Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.

BACKGROUND: Few data are available for patients with a late onset (≥ 50 years) of neuromyelitis optica (LONMO) or neuromyelitis optica spectrum disease (LONMOSD), defined by an optic neuritis/longitudinally extensive transverse myelitis with aquaporin-4 antibodies (AQP4-Ab). OBJECTIVE: To characterize LONMO and LONMOSD, and to analyze their predictive factors of disability and death. METHODS: We identified 430 patients from four cohorts of NMO/NMOSD in France, Germany, Turkey and UK. We extracted the late onset patients and analyzed them for predictive factors of disability and death, using the Cox proportional model. RESULTS: We followed up on 63 patients with LONMO and 45 with LONMOSD during a mean of 4.6 years. This LONMO/LONMOSD cohort was mainly of Caucasian origin (93%), women (80%), seropositive for AQP4-Ab (85%) and from 50 to 82.5 years of age at onset. No progressive course was noted. At last follow-up, the median Expanded Disability Status Scale (EDSS) scores were 5.5 and 6 in the LONMO and LONMOSD groups, respectively. Outcome was mainly characterized by motor disability and relatively good visual function. At last follow-up, 14 patients had died, including seven (50%) due to acute myelitis and six (43%) because of opportunistic infections. The EDSS 4 score was independently predicted by an older age at onset, as a continuous variable after 50 years of age. Death was predicted by two independent factors: an older age at onset and a high annualized relapse rate. CONCLUSION: LONMO/LONMOSD is particularly severe, with a high rate of motor impairment and death.

Unilateral ptosis associated with paramedian thalamiic infarction.

The paramedian artery arises from P1 segment of posterior cerebral artery and supplies a variable extent of thalamus but usually the dorsomedian, median, internal medullary lamina and the intralaminar nuclei. The typical clinical picture of unilateral paramedian thalamic infarctions consist of arousal and memory disorders, language or visuospatial disorders depending on the side of the lesion accompanied with gaze palsies and sensory-motor deficits. Ipsilateral ptosis associated with paramedian thalamic infarctions has been rarely reported. We report a 31 years old patient presenting with unilateral ptosis and right sided facial numbness associated with right paramedian thalamic infarction.

The impact of prolonged hunger strike: clinical and laboratory aspects of twenty-five hunger strikers.

BACKGROUND: Hunger strike is a very serious entity which may lead to severe diseases and death. AIMS: The aim of this study is to document the clinical, neurophysiological, neuroradiological, and neuropsychological aspects of prolonged hunger strike. MATERIAL AND METHOD: We investigated the clinical and laboratory characteristics of 25 hungerstrikers hospitalized during refeeding process. One sample t-test, independent samples t-test, Mann-Whitney and Pearson correlation tests were used for statistical analyses. RESULTS: Twelve of them had a continuous hunger strike ranging between 190-366 days. The other 13 had quitting intervals for various reasons with a continuous hunger ranging between 65-265 days with a total hunger duration of 153-382 days. The mean loss of body mass index (BMI) was 40.98 +/- 9.3%. Imbalance, sleep disorders, somatosensory disturbances, and adynamia were the most common complaints. At admission, one third experienced ophthalmoparesis, about half of them had paresis, one quarter had truncal ataxia. At discharge 16% had persistent ophthalmoparesis and 36% nystagmus. Only four patients (16%) could walk independently. There was no serious MRI, EEG findings. Most prominent EMG findings were the decrease in median and sural nerve cnap, median and fibular cmap, and fibular ncv values. They showed mild impairment in MMTS and most of them had attention deficit and frontal type memory impairment. CONCLUSION: It can be concluded that vitamin B intake, independent of the quantity and timing, lowers the morbidity and mortality of hunger strikers.