Biotin-thiamine-responsive basal ganglia disease (BTBGD) is a rare, inherited neurometabolic disorder caused by mutations in the SLC19A3 gene and characterized by recurrent sub-acute episodes of encephalopathy that are often triggered by infections. Patients with BTBGD have classical neuroimaging findings and a dramatic response to high doses of thiamine. Herein, we report a 2 and a half-year-old Saudi girl presented with an acute onset of ataxia, slurred speech, and dysphagia, which was preceded by a history of accidental ingestion of around 20 mL of ethyl alcohol that is used in formulating ..read more

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Background and Purpose: Although aura in epilepsy is usually considered as a phenomenon in patients with focal seizures, headache can occur as an aura or solitary epileptic symptom in patients with generalized seizures. Methods: We performed a 14-year retrospective study of patients with generalized seizures and analyzed the proportion and characteristics of patients with auras including headache. Results: Among the 102 patients diagnosed with generalized seizures, aura was reported in 45 patients and headache was the most common aura in 26 patients. The age of onset of seizures was significan ..read more

In aphasic status epilepticus (ASE), aphasia is the sole manifestation of seizure in patients with this disorder. Alzheimer’s disease (AD) is one of neurological disorders causing ASE. Herein, we report two cases of ASE associated with AD, and discuss their clinical characteristics. Patient 1 presented Broca’s aphasia, and patient 2 presented global aphasia during the ictal period. Both patients exhibited atypical ictal electroencephalographic (EEG) patterns, which improved after antiepileptic drug administration. ASE was the presenting symptom of AD in patient 1. ASE can develop at any stage ..read more

The resective epilepsy surgery can be the effective procedure to get seizure-free outcome in these drug resistant epilepsy (DRE) patients. Class I evidence firmly establishes the superiority of epilepsy surgery over medical treatments in both seizure control and quality of life for DRE patients. For the effective identification of optimal surgical candidates, it's essential to understand the prognostic factors of epilepsy surgery based on the surgical methods employed. Established positive prognostic indicators for temporal resection include the presence of hippocampal sclerosis on magnetic re ..read more

Background and Purpose: Cardiac abnormalities have been reported during ongoing seizures and refractory status epilepticus (RSE). Reduced heart rate variability (HRV) and cardiac arrhythmias may contribute to sudden unexpected death in epilepsy. We sought to explore the utility of electrocardiographic and echocardiographic changes in patients with RSE prognosis and functional outcome. Methods: Patients of RSE underwent electrocardiogram (ECG), holter, troponin-I (Trop I), N-terminal prohormone of brain natriuretic peptide (NT-proBNP), and 2-dimensional echocardiogram (2D Echo) along with conti ..read more

Seizure aggravation in women with epilepsy (WWE) tends to occur at two specific times during the menstrual cycle: the perimenstrual phase and the ovulation period. Antiseizure drugs (ASDs), especially those that induce enzymes, can accelerate the metabolism of hormones in oral contraceptives, rendering them less effective. Estrogen in contraceptive pills increases the metabolism of lamotrigine. Physiological changes during pregnancy can significantly impact the pharmacokinetics of ASDs, potentially necessitating adjustments in dosage for women with epilepsy to maintain seizure control. The use ..read more

Psychiatric disorders are commonly observed in patients with epilepsy. Among them, the phenomenon known as forced normalization is scarce. Herein, we report the case of a 41-year-old patient who showed long-term first-onset psychiatric symptoms after seizure remission and normalization of electroencephalography. After changing the antiepileptic drug regimen and psychiatric treatment, the patient’s symptoms regressed. However, the exact pathological mechanisms remain to be elucidated. Changing the regimen of antiepileptic drugs and long-term psychiatric treatment may help control this phenomeno ..read more

Background and Purpose: During the coronavirus disease 19 (COVID-19) pandemic, a considerable number of studies have focused on the difficulties for accessing the medical services and telemedicinerelated issues. However, it is not clear whether COVID-19 affects the clinical course of epilepsy. Therefore, in the current study we aimed to assess the effects of COVID-19 infection on seizure frequency in patients with epilepsy (PWE). Methods: We evaluated PWE who consecutively referred to the neurology clinics of 22 Bahman and Qaem hospitals, who had experienced a recent polymerase chain reaction ..read more

Cerebral salt wasting syndrome (CSWS) is defined as a renal loss of sodium in cerebral disorders causing hyponatremia and loss of extracellular fluid volume. Similar laboratory findings may be seen in other conditions such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). A 58-year-old male visited our emergency department because of the sudden development of seizures during sleep. Magnetic resonance imaging revealed subtle high signal intensity in the right hippocampus on diffusion-weighted imaging. Ictal rhythmic discharges were observed in the concordant area. Altered met ..read more