Atormac
brintellex
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 2663  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  » Next article
  » Previous article 
  » Table of Contents
  
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
 »Related articles
  »  Article in PDF (184 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  


  In this Article
 »  Abstract
 »  Introduction
 »  Case report
 »  Discussion
 »  References

 Article Access Statistics
    Viewed7986    
    Printed273    
    Emailed8    
    PDF Downloaded211    
    Comments [Add]    
    Cited by others 2    

Recommend this journal

   
Year : 2002  |  Volume : 50  |  Issue : 2  |  Page : 212-3

Landau-Kleffner syndrome : a case report.


Department of Neurology, G.B. Pant Hospital, Agartala, Tripura, India.

Correspondence Address:
Department of Neurology, G.B. Pant Hospital, Agartala, Tripura, India.

  »  Abstract

A healthy 5 year old boy developed aphasia, attention disorder and hyperkinesia preceded by transient formed visual hallucinations and emotional outburst, immediately after a stressful event of forced separation from his father. EEG showed generalized epileptiform activity. He was diagnosed as Landau-Kleffner syndrome (LKS). CT and MRI of the brain were normal. SPECT showed left mesial temporal hypoperfusion. He improved on antiepileptics and ACTH.

How to cite this article:
Raybarman C. Landau-Kleffner syndrome : a case report. Neurol India 2002;50:212


How to cite this URL:
Raybarman C. Landau-Kleffner syndrome : a case report. Neurol India [serial online] 2002 [cited 2020 Nov 28];50:212. Available from: https://www.neurologyindia.com/text.asp?2002/50/2/212/1389




   »   Introduction Top

Landau-Kleffner syndrome (LKS) is a rare epileptic syndrome characterized by acquired aphasia with epilepsy.


   »   Case report Top

A 5 year old boy developed episodes of sadness and crying for about one hour, immediately after the departure of his father for higher study. He also had transient formed visual hallucinations for about three to five minutes in the form of 'lion killing his father'. This was followed by inattentiveness, marked receptive and expressive aphasia and hyperkinesia. The child became totally non interactive with his environment within a week's time. He was seen by the author a month later. He could talk well and write simple sentences in his vernacular language before his illness. There was no family history of epilepsy, mental retardation or developmental speech disturbances. On examination, the child was hyperactive but communication ability was interrupted. Cranial nerves were normal and there were no long tract signs.
EEG, done in sleep state, showed generalized epileptiform activity [Figure - 1]. CT and MRI of the brain were normal. 99m-Technetium brain single photon emission computed tomography (SPECT) study showed left mesial temporal hypoperfusion. He was put on antiepileptic drug (AED) supplemented with a course of injectable adrenocorticotrophic hormone (ACTH). Follow-up revealed significant improvement in ten months, in verbal output, comprehension, attention span and hyperkinesia. Repeat EEG after 8 months showed persistent epileptiform activity.


   »   Discussion Top

The diagnosis of LKS, in this case, was made on the basis of rapid loss of language in a previously normal child, associated with abnormal EEG compatible with the diagnosis of epilepsy. While this disorder appears to be relatively uncommon, its frequency is questionable due to its unfamiliarity among the professionals and the likelihood of misdiagnosis. It is imperative that communication specialists become alert to the characteristic symptoms of LKS.[1],[2]
All children diagnosed as LKS have abnormal EEG compatible with the diagnosis of epilepsy, however, only 70% have clinical seizures.[2] The subjective emotional outburst of sadness and crying accompanied by formed visual hallucination in this patient may constitute a seizure. This type of seizure is rare in LKS. The epileptiform activity in LKS is thought to result in a functional ablation of eloquent speech areas. In one study, 13 of the 19 patients had perisylvian magneto-encephalography (MEG) spikes.[3]
The cause of the syndrome remains unknown. Relationship between the structural brain lesions and the clinical manifestations of LKS has been tried but not proved.[4],[5],[6] Cysticercosis, tumors, arteritis and acute inflammatory conditions have been reported to produce LKS. CT and MRI of the brain did not reveal structural brain lesion in the present case. However, 99m Tc brain SPECT study showed left mesial temporal hypoperfusion. A stressful event, like forced separation from his father, preceding the onset of interruption in communication ability, as observed in this case, has not been reported. LKS might have been provoked by a stressful event, however, this is only conjectural. LKS is difficult to treat. Treatment modalities used include antiepileptic drugs (AED), corticosteroids, intervenous immunoglobulin, ketogenic diet and multiple subpial transections.[7] Accepting the possible role of the epileptic discharge in producing the symptomatology of LKS[8] and the possible autoimmune reations in the etiopathogenesis of LKS,[9] the present case was put on AED supplemented with ACTH. He had significant improvement in verbal output, comprehension, attention span and hyperkinesis, in ten months. He had no recurrence of overt seizures, although repeat EEG, eight month after the initiation of the treatment, showed persistent epileptiform activity.

 

  »   References Top

1.Landau WM, Kleffner FR : Syndrome of acquired aphasia with convulsive disorder in children. Neurology 1957; 7 : 523-530.   Back to cited text no. 1    
2.Mouridsen SE : The Landau-Kleffner Syndrome : a review. Eur Child Adolesc Psychiatry 1995; 4 : 223-228.   Back to cited text no. 2    
3.Sobel DF, Aung M, Otsubo H et al : Magnetoencephalography in children with Landau-Kleffner syndrome and acquired epileptic aphasia. AJNR 2000; 21 : 301-307.   Back to cited text no. 3    
4.Otero E, Cordova S, Diaz F et al : Acquired epileptic aphasia due to neurocysticercosis. Epilepsia 1989; 30 : 569-572.   Back to cited text no. 4    
5.Pascual - Castroviejo I, Lopez Martin V et al : Is cerebral arteritis the cause of Landau-Kleffner syndrome ? Can J Neurol Sci 1992; 19 : 46-52.   Back to cited text no. 5    
6.Perinola T, Margari L, Buttinglione M et al : A case of Landau-Kleffner syndrome secondary to inflammatory demyelinating disease. Epilepsia 1993; 39 : 551-556.   Back to cited text no. 6    
7.Bergqvist AG, Chee CM, Lutchka LM et al : Treatment of acquired epileptic aphasia with the ketogenic diet. J Child Neurology 1999; 14 : 696-701.   Back to cited text no. 7    
8.Gordon N : The Landau - Kleffner syndorme : increased understanding. Brain Dev 1997; 19 : 311-316.   Back to cited text no. 8    
9.Nevsimalova S, Tauberova A, Doutlik S et al : A role of autoimmunity in the etiopathogenesis of Landau Kleffner syndrome. Brain Dev 1992; 14 : 342-345.   Back to cited text no. 9    

 

Top
Print this article  Email this article
Previous article Next article
Online since 20th March '04
Published by Wolters Kluwer - Medknow