction as a mental illness, studies concerning treatment and therapeutic models have tended to focus on its psychiatric and psychological dimensions. The aim of this article is to highlight the centrality of the social dimension, not only as potential trigger of addictive behaviors and of stigma regarding these behaviors, but also as a variable that permeates diagnosis and treatment. The reflections are based on fieldwork carried out in rehabilitation centers for drug-consuming women in the border city of Tijuana, northwestern Mexico. The results show that the predominant therapeutic models in the city are based in and reproduce the traditional role of women. The social implications of this finding are analyzed, and it is suggested that these centers could be better utilized in the process of building a society with greater gender equality.

Ataxia crónica en pediatría / [Chronic ataxia in childhood].

Chronic ataxias are an heterogeneous group of disorders that affect the child at different ages. Thus, the congenital forms, generally non progressive are observed from first months of life and are expressed by hypotonia and motor delay long before the ataxia became evident. The cerebral magnetic resonance images (MRI) may be diagnostic in some pictures like Joubert syndrome. The group of progressive hereditary ataxias, usually begin after the infant period. The clinical signs are gait instability and ocular apraxia that can be associated with oculocutaneous telangiectasias (ataxia-telangiesctasia) or with sensory neuropathy (Friedreich ataxia). In this review are briefly described congenital ataxias and in more detailed form the progressive hereditary ataxias autosomal recessive, autosomal dominants and mitochondrials. The importance of genetic study is emphasized, because it is the key to obtain the diagnosis in the majority of these diseases. Although now there are no treatments for the majority of progressive hereditary ataxias, some they have like Refsum disease, vitamine E deficiency, Coenzyme Q10 deficiency and others, thus the diagnosis in these cases is even more important. At present the diagnosis of childhood hereditary ataxia not yet treatable is fundamental to obtain suitable handling, determine a precise outcome and to give to the family an opportune genetic counseling.

Early onset cerebellar ataxia with retained tendon reflexes (EOCA) and olivopontocerebellar atrophy (OPCA): a computed tomographic study.

Computed tomographic (CT) studies in olivopontocerebellar atrophies (OPCA) and 'early onset cerebellar ataxia with retained tendon reflexes (EOCA)' are few and vary widely in methodology and criteria for cerebellar and brainstem atrophy. In this prospective study, CT scan observations on 26 patients (EOCA-11, OPCA-15) were compared with 31 controls using qualitative and quantitative assessment of cisterns, ventricles and atrophy of brain. Vermian and/or cerebellar hemispheric (predominantly anterior) atrophy was present in 80.8% and both were equally common. Cerebral cortical atrophy (26.9%) and leukoariosis (15.4%) were less frequently seen. Statistically significant atrophy of pons, brachium pontis, cerebellum and midbrain was noted in patient group. No significant differences were observed between EOCA and OPCA groups. Evidence of atrophy did not correlate with either the duration of illness or the severity of cerebellar ataxia in both the groups. The severity of brainstem atrophy in 14 patients with and 12 patients without abnormal brainstem auditory evoked response did not differ significantly. This study highlights the methodology of CT evaluation for brainstem and cerebellar atrophy, draws attention to cerebral atrophy and emphasizes the lack of significant differences in CT morphology between OPCA and EOCA patients.

Ataxia cerebelosa dominante cubana. Estudios de conduccion de nervios perifericos en pacientes y familiares asintomaticos / Cuban dominant cerebellar ataxia. Peripheral nerve conduction studies in patients and asymptomatic relatives

Se estudiaron 59 familiares de pacientes con Ataxia Cerebelosa Dominante Cubana (SCA2) durante diez años. A todos se les realizó un examen físico para valorar su estado clínico y estudios de conducción en nervios periféricos motores y sensitivos. En 13 de ellos se encontraron manifestaciones clínicas de la enfermedad. En otros 11 familiares se detectaron alteraciones electrofisiológicas en ausencia de síntomas y signos de SCA2. La principal alteración electrofisiológica encontrada fue la reducción en la aplitud de los potenciales sensitivos, lo que es expresión de una lesión axonal que se presenta desde etapas presintomáticas.

Audiçäo central (II): nas lesöes infratentoriais das vias auditivas / Central Hearing (II): in the undertentorial lesions of the auditory pathways

Apesar de conhecidas alteraçöes sobre a emissäo da fala, nas lesöes cerebelares, muito pouco ainda se sabe sobre os efeitos destas alteraçöes e das conexöes do cerebelo na ponte, sobre a inteligibilidade da fala, uma vez que a discriminaçäo de monossílabos é quase sempre normal nestes casos. No presente trabalho, avaliamos a audiçäo periférica e central de seis pacientes semqueixas auditivas, com diagnóstico firmado de Ataxia Cerebelar Autossômica Dominante (AGAD), com graus E1 e E2 de estadio clínico e com atrofia da ponte, quantoàs alteraçöes do VIII nervo craniano, ramo coclear e de suas conexöes, utilizando testes audiométricos convencionais, teste de audiçäo central (SSI - Synthetic Sentences Intelligibility), audiometria de tronco cerebral (BERA) e o Decay do Reflexo do Estapédio. A inteligibilidade da fala para frases curtas (teste SSI) mostrou-se discretamente alterada em alguns pacientes em um ou nos dois ouvidos, apenas quando houve competiçäao ipsilateral, com boa correlaçäo entre este testee as alteraçöes também encontradas para a audiometria de respostas elétricas (BERA). A associaçäo de um exame subjetivo e o BERA mostra melhor a sutileza das lateraçöes de discriminaçäo da fala que acompanham as alteraçöes de vias auditivascentrais infratentoriais, devendo o audiologista e o otorrinolaringologista estarem atentos para que estas, quando presentes, näo passem desapercebidas

Unilateral ataxia following herpes zoster of spinal C4 segment.

Focal lesions of central nervous system are extremely rare following cutaneous herpes zoster. A 55 year old male developed cerebellar speech, right sided ataxia and intention tremor, three weeks after herpes zoster of right spinal C4 segment. Clinical examination and investigations confirmed a focal vascular lesion in the midbrain suggestive of granulomatous angiitis which can cause focal neurological defect after herpes zoster.