Early seizures and risk of epilepsy and death after intracerebral haemorrhage: The MUCH Italy.

INTRODUCTION: It is unclear which patients with non-traumatic (spontaneous) intracerebral haemorrhage (ICH) are at risk of developing acute symptomatic seizures (provoked seizures occurring within the first week after stroke onset; early seizures, ES) and whether ES predispose to the occurrence of remote symptomatic seizures (unprovoked seizures occurring more than 1 week after stroke; post-stroke epilepsy, PSE) and long-term mortality. PATIENTS AND METHODS: In the setting of the Multicenter Study on Cerebral Haemorrhage in Italy (MUCH-Italy) we examined the risk of ES and whether they predict the occurrence of PSE and all-cause mortality in a cohort of patients with first-ever spontaneous ICH and no previous history of epilepsy, consecutively hospitalized in 12 Italian neurological centers from 2002 to 2014. RESULTS: Among 2570 patients (mean age, 73.4 ± 12.5 years; males, 55.4%) 228 (8.9%) had acute ES (183 (7.1%) short seizures and 45 (1.8%) status epilepticus (SE)). Lobar location of the hematoma (OR, 1.49; 95% CI, 1.06-2.08) was independently associated with the occurrence of ES. Of the 2,037 patients who were followed-up (median follow-up time, 68.0 months (25th-75th percentile, 77.0)), 155 (7.6%) developed PSE. ES (aHR, 2.34; 95% CI, 1.42-3.85), especially when presenting as short seizures (aHR, 2.35; 95% CI, 1.38-4.00) were associated to PSE occurrence. Unlike short seizures, SE was an independent predictor of all-cause mortality (aHR, 1.50; 95% CI, 1.005-2.26). DISCUSSION AND CONCLUSION: The long-term risk of PSE and death after an ICH vary according to ES subtype. This might have implications for the design of future clinical trials targeting post-ICH epileptic seizures.

Pregnancy in Charcot-Marie-Tooth disease: Data from the Italian CMT national registry.

OBJECTIVE: To collect information on frequency of pregnancy and delivery complications in Charcot-Marie-Tooth (CMT) disease and on CMT course during pregnancy. METHODS: Through an ad hoc online questionnaire, we investigated pregnancy and neuropathy course in women with CMT adhering to the Italian CMT Registry. Data were compared to those of controls (recruited among friends and unaffected relatives) and the Italian (or other reference) population. RESULTS: We collected data on 193 pregnancies from 86 women with CMT (age 20-73 years) with 157 deliveries (81.4%) after a mean of 38.6 gestational weeks. In women with CMT, there were no differences compared to controls (59 pregnancies and 46 deliveries from 24 controls) and the reference population for miscarriages (11.4%) and planned (21.0%) and emergency (14.0%) cesarean sections. We found a significantly higher frequency of placenta previa (1.6% vs 0.4%), abnormal fetal presentations (8.4% vs 4.5%), and preterm deliveries (20.3% vs 6.9%; most in week 34-36 of gestation) compared to reference populations. Excluding twins, newborn weight did not differ from the reference population. Postpartum bleeding rate in patients with CMT (2.1%) was similar to that of the general population (2.4%). CMT status worsened during 18 of 193 pregnancies (9.3%) with no recovery in 16 of them and with similar figures in the CMT1A and non-CMT1A subtypes. CONCLUSIONS: We observed higher rates of placenta previa, abnormal presentations, and preterm deliveries in CMT, but pregnancy outcome and newborn weight and health were similar to those of the reference populations. Worsening of CMT is not infrequent and occurs not only in CMT1A. Pregnant women with CMT should be monitored with particular care.

Pain at the onset of Amyotrophic Lateral Sclerosis: a cross-sectional study.

OBJECTIVE: We evaluated ALS patients reporting pain, either generalized or localized, at disease onset and determined whether this feature defined a specific ALS phenotype. PATIENTS AND METHODS: We considered all consecutive ALS patients referred to our Motor Neuron Diseases Center between 2006 and 2016 and included only patients who fulfilled the El Escorial revised criteria for probable and definite ALS diagnosis. We then identified those cases who reported pain at disease onset and compared them to all remaining cases. Secondary causes of pain have been excluded. RESULTS: Our initial sample consisted of 108 patients (55 men and 53 women). We identified 5 cases with generalized pain and 16 cases with localized pain at disease onset, corresponding to 4.6% and 14.8% of the initial sample, respectively. Cases with generalized pain were all female and had an earlier disease onset (49.6 ± 1.5 vs 66.6 ± 10.2 yrs, p = 0.002). Cases with localized pain showed a preponderance of upper motor neuron symptoms/signs at disease onset. Patients with pain, either localized or generalized, had a significantly higher involvement of the limbs (82.6% vs 100%, p = 0.022), while the bulbar district was spared at disease onset (17.4% vs 0%, p = 0.008). More specifically, the proximal upper and distal lower limbs were more frequently affected by ALS in patients with pain at disease onset. In two cases, the clinical presentation was notable for the resemblance with complex regional pain syndrome. CONCLUSION: The presence of pain at disease onset seems to relate to peculiar clinical features of ALS and may be pathophysiologically associated with neurodegeneration.