Systemic sclerosis (SSc) is a connective tissue disease manifest by fibrotic tissue changes, microvascular disease, and autoimmune abnormalities. The prevalence of different neurological manifestations in SSc has ranged from 0.8% 1 to 18.5% 2 according to the adopted criteria. Trigeminal sensory neuropathy (TSN) causes numbness in the mandibular or maxillary divisions of the nerve in about 2/3 of the cases, and in the distribution of all divisions in the remaining cases. The sensory abnormalities evolve slowly and usually spread contralaterally in an asymmetric pattern. The numbness may be accompanied by burning dysesthesia that is distinct from trigeminal neuralgia 3 . TSN is an infrequent complication of SSc. Although epidemiological studies are scarce, the prevalence of TSN associated with SSc in the largest series available was 4% 4 . A fairly diligent review of the literature revealed no previous report of TSN as a complication of SSc in Brazil. We present three cases of such association. CASES Case 1A 42-year-old male had presented progressive facial hypoesthesia and dysesthesia for the last three years and was diagnosed with the diffuse form of SSc two years ago. At his first neurological evaluation, he had sclerodermic fascies, sclerodactyly, Raynaud's phenomenon, bilateral facial and lingual hypoesthesia, left hemifacial dysesthesia, and an absent left corneal reflex. Nailfold capillaroscopy was performed and showed devascularization and ectasias. ANA was positive (1/640) and anti-RNP was also positive. Nerve conduction studies (NCS) revealed sensory myelinic polyneuropathy in all limbs and face. Blink reflex studies (BRS) demonstrated absent responses on both sides to left supraorbital nerve stimulation. Electromyography (EMG) was normal. He was on methotrexate and prednisone without improvement of the TSN. Gabapentin 800 mg daily and nortryptilin 100 mg daily were introduced with only a partial response at their maximum tolerable doses. Case 2A 47-year-old female had presented Raynaud' s phenomenon and arthritis in her hands for the last three years and was feeling progressive numbness in the inferior half of the face for the last two years, without any previous diagnosis. At her first neurological evaluation, she had sclerodermic fascies, sclerodactyly, hypoesthesia in the maxillary and mandibular divisions territory bilaterally. ANA was positive (1/80), an esophagogram showed an increased esophageal caliber and a thoracic tomography confirmed this finding and revealed signs of pulmonary fibrosis. All electrophysiological studies performed (NCS, BRS, EMG) were normal. She had never been on medication for TSN or SSc yet. Case 3A 50-year-old female had presented hypoesthesia only in the maxillary division of the right trigeminal nerve about three years before her first neurological evaluation. Six months later, she started presenting hypoesthesia in the mandibullary division, effort dyspnea, Raynaud' s phenomenon and myalgia. She was diagnosed with SSc in overlap with myositis 10 months before our firs...
The most frequent neurologic manifestations of hepatitis C virus infection include peripheral neuropathy axonal type and central nervous system (CNS) vasculitis. Affected patients usually have cryoglobulinemia and other signs of vasculitis. Demyelinating lesions, both central and peripheral are rarely described. We present a case of simultaneous peripheral nervous system and CNS demyelination that comes in relapsing episodes, with negative cryoglobulins.
RESUMOIntrodução: Embora a fotostimulação intermitente (FEI) seja rotineiramente utilizada nos laboratórios de eletrencefalografia (EEG), raramente é utilizada de forma padronizada. A FEI é um método de ativação utilizado no EEG de rotina que pode desencadear tanto respostas fisiológicas quanto potencialmente patoló-gicas. Historicamente, o termo fotossensibilidade se refere às respostas anormais à estimulação com luz estroboscópica durante o registro do EEG. Objetivo: O objetivo desta publicação é revisar os aspectos diagnósticos do procedimento da FEI, baseados no encontro de consenso realizado em Heemstede na Holanda, em 1996, com o propósito de facilitar e padronizar a detecção de pacientes fotossensíveis.Unitermos: Fotostimulação intermitente; fotossensibilidade; resposta foto-paroxística. ABSTRACTPhotic stimulation: Standardization of the method based on european consensus Introduction: Although intermittent photic stimulation (IPS) is a widespread and routinely used procedure in EEG laboratories, only relatively recently has a standardization of the IPS method been proposed. IPS is an activation method used during EEG procedure that may trigger either physiological or potentially pathological responses. Historically, the term photosensitivity is referred to abnormal responses to stroboscopic light during EEG. Objective: The purpose of this publication was to provide an overview of the diagnostic aspects of IPS procedure, based on data presented at Consensus Meetings held in Heemstede, the Netherlands, in 1996, with the purpose of facilitating the detection of photosensitive patients.Keywords: Intermittent photic stimulation; photosensitivity; photoparoxysmal response.A fotostimulação intermitente (FEI) é um método de ativação utilizado no eletrencefalograma (EEG) de rotina que pode desencadear tanto respostas fisiológicas quanto potencialmente patológicas. Entre as últimas destacam-se as crises epilépticas, sobretudo as generalizadas, principalmente as mioclonias, mas também ausências e crises tôni-co-clônicas generalizadas. Historicamente, o termo fotossensibilidade se refere às respostas anormais à estimulação com luz estroboscópica durante o registro do EEG. 13Em 1999, um grupo europeu de especialistas, com o apoio da International League against Epilepsy (ILAE), após realizar um inventário em vários laboratórios de EEG de países europeus sobre a prática de realização da FEI, publicou recomendações para padronização deste método para identificação de indivíduos fotossensíveis. 5O objetivo dessa medida foi tornar o método seguro, prático e reprodutível, tanto na prática clínica como para fins de pesquisa.
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