Meningioangiomatosis: an uncommon cause of focal epilepsy with characteristic neuroimaging and neuropathology

2021 ◽  
Vol 14 (6) ◽  
pp. e242953
Author(s):  
Kunle Oyedokun ◽  
Maha ME Agabna ◽  
Anil Israni ◽  
Daniel du Plessis
Keyword(s):  
Inflammatory Process ◽  
Focal Epilepsy ◽  
Brain Biopsy ◽  
Acute Onset ◽  
Precise Nature ◽  
Secondary Generalisation ◽  
Focal Seizures ◽  
Neoplastic Process ◽  
Process Decision

A 3-year-old boy presented with acute onset of prolonged right sided focal seizures with secondary generalisation. The investigation findings were suggestive of a neoplastic process more than an inflammatory process. Decision to perform brain biopsy from the lesion to establish the precise nature of lesion was undertaken.

Dramatic Onset of Focal Seizures: Differentiating Structural Focal Epilepsy from Ring Chromosome 20

2017 ◽  
Vol 48 (S 01) ◽  
pp. S1-S45
Author(s):  
A. Herting ◽  
T. Cloppenborg ◽  
A. Hofmann-Peters ◽  
T. Polster
Keyword(s):  
Focal Epilepsy ◽  
Ring Chromosome ◽  
Focal Seizures ◽  
Chromosome 20

scholarly journals Benign Focal Epilepsy of Childhood and Gastroesophagael Reflux

1994 ◽  
Vol 8 (1) ◽  
pp. 45-48
Author(s):  
Anne G Sheehan ◽  
Sherry Pelensky ◽  
Colin Van Orman ◽  
Steven R Martin
Keyword(s):  
Gastroesophageal Reflux ◽  
Sudden Infant Death Syndrome ◽  
Infant Death ◽  
Chronic Cough ◽  
Focal Epilepsy ◽  
Sudden Infant Death ◽  
Acute Onset ◽  
Sudden Infant ◽  
Recurrent Pneumonia ◽  
Essentially Normal

Gastroesophageal reflux has been associated with, and implicated in, a number of conditions, including respiratory disease (recurrent pneumonia, chronic cough, asthma), sudden infant death syndrome, dysphagia and central nervous disorders. An eight-year-old girl presented with an acute history that suggested gastroesophageal reflux. An esophageal motility study was abnormal and 24 h pH study demonstrated gastroesophageal reflux. Before the manometric study, a seizure was observed and subsequent neurological evaluation confirmed the diagnosis of benign focal epilepsy of childhood, which was treated with carbamazepine. The symptoms resolved after eight weeks and the repeat reflux investigations were essentially normal. Oropharyngeal symptoms are common in benign focal epilepsy of childhood, a condition which is very responsive to therapy. Symptoms suggestive of this diagnosis - acute onset, with unusual oropharyngeal sensations, or seizures-occurring mainly at night may initially be confused with gastroesophageal reflux. Benign focal epilepsy of childhood should be considered in reflux presenting outside infancy.

Orbital and Periorbital Cellulitis

1995 ◽  
Vol 16 (5) ◽  
pp. 163-167
Author(s):  
Keith R. Powell
Keyword(s):  
Inflammatory Process ◽  
Subcutaneous Tissue ◽  
Acute Onset ◽  
Orbital Cellulitis ◽  
The Body ◽  
Vein Thrombosis ◽  
Orbital Septum ◽  
Specific Complication ◽  
Thin Skin ◽  
Doctor's Office

The acute onset of eyelid redness and swelling in a child usually results in a quick visit to the doctor's office or an emergency room. The differential diagnosis for these signs ranges from relatively innocuous problems, such as allergy or an insect sting, to potentially vision-affecting or even life-threatening diseases, such as orbital cellulitis or cavernous vein thrombosis. The orbital contents often are protected from an inflammatory process by the orbital septum, a continuation of the periosteum of the bony orbit to the margins of both the upper and lower eyelids (Figure 1). An inflammatory process occurring in the structures superficial to the orbital septum is defined as preseptal or periorbital cellulitis; an inflammatory process in structures deep to the orbital septum is defined as orbital cellulitis of a specific complication thereof. Bacterial infection can cause both periorbital and orbital cellulitis. Another anatomic feature of importance is that the skin of the eyelid is the thinnest skin of the body. The subcutaneous tissue of the eyelid is composed of musculofibrous tissue and no fat. This combination of thin skin and loose subcutaneous tissues makes it possible for the eyelid to swell dramatically as it fills with edematous fluid. Epidemiology and Pathogenesis of Periorbital Cellulitis

scholarly journals Cerebral Amyloid Angiopathy: Multiple neurolgic problems

2017 ◽  
Vol 1 (3) ◽  
pp. 01-01
Author(s):  
Paul Gilbert
Keyword(s):  
White Matter ◽  
Cerebral Amyloid Angiopathy ◽  
Clinical Symptoms ◽  
Brain Biopsy ◽  
Acute Onset ◽  
Amyloid Angiopathy ◽  
Migraine Headaches ◽  
Diffuse White Matter ◽  
Cerebral Amyloid ◽  
Intravenous Steroids

A 72-Year-old female with a history of migraine headaches presented with an acute onset of expressive aphasia, difficulty with memory and worsening of her headaches. An MRI of the brain was done which revealed diffuse white matter T2 hyperintensities (Figures 1). Due to worsening of the patient’s clinical symptoms a repeat MRI was performed four days later that revealed multiple micro-bleeds (Figure 2), as well as a lobar hemorrhage in left temporal lobe (Figure 2). An extensive workup including HIV testing, CSF examination for infectious etiology including protein 14-3-3 and demylineating disease was negative. Paraneoplastic and autoimmune workup was also non-diagnostic. A brain biopsy was performed due to the extensive white matter disease, which revealed Cerebral Amyloid Angiopathy (CAA) with focal granulomatous angiitis. The patient was treated with intravenous steroids with no significant improvement clinically. Two months after diagnosis, her disease course has remained static, without improvement or deterioration.

scholarly journals Neuroimages and Neuropathology of a Stroke-Like Cerebral Lymphomatoid Granulomatosis

2020 ◽  
pp. 1-2
Author(s):  
Massimo Marano ◽  
Chiara Brunelli ◽  
Costanza De Marco ◽  
Maurizio Martini ◽  
Carlo Cosimo Quattrocchi ◽  
...  
Keyword(s):  
Brain Biopsy ◽  
Acute Onset ◽  
Lymphomatoid Granulomatosis ◽  
External Carotid ◽  
Left Internal Carotid Artery ◽  
Hyperintense Lesion ◽  
Fluent Aphasia ◽  
Total Body ◽  
The Right ◽  
Weighted Sequences

A 70-year-old man presented to the Emergency Department reporting the acute onset of non-fluent aphasia, hyposthenia, and hemi-anesthesia of the right body. Brain computerized tomography revealed a subcortical hypodense lesion in the middle cerebral artery territory. Neck ultrasounds of internal and external carotid arteries and of the vertebral arteries showed a focal moderate stenosis of the left internal carotid artery due to a soft atheromasic plaque. These findings that were initially consistent with a diagnosis of an ischemic stroke were not confirmed by magnetic resonance (MR). The latter showed an hyperintense lesion on FLAIR and T2-weighted sequences located in the left centrum semiovale, corona radiata, and thalamus, with a well-defined regular rim and a mild compressive effect on the lateral ventricle, with diffusivity restriction but without ADC reduction and with a punctate and serpiginous gadolinium enhancement on T1 sequences (Figure 1). Within the first day of observation, the patient started complaining progressive mental deterioration, in absence of any other possible causes, and a total body CT scan excluded any other organ involvement. Patient was then referred to the neurosurgeon in order to perform a brain biopsy. The neuropathology was compatible with the diagnosis of cerebral lymphomatoid granulomatosis (LG) (Figure 1).

scholarly journals Frequency of peri-ictal apnea and cardiac arrhythmias in epileptic seizures

2021 ◽  
Vol 57 (1) ◽  
Author(s):  
Rady Yousif ◽  
M. Ossama Abdulghani ◽  
A. Gaber ◽  
Naglaa El Khayat ◽  
Y. A. Abo Elnaga ◽  
...  
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Cardiac Arrhythmias ◽  
Focal Epilepsy ◽  
Epileptic Seizures ◽  
Electrode System ◽  
Seizure Onset ◽  
Unexpected Death ◽  
Focal Seizures ◽  
Epileptic Patients

Abstract Background Available data from witnessed and monitored sudden unexpected death in epilepsy (SUDEP) cases postulate that ictal central apnea (ICA) and ictal arrhythmias are the main causes of SUDEP. ICA is a frequent semiological feature of focal epilepsy and occasionally the only clinical manifestation of focal seizures. The aim of this study was to assess the frequency of ICA and cardiac arrhythmias in epileptic patients and to study the risk factors and predictors of their occurrence. Methods Fifty patients diagnosed with epilepsy were recruited in this study. All participants underwent prolonged surface video electroencephalography (VEEG) study using the 10-20 international electrode system with concomitant polysomnography including electrocardiography (ECG), heart rate monitoring, and peripheral capillary oxygen saturation (SpO2) using pulse oximetry. Also inductance plethysmography was used to record chest and abdominal excursions. Results Complete datasets were available in 50 patients and 112 seizures were recorded. ICA occurred exclusively in focal epilepsy (P <0.001). Temporal lobe epilepsy was associated with higher occurrence of ICA in comparison to extratemporal epilepsy (P <0.001). In addition, seizures lateralized to the left hemisphere were associated with higher occurrence of ICA (P <0.001). On the other side, tachycardia was found to be more associated with temporal lobe epilepsy and left hemispheric seizure onset (P <0.001). Conclusion ICA occurred exclusively in focal seizures and tachycardia magnitude was more with focal seizures, and both had higher percentage in temporal lobe epilepsy in comparison to other types and in seizures with left hemispheric onset.

scholarly journals Efficacy and safety of levetiracetam as adjunctive therapy for refractory focal epilepsy

2021 ◽  
Vol 79 (4) ◽  
pp. 290-298
Author(s):  
Maria Luiza Giraldes de MANREZA ◽  
Tatiane Amaral PAN ◽  
Eduardo Quinalha CARBONE ◽  
Antonio Carlos Amedeo VATTIMO ◽  
Renata HERRERA ◽  
...  
Keyword(s):  
Focal Epilepsy ◽  
Controlled Trial ◽  
Premature Mortality ◽  
Phase Iii ◽  
Adverse Event Rate ◽  
Control Group ◽  
Efficacy And Safety ◽  
Double Blind ◽  
Good Tolerability ◽  
Focal Seizures

ABSTRACT Background: Epilepsy affects about 50 million people worldwide and around 30% of these patients have refractory epilepsy, with potential consequences regarding quality of life, morbidity and premature mortality. Objective: The aim of treatment with antiseizure medications (ASMs) is to allow patients to remain without seizures, with good tolerability. Levetiracetam is a broad-spectrum ASM with a unique mechanism of action that differs it from other ASMs. It has been shown to be effective and safe for treating adults and children with epilepsy. Methods: This was a phase III, multicenter, randomized, double-blind, placebo-controlled trial to evaluate the efficacy and safety of levetiracetam in children and adults (4-65 years) as an adjuvant treatment for focal-onset seizures. It was conducted among 114 patients undergoing treatment with up to three ASMs. The primary efficacy analysis was based on the proportion of patients who achieved a reduction of ≥ 50% in the mean number of focal seizures per week, over a 16-week treatment period. The patients were randomized to receive placebo or levetiracetam, titrated every two weeks from 20 mg/kg/day or 1,000 mg/day up to 60 mg/kg/day or 3,000 mg/day. Results: Levetiracetam was significantly superior to placebo (p = 0.0031); 38.7% of the participants in the levetiracetam group and 14.3% in the control group shows reductions in focal seizures. Levetiracetam was seen to have a favorable safety profile and an adverse event rate similar to that of placebo. Conclusion: Corroborating the results in the literature, levetiracetam was shown to be effective and safe for children and adults with refractory focal-onset epilepsy.

TMOD-20. CASE REPORT: THE ODYSSEY TO THE RIGHT DIAGNOSIS. SURPRISING GLIOBLASTOMA MIMICS

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi219-vi220
Author(s):  
Patrick Dorin ◽  
Wolfgang Jochum ◽  
Arno Lauber ◽  
Marian Christoph Neidert ◽  
Thomas Hundsberger
Keyword(s):  
Limbic Encephalitis ◽  
Brain Mri ◽  
Brain Biopsy ◽  
Immunoglobulin Therapy ◽  
Tumor Stage ◽  
Acute Onset ◽  
Blurred Vision ◽  
Intravenous Immunoglobulin Therapy ◽  
Autoimmune Limbic Encephalitis

Abstract A 57-year-old female presented at the emergency room with acute onset aphasia but unremarkable cCT (incl. angiography and perfusion). Suspecting an ischemic stroke, she received thrombolysis with quick recovery of aphasia. Afterwards, myoclonic jerks of the face and right arm occurred leading to anticonvulsant therapy. Follow-up cMRI surprisingly demonstrated swollen T2w-hyperintense and Gd-enhancing left limbic, temporal and frontal lobes. Suspected herpes-simplex-encephalitis was treated with aciclovir despite unremarkable CSF results (no pleocytosis, no BBB disruption, negative HSV-PCR) on day 2 and follow-up (day 5). Due to persisting cognitive deficits, autoimmune limbic encephalitis was suspected, and intravenous immunoglobulin therapy was added. Three weeks later, she experienced new neurological symptoms (weakness, blurred vision, vomiting, headache). Follow-up brain MRI demonstrated a massive increase of multifocal Gd-enhancing lesions. Partial resection revealed the diagnosis of an IDH-wildtype glioblastoma (GB). Next generation oncogene panel testing demonstrated a GOPC-ROS1 fusion which is rarely found in GB. Due to the gliomatosis-like infiltration of both hemispheres, radiotherapy was deemed to be too toxic. Instead, she received two cycles of lomustine in absence of a MGMT-promotor methylation. Two months later cMRI showed a symptomatic second multilocular progression. 2nd-line therapy with a ROS-inhibitor was rejected, whereupon she died five weeks later. Our case is in several aspects peculiar: It demonstrates that rare GB-mimics (i.e. HSV- and autoimmune limbic encephalitis) can only be ruled out in a fast manner by brain biopsy. Watchful waiting may neglect fast progression of GB leading to the inability to provide optimal treatment (i.e. radiotherapy). Thrombolysis is strictly contraindicated in primary brain tumors, but was unharmful in our case most probably to the early tumor stage without relevant neoangiogenesis. Rare genetic abnormalities like ROS1-fusions which are reported mostly in childhood glioblastoma may be present and serve as a therapeutic target also in adult GB.

scholarly journals Recurrent ominous spinal mass

2019 ◽  
Vol 5 (3) ◽  
pp. 75
Author(s):  
Maria Gollwitzer ◽  
Wolfgang Hasenauer ◽  
Anja Grimmer ◽  
Andreas Gruber
Keyword(s):  
Patient Management ◽  
Inflammatory Process ◽  
Low Dose ◽  
Recurrent Disease ◽  
Underlying Disease ◽  
Acute Onset ◽  
Hypertrophic Pachymeningitis ◽  
Rare Form ◽  
Acute Patient ◽  
Spinal Mri

<p class="abstract">Hypertrophic pachymeningitis is a very rare form of diffuse inflammatory dural fibrosis. It is typically encountered intracranially rather than spinally and can have a progressive and recurrent course as encountered in our patient. The aetiology of this disorder is multifactorial. While acute management of hypertrophic pachymeningitis consists of immediate decompression in conjunction with steroid medication and treatment of the underlying disease, the management of chronic and recurrent disease is controversially debated in the current literature. A previously healthy 49-year-old woman presented with progressive back pain and acute onset of incomplete paraplegia. Spinal MRI showed a large ventral semicircular mass with intense contrast enhancement from Th3-8. Acute patient management consisted of immediate decompression and debulking the space-occupying lesion in conjunction with antibiotic and steroid therapy. Histopathologic examination of the surgical specimens revealed a chronic inflammatory process. The patient fortunately recovered from the severe paraparesis and upon discharged, spinal MRI disclosed a further regressing residual spinal mass. Over time, our patient suffered two times a relapse and was finally treated with Methotrexate and low-dose steroids. Under this medication she has made a nearly complete neurological recovery. This case report highlights a rare disease with challenging management, which should be considered diagnostically in patients with intraspinal space-occupying lesions. In our experience combination of Methotrexate and low-dose steroids might be an effective and safe treatment.</p>

scholarly journals Acute Onset Focal Epilepsy Mimicking Stroke

Cureus ◽  
2021 ◽  
Author(s):  
Marilena Mangiardi ◽  
Sabrina Anticoli ◽  
Luca Bertaccini ◽  
Valeria Cozzolino ◽  
Francesca Romana Pezzella
Keyword(s):  
Focal Epilepsy ◽  
Acute Onset
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