The Intersection Between Cerebellar Ataxia and Neuropathy: a Proposed Classification and a Diagnostic Approach.

Neuropathy is a common associated feature of different types of genetic or sporadic cerebellar ataxias. The pattern of peripheral nerve involvement and its associated clinical features can be an invaluable aspect for narrowing the etiologic diagnosis in the investigation of cerebellar ataxias. In this review, we discuss the differential diagnosis of the intersection between peripheral nerve and cerebellar involvement, and classify them in accordance with the predominant features. Genetics, clinical features, neuroimaging, and neurophysiologic characteristics are discussed. Furthermore, a diagnostic approach for cerebellar ataxia with neuropathy is proposed according to the different clinical characteristics. This is an Educational and Descriptive review with the aim of medical education for the approach to the patients with cerebellar ataxia and neuropathy. The diagnostic approach to the patient with cerebellar ataxia with neuropathy requires a detailed medical history, phenotyping, characterization of disease progression and family history. Neuroimaging features and the neurophysiological findings play pivotal roles in defining the diagnosis. Establishing an organized classification method for the disorders based on the clinical features may be very helpful, and could be divided as those with predominant cerebellar features, predominant neuropathic feature, or conditions with both cerebellar ataxia and neuropathy. Second, determining the mode of inheritance is critical on cerebellar ataxias: autosomal dominant and recessive cerebellar ataxias, mitochondrial or sporadic types. Third, one must carefully assess neurophysiologic findings in order to better characterize the predominant pattern of involvement: damage location, mechanism of lesion (axonal or demyelinating), motor, sensory or sensory motor compromise, large or small fibers, and autonomic system abnormalities.

Análise comparativa da avaliação da lordose lombar aferida na radiografia em ortostase e na ressonância magnética / Comparative analysis of assessment in computed radiography lumbar lordosis measuredn while standing and magnetic resonance imaging

Objetivo: Comparar os ângulos encontrados na radiografia em ortostase e na ressonância magnética (RM) em decúbito dorsal com coxim sobre os membros inferiores. Métodos: Estudo prospectivo, de caráter descritivo, no qual se avaliou uma amostra de 100 pacientes, sendo 51 do sexo masculino, em que o grau de lordose lombar foi aferido por três examinadores independentes por meio da radiografia em ortostase e da RM em decúbito, com coxim, nos níveis de L1 a L5, de acordo com o método de Cobb. Resultados: A média dos valores encontrados na radiografia e na RM com coxim de L1 a L5 foi de 42,2º e 31,5º, respectivamente (p < 0,001), demonstrando que a angulação da lordose lombar aferida na RM subdimensiona a verdadeira angulação em 25%. Conclusão: A RM é insuficiente para a avaliação da lordose lombar, sendo de extrema importância a avaliação radiológica para avaliar a verdadeira angulação da lordose lombar.

Objective: To compare the angles found in radiography and magnetic resonance imaging (MRI) orthostatic supine with a pad on the lower limbs. Methods: Measurement of lumbar lordosis angles from 100 patients referred to MRI and computed radiography studies, using Cobb?s method (with L1and L5 plateaus as reference). Measurements were done by three independent observers with different skills, and interobserver agreement was evaluated. The lumbar lordosis angle obtained in computedradiography and MRI were compared using statistical analysis with paired t-test. Results: The mean lordosis angles measured in computed radiography and MRI were respectively 42.2º and 31.5º (p < 0.001). Conclusion: There was good interobserver agreement. There was a statistically significantdifference between the angles of lumbar lordosis in computed radiography and MRI. The data suggest that MRI is not reliable in the evaluation of the lumbar lordosis when used alone.