The authors report a case of sensorimotor polyneuropathy, diffuse cutaneous hyperpigmentation, skin sclerodermiform thickening and papular lesions in the infraclavicular and abdominal region. Besides weight loss, there were diabetes mellitus and hypothyroidism. The alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Cutaneous alterations are present in 68% of patients with diffuse cutaneous hyperpigmentation, plethora and acrocyanosis. Leukonychia, necrotizing vasculitis, hypertrichosis and cutaneous thickening of sclerodermiform type are also cited. The onset of multiple cutaneous angiomas in this syndrome has been observed in 24-44% of patients.

We report on a 14 y/o HIV-negative girl with diagnosis of miliary tuberculosis (meningitis and pulmonary disease) who presented with right hemichorea three weeks after initiation of prednisone, rifampin, isoniazide, pyrazinamide and ethambutol. Brain MRI showed ischemic lesions in left caudate and lenticulo-capsular area (Figure). Hemichorea is a rare neurological manifestation of tuberculosis 1. Basal ganglia infarcts caused by vasculitis probably decreased the GABAergic transmission in indirect basal ganglion pathway causing hemichorea. The major differential diagnoses in acquired hemichorea are Sydenham's chorea, nonketotic hyperglycemia and cerebral toxoplasmosis 2. We emphasize the importance of brain MRI in the diagnostic evaluation of these patients.

RESUMOA Síndrome de Tolosa Hunt é uma doença rara, cuja etiopatogenia é desconhecida. Apresenta-se como uma oftalmoplegia dolorosa de um ou mais nervos cranianos oculomotores, que regride espontaneamente e responde bem ao tratamento com corticoides. O presente estudo trata-se de um relato de caso de um paciente que apresentou seguidos casos de oftalmoplegias dolorosas, envolvendo o nervo oculomotor e o abducente sendo tratado com corticoesteroides obteve uma resposta dramática. Objetiva-se ainda descrever as características fisiopatológicas, clínicas, o diagnóstico diferencial, visto que é um diagnóstico de exclusão, e medidas terapêuticas instituídas de acordo com o International Headache Society 2004 (ISH-2004 através da apresentação do caso clínico conduzido com as normas do estudo supracitado.Descritores: Síndrome de Tolosa-Hunt/diagnóstico; Oftalmoplegia/quimioterapia; Prednisona/uso terapêutico; Dor/classificação; Classificação Internacional de Doenças; Relato de casos

A 69-year-old woman was admitted for investigation of an acute-onset right hemichorea 1. T1-weighted brain magnetic resonance imaging (MRI) showed hyperintensity in the left basal ganglia (Figs 1A and B). Although diabetic, she had no hyperglycemia. Interestingly, two weeks earlier, the patient was admitted due to nonketotic hyperglycemia. Brain computed tomography, previously reported as normal (Fig 1C), showed subtle