Epilepsia partialis continua due to COVID-19 pneumonia.

BACKGROUND: Epilepsia partialis continua (EPC) is an uncommon condition with several different etiological causes. In this article, we presented a case of EPC due to COVID-19 infection. CASE REPORT: A 77-year-old woman with diabetes, asthma, hypertension, and chronic renal failure went to the emergency room with shortness of breath. The patient was awake and had slight hemiparesis sequela on the left due to a cerebrovascular incident 20 years earlier on neurological assessment. Non-contrast thorax computed CT revealed patchy ground-glass alveolo-acinar density increases in bilateral lung subzones, confirming COVID-19 pneumonia. After getting a positive COVID-19 PCR test, the patient was admitted to the department of infectious diseases. After a week in the hospital, seizures involving the right arm, leg, and part of the face appeared. The patient could not respond to questions. The patient's seizures lasted 12-24 hours. EEG was compatible with Epilepsia partialis continua. The cerebrospinal fluid examination was normal. Both clinical and EEG findings of the patient improved with treatment. CONCLUSIONS: Several causes may contribute to the onset of Epilepsia partialis continua. COVID-19 infection might be one of the etiological explanations for the diagnosis of Epilepsia partialis continua and the prognosis may be very good too.

Epilepsia partialis continua in relapsing-remitting multiple sclerosis: A possible distinct relapse phenotype.

Epilepsia partialis continua (EPC) is a rare phenomenon in multiple sclerosis (MS). We describe a patient with relapsing-remitting MS and three episodes of EPC, with refractoriness to anti-seizure drugs but corticosteroid-responsiveness. No lesions likely attributable to her episodes of EPC were seen on 1.5 Tesla MRI, which we hypothesize was due to the small volume of presumed cortical/juxtacortical lesions involving the primary motor cortex. The association with relapsing-remitting disease, corticosteroid responsiveness, and dissemination of episodes of EPC in both space and time in our patient suggest that EPC may represent a distinct relapse phenotype in MS.

'Hand mechanogram' in epilepsia partialis continua.

Epilepsia partialis continua manifests as low-frequency, rhythmic involuntary movements of a focal body part. We report a young man, HIV-positive and with syphilis, who developed right-hand epilepsia partialis continua associated with a small left-sided cortico-subcortical frontal lesion. A pen and paper test provided 'mechanographic' data on frequency, amplitude and rhythmicity of the hand movements, helping distinguish it from other causes of low-frequency repetitive hand movements.

Sporadic Creutzfeldt-Jakob disease presenting as epilepsia partialis continua and non-ictal nystagmus.

BACKGROUND AND PURPOSE: Creutzfeldt-Jakob disease (CJD) is a rare form of rapidly progressive neurodegenerative disorder. Seizures are uncommon in the early stage of CJD, increasing diagnostic difficulty. METHODS: An autopsy-proven case of CJD presenting initially as an epilepsia partialis continua is reported, in which the initial workup was unremarkable. Retrospectively, the presence of nystagmus, which proved to be non-epileptic, pointed to a cerebellar lesion before a diagnosis of clinically probable CJD was made. RESULTS: A 70-year-old man presented with a 3-week history of intermittent rhythmic jerking tremors in his left limbs, interfering with his gait. Examination showed left body clonic movements. Electroencephalography revealed an ictal right centroparietal pattern of focal status epilepticus. Video-oculography revealed right-beating nystagmus (mean slow phase velocity [SPV] 3.4º/s) in the dark and left-beating nystagmus (SPV 2.6º/s) in the light, left-beating nystagmus after head shaking (SPV 4º/s) and during mastoid vibration (SPV 11º/s) and mildly hypoactive horizontal head impulses. Search for occult malignancy, serologies, cerebrospinal fluid analyses, anti-onconeural antigen, auto-immune panel and brain magnetic resonance imaging were unrevealing. Rapid neurological decline was observed. Three weeks later, cerebrospinal fluid was positive for 14.3.3 protein, electroencephalography showed generalized periodic sharp wave complexes and brain magnetic resonance imaging revealed diffusion restriction and T2/fluid-attenuated inversion recovery hyperintensities in the cerebellum, basal ganglia, thalamus and cortex. He died 1 month later. Neuropathological study confirmed the diagnosis of CJD. CONCLUSION: This case highlights that CJD should be considered in the differential diagnosis of new onset epilepsia partialis continua and that neuro-ophthalmological examination can be helpful in pointing to early asymmetric cerebellar involvement.

Diagnostic algorithm for children presenting with epilepsia partialis continua.

OBJECTIVE: To characterize a cohort of children with epilepsia partialis continua (EPC) and develop a diagnostic algorithm incorporating key differential diagnoses. METHODS: Children presenting with EPC to a tertiary pediatric neurology center between 2002 and 2019 were characterized. RESULTS: Fifty-four children fulfilled EPC criteria. Median age at onset was 7 years (range 0.6-15), with median follow-up of 4.3 years (range 0.2-16). The diagnosis was Rasmussen encephalitis (RE) in 30 of 54 (56%), a mitochondrial disorder in 12 of 54 (22.2%), and magnetic resonance imaging (MRI) lesion-positive focal epilepsy in 6 of 54 (11.1%). No diagnosis was made in 5 of 54 (9%). Children with mitochondrial disorders developed EPC earlier; each additional year at presentation reduced the odds of a mitochondrial diagnosis by 26% (P = .02). Preceding developmental concerns (odds ratio [OR] 22, P < .001), no seizures prior to EPC (OR 22, P < .001), bilateral slowing on electroencephalogram (EEG) (OR 26, P < .001), and increased cerebrospinal fluid (CSF) protein level (OR 16) predicted a mitochondrial disorder. Asymmetry or hemiatrophy was evident on MRI at presentation with EPC in 18 of 30 (60%) children with RE, and in the remainder at a median of 6 months (range 3-15) after EPC onset. The first diagnostic test is brain MRI. Hemiatrophy may permit a diagnosis of RE with unilateral clinical and EEG findings. For children in whom a diagnosis of RE cannot be made on first scan but the clinical and radiological presentation resembles RE, repeat imaging every 6 months is recommended to detect progressive unicortical hemiatrophy, and brain biopsy should be considered. Evidence of intrathecal inflammation (oligoclonal bands and raised neopterin) can be supportive. In children with bihemispheric EPC, rapid polymerase gamma testing is recommended and if negative, sequencing mtDNA and whole-exome sequencing on blood-derived DNA should be performed. SIGNIFICANCE: Children presenting with EPC due to a mitochondrial disorder show clinical features distinguishing them from RE and structural epilepsies. A diagnostic algorithm for children with EPC will allow targeted investigation and timely diagnosis.

Lingual epilepsia partialis continua: a detailed video-EEG and neuroimaging study.

Motor epilepsia partialis continua (EPC) is a frequent and widely described variant of simple focal motor status epilepticus. However, lingual EPC is an unusual epileptic condition. We present a case of lingual EPC secondary to low-grade glioma in which the EEG and neuroimaging features were particularly remarkable. The video-EEG showed lateralized periodic discharges with superimposed rhythmic activity and frequent recurrent focal epileptic seizures. Moreover, brain magnetic resonance imaging showed a right temporo-insular cortico-subcortical lesion which was hyperintense on FLAIR, suggestive of low-grade glioma. In addition, diffusion-weighted imaging and arterial spin labelling series showed restricted diffusion in the right temporo-insular and parietal cortex and increased cerebral flow, respectively. All these findings are in keeping with changes related to persistent focal status epilepticus. Finally, we review the literature and discuss the differential diagnosis of this rare epileptic entity. [Published with video sequence].

Infantile epilepsy with multifocal myoclonus caused by TBC1D24 mutations.

PURPOSE: To summarize the clinical features and neuroimaging changes of epilepsy associated with TBC1D24 mutations. METHODS: Genetic testing was conducted in all epilepsy patients without acquired risk factors for epilepsy. Epilepsy patients identified with TBC1D24 compound heterozygous mutations by next-generation sequencing (NGS) epilepsy panel or whole exome sequencing (WES) were enrolled. The enrolled patients were followed up to summarize the clinical features. RESULTS: Nineteen patients were identified with TBC1D24 compound heterozygous mutations. Nine patients carried the same pathogenic variant c.241_252del. The seizure onset age ranged from 1 day to 8 months of age (median age 75 days). The most prominent features were multifocal myoclonus and epilepsia partialis continua (EPC). Myoclonus could be triggered by fever or infection in 15 patients, and could be terminated by sleep or sedation drugs. Psychomotor developmental delay was presented in 11 patients. Six patients exhibited hearing loss. Brain MRIs were abnormal in eight patients. Twelve patients were diagnosed with epilepsy syndromes including one patient who was diagnosed with Dravet syndrome. Two patients died due to status epilepticus at 4 months and 19 months of age, respectively. CONCLUSION: TBC1D24 mutation related epilepsy was drug-resistant. Multifocal myoclonus, EPC, and fever-induced seizures were common clinical features. Most patients presented psychomotor developmental delay. The neuroimaging abnormality and hearing loss could exacerbate during follow-up.

A case of epilepsia partialis continua of abdominal muscles after brain tumor surgery.

Epilepsia partialis continua (EPC) is a rare form of focal motor status epilepticus characterized by continuous muscular twitches or jerks involving a limited part of the body, usually facial region and distal limb. Although the cerebrovascular disease is known to be one of the most common causes of this condition, other reported cases with predominant abdominal involvement have different aetiologies, including, tumors, focal cortical dysplasia, and central nervous system infections. No cases of epilepsia partialis continua of the abdominal wall occurred after brain surgery have been previously reported. We describe the clinical, electrophysiological, and neuroimaging findings in an adult patient presenting with persistent unilateral abdominal myoclonus configuring an EPC as the evolution of a super-refractory hemibody convulsive status epilepticus, occurred after brain tumor surgery.

Rasmussen syndrome: an atypical presentation in ten patients.

The aim of this study was to analyse the electroclinical and imaging findings and outcome of patients with Rasmussen syndrome (RS) with atypical manifestations. We conducted a retrospective, descriptive study of 10 of 44 consecutive patients with RS with atypical features, followed between 1999 and 2017. Six patients were boys and four were girls. The mean and median ages at onset of the seizures were 8.8 and 6.5 years, respectively (range: 4.6-13 years). All of the patients except one had seizures. Eight patients (80%) had epilepsia partialis continua that started at a mean age of 7.5 years (range: 7-15 years). In our series, hemiparesis without seizures was the first manifestation in three patients, one of whom had dual pathology. In two patients, the first manifestation was dyskinetic movements, followed by delayed-onset seizures associated with unilateral caudate atrophy. Two patients had a focal lesion mimicking focal cortical dysplasia as the first MRI abnormality; one of these two patients had epileptic spasms in clusters. Bilateral cerebral hemisphere involvement was observed in three patients during the course of the disease. Six of eight patients responded well to surgical treatment. Progressive hemiparesis alone or with delayed-onset seizures, dyskinetic movements associated with seizures, a focal lesion mimicking focal cortical dysplasia, and bilateral brain involvement were the atypical features recognized. Our series of patients responded well to surgery. Clinical, video-EEG, and neuroradiological follow-up is important for early confirmation of RS in order to initiate adequate management of the condition.

Neuropsychological assessment as an objective tool to monitor treatment response in anti-N-methyl-D-aspartate receptor encephalitis.

We report a 1-year follow-up of a young woman with anti-N-methyl-D-aspartate receptor encephalitis. Management of autoimmune encephalitis remains challenging as objective and clinically relevant biomarkers are sought, which allow for the monitoring of treatment response. While further investigation is required, we believe that this case highlights the importance of following a comprehensive neuropsychological profile as a clinically relevant biomarker to guide therapeutic decision-making. By relying on the neuropsychological assessment of the patient, treatment with more toxic medications was avoided and her antiepileptic drug regimen was simplified.

Epilepsia partialis continua: Correlation of semiology, outcome and electrophysiologic features.

OBJECTIVE: Epilepsia partialis continua (EPC) is a special form of cortical epilepsy. Several studies have described the ictal and interictal electroencephalography (EEG) findings in patients with EPC; however, lateralizing and localizing values of these findings have been evaluated rarely. This study investigated the correlation of semiologic and EEG findings, and outcomes in patients with EPC. PATIENTS AND METHODS: Clinical and EEG findings and outcomes, and their correlations were studied prospectively in 15 patients who were diagnosed as having EPC upon presentation to Istanbul Medical Faculty Hospital between January 2010 and April 2014, and retrospectively in 5 previously evaluated patients. RESULTS: EEG findings were lateralizing in 11 (47.8%) of the overall 23 EEG recordings, 7 (30.4%) of which were also localizing. Eleven (55%) of the 20 patients had poor prognosis. Patients with interictal lateralizing EEG findings had better outcomes compared with patients who had interictal non-lateralizing EEG findings (P = 0.016). Periodic epileptiform discharges (PEDs) were noted in the 6 EEGs (3 ictal EEGs and 3 interictal EEGs) of 5 patients, all of whom had poor outcomes (P = 0.04). All four patients with false lateralizing EEG findings had poor outcomes. CONCLUSION: EEG has low lateralizing and localizing value in EPC but patients with interictal lateralizing EEG findings have better outcomes, which may be used as a prognostic tool in EPC. The presence of PEDs and false lateralizing findings in EEG might be associated with poor prognosis in EPC.

Bottom-of-sulcus focal cortical dysplasia presenting as epilepsia partialis continua multimodality characterization including 7T MRI.

INTRODUCTION: Bottom-of-sulcus focal cortical dysplasias are an under recognized, surgically treatable cause of focal epilepsy. Resection can dramatically reduce the seizure burden for children with refractory epilepsy, or eliminate seizures altogether. MATERIAL AND METHODS: We report the case and present the results of multimodality evaluation of a 15-year-old young man who presented with long-standing partial epilepsy affecting his right leg, which over the years became refractory to therapy. RESULTS: High-resolution 3T MRI images acquired as a dedicated epilepsyprotocol were initially interpreted as unremarkable. On further review by an experienced specialist aware of clinical and electroencephalographic findings, a subtle focal cortical dysplasia was identified at the bottom of a sulcus near the medial aspect of the left precentral gyrus. After confirmation of the extent of the lesion with PET and ultra-high field 7T MRI, the patient underwent cortical mapping and focal resection and remains free of seizures. COCLUSIONS: This case emphasizes the need for a multidisciplinary approach to the evaluation of refractory focal epilepsy in children and highlights the potential role of ultra-high field 7T MRI in identifying the often subtle causative anatomic abnormalities.