Younger age at stroke onset but not thrombolytic treatment predicts poststroke epilepsy: An updated meta-analysis.

AIMS: Stroke is the most commonly identified cause of late-onset epilepsy. Risk factors for poststroke epilepsy (PSE) are partially elucidated, and many studies have been performed in recent years. We aimed to update our previous systematic review and meta-analysis on risk factors for PSE. METHODS: PubMed, Google Scholar, and Scopus databases were searched. Articles published in English (1987-2019) were included. Odds ratios (OR) and mean values were calculated for examined variables. RESULTS: Thirty studies with different designs were included, enrolling 26,045 patients who experienced stroke, of whom 1800 had PSE, corresponding to a prevalence of 7%. Cortical lesions (OR: 3.58, 95% confidence interval (CI): 2.35-5.46, p < 0.001), hemorrhagic component (OR: 2.47, 95% CI: 1.68-3.64, p < 0.001), early seizures (ES) (OR: 4.88, 95% CI: 3.08-7.72, p < 0.001), and younger age at stroke onset (difference in means: 2.97 years, 95% CI: 0.78 to 5.16, p = 0.008) favor PSE. Sex and acute treatment with recombinant tissue plasminogen activator (rtPA) do not predict the occurrence of PSE. CONCLUSION: Despite limitations due to the uneven quality and design of the studies, the present meta-analysis confirms that cortical involvement, hemorrhagic component, and ES are associated with a higher risk of PSE. In this update, younger age at stroke onset but not thrombolytic treatment seems to increase the risk for PSE. This article is part of the Special Issue "Seizures & Stroke".

Rating scale for psychogenic nonepileptic seizures: scale development and clinimetric testing.

Our aim was to develop a clinimetric scale evaluating motor phenomena, associated features, and severity of psychogenic nonepileptic seizures (PNES). Sixty video/EEG-recorded PNES induced by suggestion maneuvers were evaluated. We examined the relationship between results from this scale and results from the Clinical Global Impression (CGI) scale to validate this technique. Interrater reliabilities of the PNES scale for three raters were analyzed using the AC1 statistic, Kendall's coefficient of concordance (KCC), and intraclass correlation coefficients (ICCs). The relationship between the CGI and PNES scales was evaluated with Spearman correlations. The AC1 statistic demonstrated good interrater reliability for each phenomenon analyzed (tremor/oscillation, tonic; clonic/jerking, hypermotor/agitation, atonic/akinetic, automatisms, associated features). KCC and the ICC showed moderate interrater agreement for phenomenology, associated phenomena, and total PNES scores. Spearman's correlation of mean CGI score with mean total PNES score was 0.69 (P<0.001). The scale described here accurately evaluates the phenomenology of PNES and could be used to assess and compare subgroups of patients with PNES.

Seizures with Migraine-like Attacks after Radiation Therapy (SMART): A new meaning of an old acronym.

Stroke-like migraine attacks after radiation therapy (SMART) is a late-onset complication of cerebral irradiation, clinically characterized by headache, seizures and focal deficits. We describe two patients with SMART presenting with focal status epilepticus and headache. We believe that SMART is a misnomer that misjudge seizures among clinical features and we suggest to rename this entity as " Seizure with Migraine-like Attacks after Radiation Therapy". The new acronym, modified in its meaning but not in its form (SMART), better reflects the main clinical features and may allow neurologists to recognize this condition more easily.

Wernicke's encephalopathy after allogeneic stem cell transplantation.

Wernicke's encephalopathy is an acute neuropsychiatric condition due to thiamine deficiency frequently associated with chronic alcohol abuse. We describe 2 cases of patients who experienced acute Wernicke's encephalopathy after allogeneic stem cell transplantation associated with the use of commercial total parental nutrition. Early diagnosis with magnetic resonance imaging and timely treatment with thiamine resulted in rapid resolution of clinical and radiological signs. In conclusion, the prolonged use of commercial total parental nutrition formulas must be supplemented with thiamine in the form of intramuscularly administered multivitamins.

Novel spastin (SPG4) mutations in Italian patients with hereditary spastic paraplegia.

Spastic paraplegia type 4 is caused by mutations in the gene that encodes spastin (SPG4), a member of the AAA protein family. A cohort of 34 unrelated Italian patients with pure spastic paraplegia, of which 18 displayed autosomal dominant inheritance and 16 were apparently sporadic, were screened for mutations in the SPG4 gene by denaturing high performance liquid chromatography. We identified a previously reported mutation in a sporadic patient with pure hereditary spastic paraplegia. We also identified eight unrelated patients with pure autosomal dominant hereditary spastic paraplegia carrying five novel mutations in the SPG4 gene (one missense mutation, c.1304 C>T; one nonsense mutation, c.807C>A; two frameshift mutations, c.1281dupT, c.1514_1515insATA; and one splicing mutation, c.1322-2A>C). The frequency for SPG4 mutations detected in autosomal dominant hereditary spastic paraplegia was 44.4%. This study contributes to expand the spectrum of SPG4 mutations in Italian population.

Teaching neuroimages: Pseudohypertrophic cerebral cortex in end-stage Creutzfeldt-Jakob disease.

A 43-year-old woman presented with 1 month of progressive lower limb burning sensation, blurred vision, and gait disturbance. Her mother died of Creutzfeldt-Jakob disease (CJD). Neurologic examination revealed only cerebellar ataxia. EEG revealed periodic generalized discharges. MRI (figure 1) showed FLAIR hyperintensity in caudate and lenticular nuclei. Molecular analysis confirmed genetic CJD (PRNP E200K mutation). One month later, she became comatose. After 10 months, EEG showed diffuse background flattening without periodic abnormalities, and MRI (figure 2) disclosed diffuse pseudohypertrophy of cerebral cortex. MRI in this patient with end-stage CJD allowed us to reveal in vivo the macroscopic spongiform changes usually observed at autopsy.

Non-paraneoplastic limbic encephalitis characterized by mesio-temporal seizures and extratemporal lesions: a case report.

Limbic encephalitis (LE) can be either paraneoplastic or a non-paraneoplastic autoimmune disorder. Magnetic resonance imaging (MRI) of the brain on T2-weighted fluid-attenuated inversion recovery (FLAIR) classically shows hyperintensities of the temporal structures, but multifocal involvement of extratemporal cortex has also been described in paraneoplastic LE. Here we describe a 27-year-old woman whose idiopathic autoimmune (glutamic acid decarboxylase-antibody positive) LE debuted with multiple daily mesio-temporal seizures, amnesia and multifocal extratemporal cortical MRI abnormalities. Mesio-temporal MRI signal increase was found after 20 days. This case report highlights that early diagnosis of non-paraneoplastic LE may be considered in patients with multiple daily mesio-temporal seizures and amnesia even in the absence of early typical MRI abnormalities.

Glucose metabolism in the idiopathic blepharoptosis: utility of the Oral Glucose Tolerance Test (OGTT) and of the Insulin Resistance Index.

BACKGROUND: Diabetes mellitus (DM), neuromuscular, hereditary or immunological disorders are the most common identified causes of blepharoptosis. However, in about 15-25% they remained uncertain. OBJECTIVE: To determined the role of glucose metabolism abnormality in idiopathic blepharoptosis. METHODS: We identified 162 patients with unilateral idiopathic blepharoptosis and 128 control subjects. In all we evaluated a glucose and insulin levels at fasting and after 2 h-OGTT. In addition we determined insulin resistance (IR), by HOMA-index. RESULTS: Following a 2 h-OGTT the prevalence of undiagnosed glucose metabolism abnormality was significantly higher in blepharoptosis patients vs. control group (P<.001). The IR was documented in 129 patients (78%), of whom 55 (34%) had Impaired Glucose Tolerance (IGT), 36 (22%) newly diagnosed DM (NDDM) and 38 (30%) only IR. The Body Mass Index, blood pressure, serum lipids, triglycerides and smoking were not associated with an increased risk of developing ptosis. Conversely, waist circumference were significantly increased in blepharoptosis patients (P=.003). CONCLUSIONS: In this study we focused on emerging evidence that prediabetic status may represent a risk factor for developing blepharoptosis. We propose that 2 h-OGTT and mainly HOMA-index should be determined as a rule in all patients with idiopathic blepharoptosis.

Simple decompression versus anterior submuscular transposition of the ulnar nerve in severe cubital tunnel syndrome: a prospective randomized study.

OBJECTIVE: The authors report the results of a clinical series of selected patients with severe cubital tunnel syndrome. The degree of ulnar nerve compression was evaluated by use of a grading system that includes measurements of motor and sensitive function. The submuscular transposition with flexor-pronator mass Z lengthening was compared with simple decompression through a prospective randomized study. METHODS: From February 1998 to June 2003, 70 patients with severe cubital tunnel syndrome were included in this study: 35 patients were submitted to simple decompression (Group A), and 35 patients were treated by anterior deep submuscular transposition (Group B). The preoperative status was determined by use of Dellon's classification. The selected patients had Dellon's Grade 3 (severe syndrome). The mean follow-up period after surgery was 47 months for Group A and 46.94 months for Group B. RESULTS: Postoperative clinical and electrophysiological outcomes were assessed 6 months after surgery in all 70 patients. According to the Bishop scoring system, 19 patients (54.3%) of Group A were clinically graded as excellent, 9 (25.7%) were graded as good, and 7 (20%) were graded as fair; in Group B, 18 patients (51.43%) were graded as excellent, 11 (31.43%) as good, and 6 (17.14%) as fair. Neither severe complications nor recurrences were observed in the two groups. CONCLUSION: No statistically significant difference was found between the two groups with regard to the clinical or the electrophysiological outcome. The surgical treatment gains in Group A and B were 80% and 82.86%, respectively (good to excellent results).