A Rare Cause of Epilepsy: Ulegyria Revisited in a Series of 10 Patients

2021 ◽  
pp. 155005942110433
Author(s):  
Aylin Bican Demir ◽  
Pinar Eser ◽  
Ahmet Bekar ◽  
Bahattin Hakyemez ◽  
İbrahim Bora
Keyword(s):  
Hippocampal Sclerosis ◽  
Age At Onset ◽  
Epileptic Seizures ◽  
Term Infants ◽  
Patients Âgés ◽  
Scalp Eeg ◽  
Imaging Characteristics ◽  
Pathology Reports ◽  
The One ◽  
Operative Notes

Introduction. Ulegyria results from perinatal hypoxic-ischemic brain injury in term infants. The specific mushroom-shaped configuration of ulegyria results from small atrophic circumvolutions at the bottom of a sulcus underlying an intact gyral apex. Clinically, ulegyria is generally associated with epilepsy. Here, we aimed to delineate the characteristics of patients with ulegyria and the epileptic seizures they experience. Material and methods. Medical records including radiology and pathology reports, video-electroencephalographic (EEG) analysis, operative notes, hospital progress and outpatient clinic notes were reviewed retrospectively in a total of 10 ulegyria patients. Results. Patients ages ranged between 24 and 58 years (mean, 32 ± 9.8 years). Past medical history was confirmed for neonatal asphyxia in 2 (20%). Neurological examination was remarkable for spastic hemiparesis in 1 (10%) patient with perisylvian ulegyria and for visual field deficits in 2 patients (20%) with occipital ulegyria. Ulegyria most commonly involved the temporoparietal region (n = 5, 50%) followed by the perisylvian area (n = 2, 20%). Except the one with bilateral perisylvian ulegyria, all patients had unilateral lesions (n = 9, 90%). Hippocampal sclerosis accompanied ulegyria in 2 patients (20%). All patients experienced epileptic seizures. Mean age at seizure onset was 8.8 ± 5.4 years (range, 2-20 years). Interictal scalp EEG and EEG-video monitoring records demonstrated temporoparietal and frontotemporal activities in 5 (50%) and 2 (20%) patients, respectively. The seizures were successfully controlled by antiepileptic medication in 8 patients (n = 8, 80%). The remaining 2 patients (%20) with concomitant hippocampal sclerosis required microsurgical resection of the seizure foci due to medically resistant seizures. Discussion. Ulegyria is easily recognized with its unique magnetic resonance imaging characteristics and clinical presentation in the majority of cases. It is highly associated with either medically resistant or medically controllable epileptic seizures. The treatment strategy depends on the age at onset and extends of the lesion that has a significant impact on the severity of the clinical picture.

scholarly journals Autoimmune Global Amnesia as Manifestation of AMPAR Encephalitis and Neuropathologic Findings

2021 ◽  
Vol 8 (4) ◽  
pp. e1019
Author(s):  
Gerda Ricken ◽  
Tobias Zrzavy ◽  
Stefan Macher ◽  
Patrick Altmann ◽  
Johannes Troger ◽  
...  
Keyword(s):  
Psychiatric Symptoms ◽  
Hippocampal Sclerosis ◽  
Disease Onset ◽  
Epileptic Seizures ◽  
Antibody Testing ◽  
Fluid Attenuated Inversion Recovery ◽  
Global Amnesia ◽  
The One ◽  
Mri Changes ◽  
Inflammatory Infiltrates

ObjectiveTo report an unusual clinical phenotype of alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis and describe associated neuropathologic findings.MethodsWe retrospectively investigated 3 AMPAR encephalitis patients with autoimmune global hippocampal amnesia using comprehensive cognitive and neuropsychologic assessment, antibody testing by in-house tissue-based and cell-based assays, and neuropathologic analysis of brain autopsy tissue including histology and immunohistochemistry.ResultsThree patients presented with acute-to-subacute global amnesia without affection of cognitive performance, attention, concentration, or verbal function. None of the patients had epileptic seizures, change of behavior, personality changes, or psychiatric symptoms. The MRI was normal in 1 patient and showed increased fluid-attenuated inversion recovery/T2 signal in the hippocampus in the other 2 patients. Two patients showed complete remission after immunotherapy. The one patient who did not improve had an underlying adenocarcinoma of the lung and died 3.5 months after disease onset because of tumor progression. Neuropathologic analysis of the brain autopsy revealed unilateral hippocampal sclerosis accompanied by mild inflammatory infiltrates, predominantly composed of T lymphocytes, and decrease of AMPAR immunoreactivity.ConclusionAMPAR antibodies usually associate with limbic encephalitis but may also present with immune responsive, acute-to-subacute, isolated hippocampal dysfunction without overt inflammatory CSF or MRI changes.

Ictal Hyperperfusion Patterns in Relation to Ictal Scalp EEG Patterns in Patients with Unilateral Hippocampal Sclerosis: A SPECT Study

Epilepsia ◽  
2007 ◽  
Vol 48 (2) ◽  
pp. 270-277 ◽  
Author(s):  
Ji Hyun Kim ◽  
Ki Chun Im ◽  
Jae Seung Kim ◽  
Sang-Ahm Lee ◽  
Jung Kyo Lee ◽  
...  
Keyword(s):  
Hippocampal Sclerosis ◽  
Scalp Eeg

scholarly journals Neurocysticercosis as a probable risk factor for hippocampal sclerosis

2018 ◽  
Vol 76 (11) ◽  
pp. 783-790 ◽  
Author(s):  
Gagandeep Singh ◽  
Josemir W. Sander
Keyword(s):  
Hippocampal Sclerosis ◽  
Taenia Solium ◽  
Population Based ◽  
Temporal Lobectomy ◽  
Epileptogenic Zone ◽  
Drug Resistant ◽  
Common Risk Factors ◽  
Longitudinal Imaging ◽  
Drug Resistant Epilepsy ◽  
Pathology Reports

ABSTRACT Neurocysticercosis is one of the most common risk factors for epilepsy but its association with drug-resistant epilepsy remains uncertain. Conjectures of an association with drug-resistant epilepsy have been fueled by reports of an association between calcific neurocysticercosis lesions (CNL) and hippocampal sclerosis (HS) from specialized epilepsy centers in Taenia solium-endemic regions. The debate arising from these reports is whether the association is causal. Evidence for the association is not high quality but sufficiently persuasive to merit further investigation with longitudinal imaging studies in population-based samples from geographically-diverse regions. The other controversial point is the choice of a surgical approach for drug-resistant epilepsy associated with CNL-HS. Three approaches have been described: standard anteromesial temporal lobectomy, lesionectomy involving a CNL alone and lesionectomy with anteromesial temporal lobectomy (for dual pathology); reports of the latter two approaches are limited. Presurgical evaluation should consider possibilities of delineating the epileptogenic zone/s in accordance with all three approaches.

Stargardt and Fundus Flavimaculatus; Pathophysiology, Clinical Findings, Imaging Characteristics, and Diagnosis

2021 ◽  
pp. 185-189
Keyword(s):  
Visual Acuity ◽  
Fundus Autofluorescence ◽  
Age At Onset ◽  
Pattern Electroretinogram ◽  
Clinical Findings ◽  
Macular Dystrophy ◽  
Macular Lesion ◽  
Imaging Characteristics ◽  
Wide Variability ◽  
Severity Of The Disease

Stargardt disease (STGD) is the most common form of recessively inherited macular dystrophy. It is characterized by the presence of an atrophic macular lesion, which is surrounded by irregular, white-yellow, deep retinal lesions (flecks). There is wide variability in age at onset, visual acuity, fundus appearance, and severity of the disease. Fundus examination can be normal but visual acuity can be reduced early in the course of the disease. In these patients, pattern electroretinogram (PERG) and fundus autofluorescence (FAF) will be helped in establishing the diagnosis of STGD. The typical sign of “choroidal silence” or dark choroid on fluorescein angiography (FA) is not present in all patients with STGD and is not specific to this condition.

Classification of seizures and epilepsy

2019 ◽  
pp. 935-944
Author(s):  
Andrew McEvoy ◽  
Tim Wehner ◽  
Victoria Wykes
Keyword(s):  
Focal Epilepsy ◽  
Focal Cortical Dysplasia ◽  
Hippocampal Sclerosis ◽  
Epileptic Seizures ◽  
Recurrence Risk ◽  
Medical Emergency ◽  
Unprovoked Seizure ◽  
Generalized Seizures ◽  
The Brain

Epileptic seizures are transient neurologic alterations due to abnormal excessive or synchronous neuronal cerebral activity. They may cause subjective symptoms (aura), and objective autonomic, behavioural, or cognitive alterations in any combination. Focal seizures are initially generated in one circumscribed area in the brain, whereas generalized seizures involve bihemispheric neuronal networks from the seizure onset. Epilepsy is a brain disease defined by the occurrence of two unprovoked seizures more than 24 h apart or one unprovoked seizure with underlying pathological or genetic factors resulting in a similar recurrence risk. Focal epilepsy syndromes are best classified by aetiology or anatomical area of origin. A seizure that does not self-terminate results in status epilepticus, and constitutes a medical emergency that requires immediate treatment. Focal cortical dysplasia and hippocampal sclerosis are the commonest aetiologies of epilepsy amenable to surgical treatment and are reviewed here. The limbic pathway may be involved in seizure propagation, and the anatomy is described.

scholarly journals Seasonal variations in the occurrence of acute appendicitis and their relationship with the presence of fecaliths in children

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Yao-Jen Hsu ◽  
Yu-Wei Fu ◽  
Taiwai Chin
Keyword(s):  
Acute Appendicitis ◽  
Medical Records ◽  
Laboratory Data ◽  
Lymphoid Hyperplasia ◽  
Clinical Findings ◽  
Number Of Patients ◽  
Computed Tomography Scans ◽  
Pathology Reports ◽  
Operative Notes ◽  
Surgical Condition

Abstract Background Acute appendicitis (AA) is the most common surgical condition in children. Although a higher incidence of AA in summer has been reported, the reason for this observation remains unclear. The purpose of this study was to compare the clinical findings of AA patients who underwent appendectomies during the summer months with those who underwent the procedure during the non-summer months. Methods The clinical data of 171 patients who underwent appendectomy from January 2013 to December 2016 were reviewed. The patients were divided into a summer group (from May to October) and a non-summer group (from November to April) based on the month when appendectomy was performed. All patients were under 18 years of age at the time of surgery. The medical records including laboratory data, computed tomography scans, pathology reports and operative notes were reviewed. Results The number of patients with AA was higher in the summer group than in the non-summer group (101 vs. 70 patients). No significant differences in the laboratory results between the two groups of patients were observed. The percentage of AA patients who presented with a fecalith was significantly lower in the summer group (33.6%) than in the non-summer group (55.7%). No significant differences in the incidence of appendiceal perforations and abscesses, as well as postoperative complications were observed between the two groups. Conclusions The percentage of AA patients with fecaliths in summer was lower than that in the non-summer months. The increase in the number of AA patients in summer may be due to the increased occurrence of lymphoid hyperplasia, which may be correlated with the yearly outbreak of enterovirus infection during this period.

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