Efficacy of Surgical Treatment of De Novo, Adult-Onset, Cryptogenic, Refractory Focal Status Epilepticus

We present a case study of a 67-year-old man who presented with a new onset of recurrent tonic-clonic seizures. He had tested positive to gamma-aminobutyric acid B receptor antibodies in his blood and cerebrospinal fluid, and subsequent CT imaging and transrectal biopsy confirmed the presence of a locally advanced mixed small cell and Gleason 9 adenocarcinoma of the prostate. His seizures remained resistant to treatment with multiple antiepileptic drugs, including sodium valproate, clobazam, topiramate, carbamazepine, phenytoin and lacosamide. He progressed to status epilepticus, which required intravenous immunoglobulin and steroids, followed by plasma exchange 1 week later. The status epilepticus was refractory and required multiple admissions to the intensive care unit.

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A De Novo Dominant Negative Mutation in DNM1L Causes Sudden Onset Status Epilepticus with Subsequent Epileptic Encephalopathy

Neuropediatrics ◽

10.1055/s-0039-1685217 ◽

2019 ◽

Vol 50 (03) ◽

pp. 197-201

Author(s):

S. Schmid ◽

M. Wagner ◽

C. Goetz ◽

C. Makowski ◽

P. Freisinger ◽

...

Keyword(s):

Status Epilepticus ◽

Missense Mutation ◽

De Novo ◽

Mitochondrial Fission ◽

Refractory Status Epilepticus ◽

Sudden Onset ◽

Epileptic Encephalopathy ◽

Neurologic Outcome ◽

Dominant Negative Mutation ◽

Refractory Status

AbstractMitochondrial dynamics such as fission and fusion play a vital role in normal brain development and neuronal activity. DNM1L encodes a dynamin-related protein 1 (Drp1), which is a GTPase essential for proper mitochondrial fission. The clinical phenotype of DNM1L mutations depends on the degree of mitochondrial fission deficiency, ranging from severe encephalopathy and death shortly after birth to initially normal development and then sudden onset of refractory status epilepticus with very poor neurologic outcome. We describe a case of a previously healthy 3-year-old boy with a mild delay in speech development until the acute onset of a refractory status epilepticus with subsequent epileptic encephalopathy and very poor neurologic outcome. The de novo missense mutation in DNM1L (c.1207C > T, p.R403C), which we identified in this case, seems to determine a unique clinical course, strikingly similar to four previously described patients in literature with the identical de novo heterozygous missense mutation in DNM1L.

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De novo status epilepticus during influenza B infection: when follow-up matters

Epilepsy & Behavior Reports ◽

10.1016/j.ebr.2020.100413 ◽

2020 ◽

pp. 100413

Author(s):

G. Giovannini ◽

G. Turchi ◽

M. Mazzoli ◽

A.E. Vaudano ◽

S. Meletti

Keyword(s):

Status Epilepticus ◽

De Novo ◽

Influenza B

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Brain pathology in focal status epilepticus: evidence from experimental models

Neuroscience & Biobehavioral Reviews ◽

10.1016/j.neubiorev.2021.09.011 ◽

2021 ◽

Author(s):

Marco de Curtis ◽

Andrea O. Rossetti ◽

Diogo Vila Verde ◽

Erwin A. van Vliet ◽

Christine T. Ekdahl

Keyword(s):

Status Epilepticus ◽

Experimental Models ◽

Brain Pathology ◽

Focal Status Epilepticus

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De novo convulsive status epilepticus in patients with multiple sclerosis treated with dalfampridine

Multiple Sclerosis Journal ◽

10.1177/1352458518790379 ◽

2018 ◽

Vol 25 (4) ◽

pp. 618-621 ◽

Cited By ~ 3

Author(s):

Emilie Panicucci ◽

Mikael Cohen ◽

Veronique Bourg ◽

Fanny Rocher ◽

Pierre Thomas ◽

...

Keyword(s):

Multiple Sclerosis ◽

Status Epilepticus ◽

De Novo ◽

Case Reports ◽

Brain Magnetic Resonance Imaging ◽

Convulsive Status Epilepticus ◽

Biological Tests ◽

History Of ◽

Magnetic Resonance Imaging Mri ◽

History Of Epilepsy

Background: Dalfampridine extended release (DAL) is a broad-spectrum voltage-gated potassium channel blocker that is indicated in multiple sclerosis to improve the nerve conduction of demyelinated axons. Seizures are a known side effect of DAL, which is contraindicated in patients with a history of epilepsy. Objective: Three cases of multiple sclerosis (MS) with de novo convulsive status epilepticus (CSE) probably related to dalfampridine administration are described. Methods: No patients had a history of seizures or renal impairment. Biological tests were normal. A brain magnetic resonance imaging (MRI) showed diffuse cortical and subcortical atrophy without active inflammatory lesions. Results: All three patients presented with CSE that was attributed to DAL and so was discontinued. Conclusion: These case reports illustrate that, aside from seizures, de novo CSE is a potential complication of MS patients treated with DAL.

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