A child with refractory complex partial seizures, right temporal ganglioglioma, contralateral continuous electrical status epilepticus, and a secondary Landau–Kleffner autistic syndrome

doi:10.1016/j.yebeh.2008.06.006

Epilepsy & Behavior

Volume 14, Issue 2, February 2009, Pages 411-417

https://doi.org/10.1016/j.yebeh.2008.06.006 Get rights and content

Abstract

A 7-year-old, right-handed girl started to have seizures at age 1 year 4 months. She developed normally until age 4 when she had worsening of seizures with auditory verbal agnosia, complete aphasia, and a behavioral disorder fulfilling the diagnostic criteria of autism. Medical therapy failed. MRI revealed a right temporal tumor. Video/EEG monitoring at age 7 showed contralateral electrical status epilepticus in wakefulness and sleep and ipsilateral onset of seizures. Resection (ganglioglioma with excessive inflammation) resulted in seizure freedom and marked reduction of the autistic features. This case is unique for being, to our knowledge, (1) the first in which a lesion located in the right, rather than left, temporal lobe resulted in secondary falsely localizing left temporal lobe electrical status epilepticus with a clinical picture of Landau–Kleffner syndrome and autism, and (2) the fourth reported patient with lesional Landau–Kleffner syndrome to respond to resective surgery.

Introduction

Landau–Kleffner syndrome (LKS) is an epilepsy syndrome involving progressive neuropsychological impairment related to the appearance of paroxysmal electroencephalographic activity. It is characterized by acquired aphasia and paroxysmal, sleep-activated electroencephalographic paroxysms predominating over the temporal or parieto-occipital regions. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy [1], [2], [3].

Most of the literature on the surgical treatment of LKS is about multiple subpial transection [4], [5], [6]. Other surgical procedures including tumor resection and temporal lobectomy have been successfully used in the treatment of LKS, to our knowledge, in only five previously reported cases [7], [8], [9], [10].

Inflammation of epileptic cortical tissue has been described in neuropathology after seizures in experimental models and in clinical cases of epilepsy, even in cases that clearly involve congenital lesions such as cortical dysplasia [11], [12], [13].

We describe here a girl with LKS who also fulfilled the criteria for autism with left temporal electrical status epilepticus, the underlying cause of which was a contralateral right temporal, excessively inflamed ganglioglioma; after surgical resection she became seizure free, with improvement of the autistic features, speech, and cognitive function.

Section snippets

Case report

Our patient is a 7-year-old girl with no family history of neurological disease and with nonconsanguineous parents. She was the product of a full-term pregnancy and normal vaginal delivery, with normal health and development until the age of 1 year 4 months, when she started to have seizures that usually lasted about 30 seconds and recurred about once every 15 days. The seizures, by report, consisted of initial cyanosis of the face and injection of the eyes followed by pallor, loss of

Discussion

Our patient’s clinical picture represents an unusual para-ictal encephalopathy manifesting all the essential features of LKS and also fulfilling diagnostic criteria of autistic disorder except that it started after 4 years of normal development. Thus, she probably is best considered a patient with LKS variant [14]. The semiology of her seizures, as well as her MRI picture, was consistent with a right temporal epileptogenic zone; however, her EEG revealed at the time of her evaluation a falsely

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Using advanced search terms, a systematic review of electronic databases was conducted, comprising MEDLINE, Embase, PsycINFO, Global Health, Web of Science and CINAHL. Included studies reported on outcome following neurosurgical treatment for epilepsy. Specifically, studies were included if they reported neuropsychological outcomes and were concerned only with temporal lobe resection.
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The use of subpial transcortical transection in patients with LKS has been reported to result in improved speech.38 Use of corpus callosotomy, hemispherotomy, and resective surgeries has also shown to help improve ESES and encephalopathy in selected patients with ESES.39,40 Dravet syndrome is an epilepsy syndrome that starts during the first year of life and is characterized by prolonged febrile hemiclonic and generalized clonic seizures often with occurrence of status epilepticus and psychomotor delay.
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The clinical seizures of LKS often respond well to treatment with antiepileptic drugs, but the speech and language difficulties often persist despite seizure control.36 Rare cases can be secondary to structural lesions such as benign temporal lobe tumors with improvement after focal resection.37 LGS is a usually devastating pediatric epilepsy syndrome, characterized by multiple seizure types, mental retardation or regression, and abnormal findings on EEG, with paroxysms of polyspike as electrode incremental fast activity, generalized slow spike and wave discharges (1.5-2 Hz) and a slow background (Fig 3).
Pediatric epileptic encephalopathies represent a clinically challenging and often devastating group of disorders that affect children at different stages of infancy and childhood. With the advances in genetic testing and neuroimaging, the etiologies of these epileptic syndromes are now better defined. The various encephalopathies that are reviewed in this article include the following: early infantile epileptic encephalopathy or Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West syndrome, severe myoclonic epilepsy in infancy (Dravet syndrome), Landau-Kleffner syndrome, Lennox-Gastaut syndrome, and epileptic encephalopathy with continuous spike-and-wave during sleep. Their clinical features, prognosis as well as underlying genetic etiologies are presented and updated.
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A review of the relationships between Landau-Kleffner syndrome, electrical status epilepticus during sleep, and continuous spike-waves during sleep
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After steroid therapy, 33% still showed ESES. In one rare case of a right temporal ganglioglioma [12], LKS was diagnosed, and unexpectedly, a contralateral electrical status epilepticus was recorded in both wakefulness and sleep. In summary, among patients with LKS, 40–50% showed ESES, and focal discharges were seen, especially in the frontal and temporal areas, in 70–100% of patients with behavior disorders.
The goal of this report is to review the relationships between Landau–Kleffner syndrome (LKS), electrical status epilepticus during sleep (ESES), and continuous spike–waves during sleep (CSWS). LKS is a clinical syndrome involving mainly acquired aphasia and sometimes seizures. Other clinical findings include cognitive impairments and global regression of behavior. The EEG may evolve from more benign conditions into ESES (or CSWS), seen in 50% of patients with LKS, or may also show focal findings. Seizures include atypical absence, generalized tonic–clonic, atonic, and partial motor attacks. Effective medications are discussed. The EEG patterns CSWS and ESES are likely equivalent terms. CSWS is used by some authors, and ESES by others. Patients with these patterns usually show mental retardation, seizures, and global regression. More benign EEG patterns, like focal discharges, may develop into these more severe generalized patterns, which are associated with atypical absences, negative myoclonus, and cognitive disturbances. Memory disorders are common, because the nearly continuous generalized discharges in sleep do not allow for the memory consolidation that also occurs during sleep. Medications and possible etiologies are discussed.

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Case ReportA child with refractory complex partial seizures, right temporal ganglioglioma, contralateral continuous electrical status epilepticus, and a secondary Landau–Kleffner autistic syndrome

Abstract

Introduction

Section snippets

Case report

Discussion

Pediatr Neurol

Epilepsy Behav

Pediatr Neurol

Brain Lang

Pediatr Neurol

Lancet Neurol

Pediatr Neurol

J Neurol Sci

Pediatr Neurol

Pediatr Neurol

Pediatr Neurol

Epilepsies in childhood: the Landau–Kleffner syndrome

Dev Med Child Neurol

The Landau–Kleffner syndrome

Epilepsy Curr

Management of Landau–Kleffner syndrome

Paediatr Drugs

Multiple subpial transection: a new approach to the surgical treatment of focal epilepsy

J Neurosurg

Multiple subpial transaction: the Yale experience

Epilepsia

Multiple subpial transections in the treatment of pediatric epilepsy

J Neurosurg (Pediatrics 2)

The Landau–Kleffner syndrome of acquired epileptic aphasia: unusual clinical outcome, surgical experience, and absence of encephalitis

Neurology

Intracranial EEG monitoring in Landau–Kleffner syndrome associated with left temporal lobe astrocytoma

Epilepsia

Brain inflammation in epilepsy: experimental and clinical evidence

Epilepsia

Focal chronic inflammatory encephalopathy in a patient with malformations of cortical development, with a review of the spectrum of chronic inflammatory epileptic encephalopathy

Epilepsia

The role of inflammation in epilepsy

Curr Pediatr Rev

Landau–Kleffner syndrome: a clinical and EEG study of five cases

Epilepsia

The Landau–Kleffner syndrome

Case Report
A child with refractory complex partial seizures, right temporal ganglioglioma, contralateral continuous electrical status epilepticus, and a secondary Landau–Kleffner autistic syndrome