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Recurrent status epilepticus associated with Hashimoto's encephalopathy.

Visée H, Mabiglia C, Vanderaspoilden V, Gazagnes MD, Glibert G - Epilepsy Behav Case Rep (2013)

Bottom Line: This evolution was especially marked by the occurrence of steroid-refractory symptoms and a poor outcome with persistent cognitive and behavioral consequences.This patient highlights the risk of multiple relapses and the need for a long follow-up period.We describe her clinical and paraclinical features, compare this patient to similar case reports, and comment on her outcome.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Brugmann University Hospital, Brussels, Belgium.

ABSTRACT
Hashimoto's encephalopathy (HE) is an infrequent disease with no well-known physiopathology. Status epilepticus is rarely reported in association with HE. We describe the 7-year evolution of a young woman who presented with recurrent status epilepticus as the main complication of HE. This evolution was especially marked by the occurrence of steroid-refractory symptoms and a poor outcome with persistent cognitive and behavioral consequences. We point out that the frontal lobes are especially implicated in these symptoms. This patient highlights the risk of multiple relapses and the need for a long follow-up period. We describe her clinical and paraclinical features, compare this patient to similar case reports, and comment on her outcome.

No MeSH data available.


Related in: MedlinePlus

(A) Interictal EEG characterized by diffuse slowing and frontal spike–waves. (B) Ictal EEG characterized by subtle SE of frontal origin.
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f0010: (A) Interictal EEG characterized by diffuse slowing and frontal spike–waves. (B) Ictal EEG characterized by subtle SE of frontal origin.

Mentions: In 2006, a 26-year-old woman without relevant medical story developed tremor, confusion, agitation, and blurred vision during a few weeks. A first episode of refractory generalized convulsive status epilepticus (GCSE) followed and required anesthetic agents to improve. A prolonged postictal status with bradypsychia, ataxia, and tremor followed. The EEG was diffusely slowed. A slight right periventricular T2/FLAIR hyperintensity was observed on brain MRI. No toxic, metabolic, or infectious etiologies were identified. She recovered but promptly had a second episode of refractory GCSE despite oral phenytoin and required anesthetic agents again. The postictal period was prolonged anew with encephalopathy status. The EEG was diffusely slowed with occasional right frontal rhythmic theta activity. The brain Tc99m-bicisate SPECT reported right frontotemporal hypoperfusion and a focal right paramedian frontal hyperperfusion. A mild elevation of protein content without oligoclonal bands was found in the cerebrospinal fluid. The autoimmune screening in the serum disclosed high titers of antithyroglobulin (274 IU/mL, normal < 60 IU/mL) and antithyroperoxidase (602.8 IU/mL, normal < 60 IU/mL) antibodies, with euthyroid status. Thyroid ultrasound was compatible with thyroiditis status. Full body PET scan and CT scan did not reveal neoplastic disease. Onconeural antibodies (anti-HU, anti-Yo, and anti-Ri) were negative. The diagnosis of HE was probable. The EEG normalized rapidly after intravenous methylprednisolone (1 g/day), and the patient recovered. Phenytoin was discontinued. At this stage, her cognitive functions were almost normal with small deficits for free recall (episodic memory), selective attention, and verbal fluency (Table 1). Ten months later, steroids were progressively reduced because of side effects (osteoporosis, Cushing-like syndrome, diabetes). A generalized convulsive seizure relapsed rapidly. Phenytoin and steroids were discontinued after six courses of cyclophosphamide. For 3 years, she was free of treatment and seizures. Postural tremor, nervousness, and anxiety persisted. In 2010, she presented a short collapse without prolonged postictal status and a transient slowed EEG. Antiepileptic drug was transiently used. In 2011, she relapsed into a GCSE resistant to lormetazepam, phenytoin, levetiracetam, and intravenous methylprednisolone. A burst-suppression state was obtained with thiopental. For two months, it followed an encephalopathy state with subtle SE of frontal origin. Plasmapheresis was performed. Different antiepileptic drugs were used without success (phenytoin, phenobarbital, levetiracetam, lacosamide, and midazolam). The brain MRI revealed T2 and FLAIR bilateral frontal hyperintensity spreading to thalamic and mesiotemporal areas, without gadolinium enhancement (Fig. 1). The brain PET scan showed bilateral frontal hyperactivity. The EEG showed successively a drug-induced burst-suppression state, generalized periodic epileptiform discharges, a diffuse slowing with subtle seizures of frontal origin, and a progressive return of alpha rhythm (Fig. 2). Three months later, both EEG and brain MRI normalized. Two years later, frontal behavior, memory loss, and loss of autonomy persisted. The neuropsychological tests showed cognitive slowing, poor spontaneous speech, apathy, poor orientation, episodic memory loss, perseverations, confabulations, and impairment of visuospatial, attention, and executive functions (Table 1). Myoclonia and transient loss of consciousness relapse under 8 mg a day of oral methylprednisolone. In 2013, she continues to use chronically antiepileptic drugs (levetiracetam, phenytoin, and phenobarbital) and low dose of oral methylprednisolone.


Recurrent status epilepticus associated with Hashimoto's encephalopathy.

Visée H, Mabiglia C, Vanderaspoilden V, Gazagnes MD, Glibert G - Epilepsy Behav Case Rep (2013)

(A) Interictal EEG characterized by diffuse slowing and frontal spike–waves. (B) Ictal EEG characterized by subtle SE of frontal origin.
© Copyright Policy - CC BY-NC-SA
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4150643&req=5

f0010: (A) Interictal EEG characterized by diffuse slowing and frontal spike–waves. (B) Ictal EEG characterized by subtle SE of frontal origin.
Mentions: In 2006, a 26-year-old woman without relevant medical story developed tremor, confusion, agitation, and blurred vision during a few weeks. A first episode of refractory generalized convulsive status epilepticus (GCSE) followed and required anesthetic agents to improve. A prolonged postictal status with bradypsychia, ataxia, and tremor followed. The EEG was diffusely slowed. A slight right periventricular T2/FLAIR hyperintensity was observed on brain MRI. No toxic, metabolic, or infectious etiologies were identified. She recovered but promptly had a second episode of refractory GCSE despite oral phenytoin and required anesthetic agents again. The postictal period was prolonged anew with encephalopathy status. The EEG was diffusely slowed with occasional right frontal rhythmic theta activity. The brain Tc99m-bicisate SPECT reported right frontotemporal hypoperfusion and a focal right paramedian frontal hyperperfusion. A mild elevation of protein content without oligoclonal bands was found in the cerebrospinal fluid. The autoimmune screening in the serum disclosed high titers of antithyroglobulin (274 IU/mL, normal < 60 IU/mL) and antithyroperoxidase (602.8 IU/mL, normal < 60 IU/mL) antibodies, with euthyroid status. Thyroid ultrasound was compatible with thyroiditis status. Full body PET scan and CT scan did not reveal neoplastic disease. Onconeural antibodies (anti-HU, anti-Yo, and anti-Ri) were negative. The diagnosis of HE was probable. The EEG normalized rapidly after intravenous methylprednisolone (1 g/day), and the patient recovered. Phenytoin was discontinued. At this stage, her cognitive functions were almost normal with small deficits for free recall (episodic memory), selective attention, and verbal fluency (Table 1). Ten months later, steroids were progressively reduced because of side effects (osteoporosis, Cushing-like syndrome, diabetes). A generalized convulsive seizure relapsed rapidly. Phenytoin and steroids were discontinued after six courses of cyclophosphamide. For 3 years, she was free of treatment and seizures. Postural tremor, nervousness, and anxiety persisted. In 2010, she presented a short collapse without prolonged postictal status and a transient slowed EEG. Antiepileptic drug was transiently used. In 2011, she relapsed into a GCSE resistant to lormetazepam, phenytoin, levetiracetam, and intravenous methylprednisolone. A burst-suppression state was obtained with thiopental. For two months, it followed an encephalopathy state with subtle SE of frontal origin. Plasmapheresis was performed. Different antiepileptic drugs were used without success (phenytoin, phenobarbital, levetiracetam, lacosamide, and midazolam). The brain MRI revealed T2 and FLAIR bilateral frontal hyperintensity spreading to thalamic and mesiotemporal areas, without gadolinium enhancement (Fig. 1). The brain PET scan showed bilateral frontal hyperactivity. The EEG showed successively a drug-induced burst-suppression state, generalized periodic epileptiform discharges, a diffuse slowing with subtle seizures of frontal origin, and a progressive return of alpha rhythm (Fig. 2). Three months later, both EEG and brain MRI normalized. Two years later, frontal behavior, memory loss, and loss of autonomy persisted. The neuropsychological tests showed cognitive slowing, poor spontaneous speech, apathy, poor orientation, episodic memory loss, perseverations, confabulations, and impairment of visuospatial, attention, and executive functions (Table 1). Myoclonia and transient loss of consciousness relapse under 8 mg a day of oral methylprednisolone. In 2013, she continues to use chronically antiepileptic drugs (levetiracetam, phenytoin, and phenobarbital) and low dose of oral methylprednisolone.

Bottom Line: This evolution was especially marked by the occurrence of steroid-refractory symptoms and a poor outcome with persistent cognitive and behavioral consequences.This patient highlights the risk of multiple relapses and the need for a long follow-up period.We describe her clinical and paraclinical features, compare this patient to similar case reports, and comment on her outcome.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Brugmann University Hospital, Brussels, Belgium.

ABSTRACT
Hashimoto's encephalopathy (HE) is an infrequent disease with no well-known physiopathology. Status epilepticus is rarely reported in association with HE. We describe the 7-year evolution of a young woman who presented with recurrent status epilepticus as the main complication of HE. This evolution was especially marked by the occurrence of steroid-refractory symptoms and a poor outcome with persistent cognitive and behavioral consequences. We point out that the frontal lobes are especially implicated in these symptoms. This patient highlights the risk of multiple relapses and the need for a long follow-up period. We describe her clinical and paraclinical features, compare this patient to similar case reports, and comment on her outcome.

No MeSH data available.


Related in: MedlinePlus