Indian Pediatrics 1995;35:908-911 

Landau Kleffner Syndrome

M.S. Bhatia
Sanjay Khanna
Sandeep Choudhary

From the Department of Psychiatry, University College of Medical Sciences and Associated Guru Teg Bahadur Hospital, Dilshad Garden, Delhi 110 095, India.

Reprint requests: Dr. M.S. Bhatia, D-1, Naraina Vihar, New Delhi 110 028, India.

Manuscript Received: May 30, 1997; Initial review completed: June 16, 1997;
Revision Accepted: April 2, 1998.
 

The Landau-Kleffner syndrome also known as Worster-Drought syndrome is characterized by a triad of receptive and expressive aphasia, EEG abnormalities and, in a majority of cases, epileptic seizures in a previously normal child, usually a boy(1- 5). The first symptoms appear between 2 and 13 years of age, with a maximum incidence between three and seven years of
age. It begins abruptly or gradually, over a few days, weeks or months, before, after or at the same time as the epileptic seizures. Since the description of this syndrome, about 200 cases(6-12) have been described. The aim of presentation of the present case is to create awareness of this syndrome.

Case Report

A 14-year-old boy presented with history of recurrent seizures (one episode in every 2-4 days) of generalized tonic clonic type for last 5 years. First episode was pre- ceded by an episode of fever (moderate, without chills or rigors) which lasted for two days. He had been started with phenytoin 300 mg daily one year back but without any relief. For last 3 years, the patient had auditory agnosia with almost total inability to understand even familiar speech sounds. Word output also de- creased and he often made abnormal in- comprehensible sounds. He started communicating by gestures only and could not follow simple commands. There was inability to read or write spontnaneously but he could copy occasionally with difficulty. He was irritable and violent at times.

The patient previously had normal development of milestones and was studying in seventh class without any failure. There was no past or family history of psychiatric or chronic physical disorder. A battery of investigations including complete hemogram, Mantoux test, blood sugar, liver and kidney function tests, X-ray Chest and skull, fundus oculi and MRl were per- formed and were found to be within normal limits.

General physical and systemic examinations Were also normal. Mental status examination showed hyperkinetic behavior, production of abnormal sounds and gestures and at times, irritability. The detailed mental status examination and psychometry could not be done. Currently patient was receiving sodium valproate 1400 mg daily, prednisolone 60 mg daily and clonazepam 0.5 mg daily, alongwith tablet ranitidine 300 mg at night for the last 4 weeks but without any improvement in seizures or behavior. Prednisolone was gradually tapered off and ranitidine was stopped. On gradually increasing the dose of clonazepam to 3 mg daily along with continuation of sodium valproate (1400 mg/ day), there was decline in the frequency of seizures to one to two attacks per month. The irritability and violent behavior was also reduced but there was no improvement in speech or communication skills. The case is still on follow up for last two months. EEG showed right sided spike and wave discharges, prominent in frontal region.

Discussion

The etiology of this syndrome is not known.- Landau and Kleffner(1) had suggested that aphasia or speech disorder may be the result of a functional ablation of the primary cortical language areas due to persistent discharges in these regions. The studies(13,14) made on a cortical biopsy reported findings suggestive of a 'Slow Virus', meningoencephalitis. The present case also bad moderate grade fever before the onset of speech disorders.

EEG abnormalities are constantly found even in those without clinical seizures(15) and they are believed to be cortical manifestations of a lower subcortical differentiating process(10). EEG abnormalities consist of discharge of spikes, spike-wave or sharp waves of high amplitude either synchronous in both hemispheres, or in foci of variable localization, generally temporal, temporo-occipital or parieto-occipital. They are not influenced by hyperventilation or photic stimuli(15). Overt epileptic seizures manifest in only about 70% of patients and are usually mild. Generalized motor seizures, typical absence seizures, simple or complex partial types are more com- mon(1l-15) but the present case showed generalized tonic-clonic type. of seizures. As illustrated by this case, anticonvulsant treatment has no obvious influence on the aphasia(2) but seizures may respond to anticonvulsants(5,8). Anticonvulsant or steroid therapy may also be tried to abolish the EEG changes in the hope that this will ameliorate language function(16), but the present case responded to a combination of sodium valproate and clonazepam. The seizures always disappear before the age of 16 years(5,15).

Behavioral abnormalities such as aggressiveness, hyperactivity, tantrums, personality disturbances and intellectual deficits are common and may be wrongly looked upon as manifestations of psycho- sis. These disorders are closely related to aphasia(17,18). Aphasia .in this disorder may evolve in a subacute or gradual fashion over weeks or even years. The speech disorder is usually a disturbance of verbal comprehension with motor aphasia, word deafness (as seen in the present case) and at times, total mutism with a fluctuating course(17,18). Prognosis of Landau Kleffner syndrome depends, above all, on he development of aphasia. Early age of onset (especially four years) of aphasia indicates that the chances of complete recovery are slight. Few cases tend to remit spontaneously. Prognosis should be evaluated only after years of follow up.




 

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