Post-stroke seizures: risk factors and management after ischemic stroke.

Stroke is the leading cause of epilepsy in the elderly, ahead of degenerative diseases, tumors and head injuries. It constitutes a significant complication and a considerable comorbidity. The aim of our study was to describe the main factors implicated in the occurrence of post-stroke seizures and to identify the predictors of seizure recurrence. We conducted a descriptive, retrospective, monocentric study from January 2010 to December 2019, including patients who presented seizures following an ischemic stroke. We classified these seizures according to the International League Against Epilepsy (ILAE) into acute symptomatic seizures (ASS) if they occur within seven days of stroke, and unprovoked seizures (US) if they occur after more than one week. Clinical, para-clinical, therapeutic and follow-up data were statistically analyzed and compared. A total of 52 patients were included (39 men, 13 women; median age 55.1 years). 21 cases (40%) had ASS and the remaining 31 cases (60%) presented US. Young age below 65 years (71%), middle cerebral artery infarcts (83%), and cortical localization (87%) were the main factors depicted in our series. Parietal lobe infarction was more associated with US than ASS (p = 0.035). 24 patients (46%) have presented a recurrence of seizures (8/21 of ASS and 16/31 of US). The use of sodium valproate in monotherapy was identified as a recurrence risk factor (p = 0.013). In patients with post-stroke seizures, parietal lobe infarcts are more associated with US. We identified a higher risk of seizure recurrence in patients treated with sodium valproate monotherapy.

Case Report: The first reported case of pulmonary alveolar proteinosis with myasthenia gravis in a 27-year-old patient.

Background: Pulmonary alveolar proteinosis is a very rare diffuse lung disease characterized by the accumulation of amorphous and periodic acid Schiff-positive lipoproteinaceous material in the alveolar spaces due to impaired surfactant clearance by alveolar macrophages. Three main types were identified: Autoimmune, secondary and congenital. Pulmonary alveolar proteinosis has been previously reported to be associated with several systemic auto-immune diseases. Accordingly, we present the first case report of pulmonary alveolar proteinosis associated with myasthenia gravis. Case: A 27-year-old female patient, ex-smoker, developed a dyspnea on exertion in 2020. The chest X-ray detected diffuse symmetric alveolar opacities. Pulmonary infection was ruled out, particularly COVID-19 infection. The chest scan revealed the "crazy paving" pattern. The bronchoalveolar lavage showed a rosy liquid with granular acellular eosinophilic material Periodic acid-Schiff positive. According to the lung biopsy results, she was diagnosed with pulmonary alveolar proteinosis. The granulocyte macrophage colony-stimulating factor autoantibodies were negative. Nine months later, she was diagnosed with bulbar seronegative myasthenia gravis, confirmed with the electroneuromyography with repetitive nerve stimulation showing significant amplitude decrement of the trapezius and spinal muscles. She was treated with pyridostigmine, oral corticosteroids and azathioprine. Given the worsening respiratory condition of the patient, a bilateral whole lung lavage was performed with a partial resolution of symptoms. Thus, this previously unreported association was treated successfully with rituximab, including improvement of dyspnea, diplopia and muscle fatigability at six months of follow-up. Conclusions: This case emphasizes on the possible association of auto-immune disease to PAP, which could worsen the disease course, as the specific treatment does not exist yet. Hence, further studies are needed to establish clear-cut guidelines for PAP management, particularly when associated to auto-immune diseases.

Early pregnancy-associated ischemic stroke during first trimester in a young woman: A case report.

Pregnancy-associated ischemic stroke is rare. The degree of the risk is the highest in the third trimester, but clinicians should be also wary from the beginning of the pregnancy as the risk still exists like demonstrated by our case.

Acute-onset chronic inflammatory demyelinating polyneuropathy with cranial nerves and respiratory tract involvement: A case report.

Sixteen percent of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely like acute idiopathic demyelinating polyneuropathy (AIDP) the demyelinating form of GBS, developing in <8 weeks 2. This entity is classified as acute-onset CIDP (A-CIDP) which presents overlapping clinical and electrophysiological findings with GBS during early stages of disease, but followed with a chronic course beyond 2 months. Also, those who have three or more treatment-related fluctuations (TRF) are included under this term. Distinguishing between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and acute idiopathic demyelinating polyneuropathy (AIDP) may be difficult during early stages but is crucial in order to guide treatment strategies without delay. These two forms share some overlapping clinical and electrophysiological findings, including some severe clinical features such as cranial nerve and respiratory tract involvement making the diagnosis of A-CIDP more difficult.

Cognitive impairment in celiac disease and non-celiac gluten sensitivity: review of literature on the main cognitive impairments, the imaging and the effect of gluten free diet.

Celiac disease (CD) and non celiac gluten sensitivity (NCGS) can be responsible for neurological complications such as ataxia and peripheral neuropathies but also cognitive impairment. This cognitive involvement is variable in its expression, its duration and its prognosis ranging from transient and reversible subtle involvement to dementia itself. Through this article, we tried to achieve a review of the literature to better understand this topic. Several mechanisms were proposed to explain the deleterious influence of gluten-related pathologies on cognitive functions: nutritional deficiencies, elevation of circulating cytokine levels due to systemic inflammation, low brain serotonin levels… Several types of dementia such as Alzheimer dementia, vascular dementia, frontotemporal dementia were reported in association with CD. Memory disorder, acalculia, inattention, visuospatial deficits and executive dysfunction must be sought systematically by a neuropsychological assessment in patients with CD or NCGS. As far as the cognitive impairment is concerned, there is no pathognomonic radiological lesion. Concerning therapeutic management; although its effect is controversial, gluten free diet should be introduced, as early as possible, because of its potentially protective effect.

Stroke disclosing primary aldosteronism: Report on three cases and review of the literature.

OBJECTIVES: There is a growing evidence of increased risk of cerebrovascular events in primary aldosteronism (PA). Nevertheless, acute neurologic ailment as presenting feature of PA is uncommon. Our aim is to highlight the diagnosis challenges in stroke unmasking PA and to discuss the underlying physiopathology and management dilemmas. MATERIALS AND METHODS: We hereby describe three consecutive rare cases of stroke revealing PA. All patients had brain imaging and thorough biological and morphological assessment to rule out other etiologies of stroke. The diagnosis of primary aldosteronism was established according to the Endocrine Society Clinical Practice Guideline, with a review of the literature on the spectrum of neurologic manifestations in PA. RESULTS: We report on three cases, two women and a man, presenting with ischemic or hemorrhagic stroke, of early onset in two of them. All of the reported patients had hypertension and hypokaliemia. This association prompted the assessment of renin angiotensin aldosterone system (RAAS) disclosing PA, which was due to bilateral adenomas in the first one or bilateral adrenal hyperplasia in the two others. All patients refused the surgical option and received spironolactone with recurrence of stroke in one of them due to treatment incompliance. CONCLUSION: Although cerebrovascular events are quite common in PA, their occurrence as initial feature can be misleading. The association of hypokaliemia and refractory hypertension in ischemic or hemorrhagic strokes should prompt an assessment of the RAAS to rule out PA and initiate adequate management as soon as possible in order to avoid further complications.

Recurrent status epilepticus in posterior reversible encephalopathy syndrome as initial feature of pediatric lupus: A newly diagnosed case and literature review.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a recently described clinico-neuroradiological syndrome with several predisposing conditions. Systemic lupus erythematosus (SLE), beginning in 15-20% in childhood, is considered as a potential underlying etiology of PRES. In children, status epilepticus (SE) rarely complicates PRES, and exceptionally occurs in SLE. METHODS: We report on an illustrative case of PRES complicating pediatric lupus revealed by recurrent SE, and we further review through a Pubmed search the previously reported cases of pediatric SLE, PRES and SE. RESULTS: We describe the case of a 12-year old girl who presented with recurrent status epilepticus associated to high blood pressure and renal involvement. Brain imaging showed classical aspects of PRES. Immunological tests including antinuclear, anti-DNA, and anticardiolipin antibodies were positive. The diagnosis of SLE was established. The Pubmed search identified a total number of 9 children with SE in SLE, and 26 with PRES, including our patient. CONCLUSIONS: We discussed the clinical and paraclinical features of PRES in SLE with epilepsy, their underlying pathophysiological aspects, and their management challenges. PRES should be considered in initial recurrent SE in children, justifying a battery of tests comprising immunological testing. Anticardiolipin antibodies seem to play a crucial role in epilepsy, PRES and renal involvement in pediatric SLE. Further studies are needed to clarify whether PRES should be considered one of the neuropsychiatric manifestations of SLE or a consequence of active disease in other organ systems or its treatment.

Occipital Sinus Thrombosis: An Exceptional Case Report.

BACKGROUND: Variations of the dural venous sinuses may result in inaccurate imaging interpretation or complications during surgical approaches. One variation of the dural venous sinuses reported infrequently in the literature is the occipital sinus. We report an exceptional case of occipital sinus thrombosis. CASE REPORT: A 48-year-old right-handed man with a 5-month history of hypertension and chronic renal failure presented with cephalalgia, vomiting, and blurred vision evolving over 48 hours. Neurological examination revealed papillary edema stage 1 with no others abnormalities. An initial brain computed tomography (CT) scan performed was normal. The opening pressure of cerebrospinal fluid (CSF) was 35 cmH2O with normal level of protein and no hypercellularity in CSF analysis. The evolution was marked by the occurrence of generalized tonic-clonic seizure. A second CT scan performed showed a hyperdensity of the occipital sinus. Magnetic resonance imaging and magnetic resonance venography studies confirmed the diagnosis with highlighting the thrombosis of the occipital sinus in association to an ectasia of the torcular. The patient received adequate anticoagulation for 6 months in association to antiepileptic drugs with a good evolution. DISCUSSION: According to our review, such a thrombosis must be a rare condition, because our literature search has shown a lack of any report describing this condition. Herein, we review the anatomy of the occipital sinus and we illustrate the characteristics of this unusual thrombosis with multiple imaging modalities. CONCLUSION: Understanding of the cerebral venous anatomy and recognition of venous variations essentially help when dealing with a pathology, which presents along with a particular venous variation, no matter how rare this combination is.

Limbic encephalitis associated with leucine-rich glioma-inactivated 1 antibodies.

We describe the case of a patient with confirmed limbic encephalitis associated with leucine-rich glioma-inactivated 1 (LGI1) antibodies. A 59-year-old man presented to the Department of Neurology with bizarre behavior, memory loss, cognitive impairment, visual hallucinations, and myoclonus and facio-brachial dystonic seizures. A brain magnetic resonance imaging (MRI) revealed no hippocampal lesions. Blood tests showed hyponatremia. An electroencephalogram showed disorganization and slowing of background activity. Antiepileptic drugs were ineffective. The patient exhibited considerable improvement following immunotherapy. The diagnosis of limbic encephalitis associated with LGI1 antibodies should be considered in patients with clinical manifestations mimicking psychiatric disorders and in cases of refractory epilepsy especially with faciobrachial dystonic seizures. There is frequently hyponatremia, and cerebral MRI may be normal. Full recovery can be expected with early diagnosis and prompt treatment.

Association of HLA-DR/DQ polymorphisms with Guillain-Barré syndrome in Tunisian patients.

UNLABELLED: Human leukocyte antigen (HLA) alleles have been implicated in many autoimmune diseases. The aim of this study is to assess whether HLA-DR/DQ alleles confer susceptibility to Guillain-Barré syndrome (GBS) in a Tunisian population. METHODS: The HLA-DR/DQ genotyping was performed using polymerase chain reaction sequence-specific primers (PCR-SSP) in 38 patients with GBS and 100 healthy Tunisian control subjects. RESULTS: GBS in Tunisian patients was found to be associated with the following alleles with these relative patient versus control frequencies (pc denotes Bonferroni corrected probability values): DRB1*13 (23.68% vs. 9.0%; pc=0.01), followed by DRB1*14 (22.36% vs.5.5%; pc<10(-3)). Two haplotypes, DRB1*14/DQB1*05 and DRB1*13/DQB1*03, were found to be associated with susceptibility to GBS. However DRB1*07/DQB1*02 and DRB1*03/DQB1*02 haplotypes were more frequently observed in controls than in patients (11.5% vs.7.9%; pc=0.007 and 23% vs. 5.26%; pc<10(-3) respectively). These haplotypes seem to confer protection against the disease. CONCLUSION: Our data demonstrated a new GBS predisposition associated with HLA-DRB1*14 and DRB1*13. Theses alleles could be predisposing genetic factors for GBS in the Tunisian population.

Cerebral venous thrombosis: clinical features, risk factors, and long-term outcome in a Tunisian cohort.

BACKGROUND: Data from African countries regarding diagnosis, prognosis, management, and outcome of patients with cerebral venous thrombosis (CVT) are limited. The aim of the present study is to characterize clinical presentation, predisposing factors, neuroimaging findings, and outcomes of the disease in the Tunisian population. METHODS: This is a prospective study including patients referred to the Neurology Department of the Military Hospital of Tunis between January 2009 and December 2012. The diagnosis of CVT was confirmed in all patients using magnetic resonance imaging and magnetic resonance venography. The demographic, clinical, radiological, and outcome data were recorded and analyzed. Median follow-up was 16 months (range 6 months to 4 years). Primary outcome was death or dependency as assessed by modified Rankin score more than 2 at the end of follow-up. RESULTS: This study included 41 patients with CVT. Mean age was 41.24 years, predominantly women (68%). The mode of onset was acute in 10 patients (24%), subacute in 26 (64%), and chronic in 5 (12%). The most common presenting features were headache, observed in 83% of the patients, followed by seizures, focal motor deficits, papilledema, and mental status changes. Lateral (56%) and superior longitudinal (51%) sinuses were the most commonly involved. Multiple sinuses were involved in 46% of cases. Nineteen patients (46%) had a D-dimer level more than 500 ng/mL. Major causes of CVT were thrombophilia (56%), either genetic or acquired, obstetric and gynecological (50%), and septic (34%). Outcome was favorable in 83% of patients. At the end of follow-up, 32 patients (78%) had complete recovery (modified Rankin Scale [mRs] score 0-1), 2 (5%) had partial recovery (mRs score 2), and 4 (10%) were dependent (mRs score 3-5). One patient (2.5%) had a recurrent sinus thrombosis. CONCLUSIONS: Our Tunisian population presented distinct risk factors profile with high frequency of thrombophilia, infections, and postpartum state. Oral contraceptive use is not a major risk factor in our population. The overall prognosis was good.

Apolipoprotein E genotypes associated with Alzheimer disease and concomitant stroke.

BACKGROUND: The ɛ4 allele of the apolipoprotein E (APOE) gene is a well-characterized genetic risk factor for Alzheimer disease (AD). The association between stroke and a higher risk for AD has also been reported. Our study sought to determine the relationship between the APOE gene and AD and the comorbid risk of stroke. METHODS: The subjects of this study consisted of 48 patients with AD and 48 members of a control group. All subjects were genotyped for APOE. RESULTS: The results clearly show a significant increased risk of AD in carriers of the APOE ε3/ε4 genotype (P = .003, odds ratio [OR] = 4.1) or ε4 allele (P = .001, OR = 4.2). The risk for stroke in AD patients was also increased for carriers of the APOE ε3/ε4 genotype (P = .02, OR = 9.0) and for carriers of the APOE ε4 allele (P = .004, OR = 5.5). CONCLUSIONS: The present study is the first to establish a relationship between APOE ε4 and concomitant AD and stroke in the Tunisian population.

Association of methylenetetrahydrofolate reductase A1298C polymorphism but not of C677T with multiple sclerosis in Tunisian patients.

BACKGROUND AND OBJECTIVE: Multiple sclerosis (MS) is a chronic neurological disease characterized by central nervous system (CNS) inflammation and demyelination of nerve axons. The aim of this study was to investigate a possible association between the methylenetetrahydrofolate reductase (MTHFR) gene and multiple sclerosis in Tunisian patients. PATIENTS AND METHODS: The genotyping of two missense variants of the methylenetetrahydrofolate reductase (MTHFR) gene, C677T and A1298C was performed in 80 multiple sclerosis patients and 200 healthy controls. RESULTS: No significant differences were found in the frequency of the MTHFR C677T polymorphism between MS patients and healthy controls. However, the genotype prevalence of the missense variant MTHFR A1298C was significantly different between patients and controls (A/C: 55% versus 7%, p<10(-3); C/C: 13.75% versus 0%, p<10(-3), respectively). CONCLUSION: Although our preliminary findings suggest no association between the MTHFR C677T variants and MS, there is evidence to suggest a significant association between the MTHFR A1298C polymorphisms and MS.