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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 16  |  Issue : 2  |  Page : 376-378

Landau kleffner syndrome – Revisited


1 Sri Sathya Sai Sanjeevani Centre for Child Heart Care and Training in Pediatric Cardiac Skills; Department of Pediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, Maharashtra, India
2 Department of Pediatrics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Science (Deemed to be University), Wardha, Maharashtra, India

Date of Submission19-Oct-2018
Date of Decision17-Apr-2020
Date of Acceptance27-May-2020
Date of Web Publication18-Oct-2021

Correspondence Address:
Dr. Chitturi Sai Sujana
Shalinata PG Girls Hostel, Room G3, Sawangi, Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdmimsu.jdmimsu_75_18

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  Abstract 


Landau Kleffner syndrome (LKS)/acquired epileptic aphasia classically develops in healthy children who acutely or progressively loose receptive and expressive language ability coincidentally with the appearance of paroxysmal Electro Enchepalo gram (EEG) changes. At present, LKS is considered as a part of the syndrome of electrical status epilepticus in sleep, which is also known as continuous spike and wave during slow wave sleep. It is a rare childhood neurological syndrome. In this article, hereby, we report a case of a 7-year-old female child with LKS and unique characteristics.

Keywords: Aphasia, Landau Kleffner syndrome, status epilepticus


How to cite this article:
Sujana CS, Meshram R. Landau kleffner syndrome – Revisited. J Datta Meghe Inst Med Sci Univ 2021;16:376-8

How to cite this URL:
Sujana CS, Meshram R. Landau kleffner syndrome – Revisited. J Datta Meghe Inst Med Sci Univ [serial online] 2021 [cited 2021 Nov 28];16:376-8. Available from: http://www.journaldmims.com/text.asp?2021/16/2/376/328478




  Introduction Top


Landau Kleffner syndrome (LKS) was first recognized by William Landau and Frank Kleffner in 1957. Initially, children with this syndrome were mistakenly thought to be either deaf or on the autism spectrum. This rare childhood neurological syndrome is a form of acquired aphasia of childhood with epilepsy usually occurring in children between the ages of 3 and 10 years.[1]

There is a male dominance in the diagnosis of LKS, with a male-to-female ratio of 1.7:1 [Figure 1].[2]
Figure 1: Child with gait abnormalities in Landau Kleffner syndrome

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Typically, children with LKS develop normally but then lose their language skills, as it affects the parts of the brain that control comprehension and speech (Broca's area and Wernicke's area).

It is characterized by sudden or gradual development of aphasia and abnormal EEG changes primarily in the temporal lobe (EEG).[3]

Many patients exhibiting LKS may have abnormal electrical brain activity in both the right and left hemispheres of the brain, which is exhibited frequently during sleep.[4]

Processing difficulties and associated behavior problems such as hyperactivity, decreased attention, and social withdrawal.


  Case Report Top


A 7-year-old female child, born out of nonconsanguinous marriage, came with complaints of unable to speak, difficulty in hearing, and difficulty in walking for 2 years.

She had a history of convulsions on the one side of the body with excessive crying episodes.

Past history

She was previously hospitalized 2 years back with a history of fever followed by weakness of the right hand and leg for the same with convulsions and diagnosed as pyogenic meningitis and was in coma for 15 days and took treatment for the same.

On anthropometric evaluation, according to the Indian Academy Of Pediatrics (IAP) classification for the weight for age suggestive of no malnutrition.

Birth History: Full Term (FT)/ Appropriate for Gestational Age (AGA)/ Appropriate for Gestational Age (NVD)/3 KGS/ Baby cried immediately after birth (BCIAB), No Neonatal Intensive Care Unit (NICU) stay.

Developmental H/O: Normal before illness.

On examination, vitals were normal.

On systemic examination

Central nervous System (CNS) – conscious, not oriented; scissoring gait present, hypertonia, global aphasia, dyslexia, dysgraphia, geographical agnosia, astereognosia, deafness, dysdiadokokinesia, deep tendon reflexes exaggerated, and right leg ankle clonus present.

Other systems were normal.

Investigations done during illness

  • Computed tomography (CT) of the brain revealed that Sylvain fissure, basal cisterns, and sulcal spaces are effaced and ventricular system are effaced, suggestive of mild cerebral edema
  • Magnetic resonance imaging (MRI) of the brain revealed extensive areas of altered signal intensity involving bilateral cerebral hemispheres, midbrain, and bilateral striato–capsulo-thalamic regions
  • EEG was suggestive of continuous runs of epileptiform discharges over bilateral centrotemporal discharges with frequent secondary generalization in sleep and was suggestive of LKS [Figure 2] and [Figure 3]
  • Brainstem evoked response audiometry demonstrated mild hearing loss at lower frequencies [Figure 3] and [Figure 4]
  • Visual evoked potential demonstrated prolonged P100 latency in both eyes. Amplitude reduced in the left eye more than in the right eye [Figure 5] and [Figure 6].
Figure 2: EEG abnormalities showing Spikes

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Figure 3: EEG abnormalities in Landau Kleffner syndrome

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Figure 4: Visual evoked potential

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Figure 5: Auditory Evoked Potential (AEP)

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Figure 6: The brainstem evoked response audiometry

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  Discussion Top


In the present case, the diagnosis of LKS was done due to the presence of rapid aphasia in a formerly normal child, along with EEG abnormalities well matched with a diagnosis of epilepsy. As this disorder is uncommon and its occurrence is rare and also unfamiliar to the professional doctors, there are high chances to misdiagnose such cases. It is imperative that communication specialists should be made alert to the characteristic features of LKS.[5],[6] The idiosyncratic emotional lability via outbursts of crying and sadness along with formed visual hallucinations in this patient may constitute a seizure. Presumably, this type of seizures is rare. In LKS, epileptiform activity is considered to result in a practical ablation of fluent speech areas. In one study, 13 out of 19 patients had perisylvian magnetoencephalography spikes.[7] The etiology of this syndrome remains unknown. The association between the clinical manifestations of LKS and structural brain lesions has been under trial, yet not proved.[8],[9],[10] Different conditions have been reported to be present along with LKS, such as tumors, cysticercosis, inflammatory conditions, and arteritis.

MRI and CT of the brain revealed cerebral and cerebellar atrophy, in the present case. LKS may have been triggered by a stressful event, but this is only conjectural and not in this case. LKS is difficult to treat and only symptomatic. Treatment modalities include antiepileptics, corticosteroids, intravenous immunoglobulin, ketogenic diet, and multiple subpial transections.[11] Acquiescent to the diagnosis of LKS and understanding the role of epileptiform discharges leading to the present symptomatology[12] and the possible autoimmune reactions in the etiopathogenesis of LKS,[13] the present case was started on antiepileptics. She had significant improvement. She had no recurrence of overt seizures, although repeat EEG, after the initiation of the treatment, showed persistent epileptiform activity.


  Conclusion Top


A case of LKS with vision loss and mild hearing loss with EEG characteristic of epileptiform discharges over bilateral centrotemporal discharges with frequent secondary generalization in sleep.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Fandiño M, Connolly M, Usher L, Palm S, Kozak FK. Landau-Kleffner syndrome: A rare auditory processing disorder series of cases and review of the literature. Int J Pediatr Otorhinolaryngol 2011;75:33-8.  Back to cited text no. 1
    
2.
Gordon J. Preventing Autism: What You Can Do to Protect Your Children Before and After Birth. John Wiley & Sons; 2013.  Back to cited text no. 2
    
3.
Pearl PL, Carrazana EJ, Holmes GL. The Landau-Kleffner syndrome. Epilepsy Curr 2001;1:39-45.  Back to cited text no. 3
    
4.
Acquired Epileptic Aphasia: Background, Pathophysiology, Etiology. Available from: https://emedicine.medscape.com/article/1176568-overview. [Last accessed on 2019 Apr 18]  Back to cited text no. 4
    
5.
Landau WM, Kleffner FR. Syndrome of acquired aphasia with convulsive disorder in children. 1956. Neurology 2001;57:S29-36.  Back to cited text no. 5
    
6.
Mouridsen SE. The Landau-Kleffner Syndrome: A review. Eur Child Adolesc Psychiatry 1995;4:223-8.  Back to cited text no. 6
    
7.
Sobel DF, Aung M, Otsubo H, Smith MC. Magnetoencephalography in children with Landau-Kleffner syndrome and acquired epileptic aphasia. AJNR Am J Neuroradiol 2000;21:301-7.  Back to cited text no. 7
    
8.
Otero E, Cordova S, Diaz F, Garcia-Teruel I, Brutto OH. Acquired epileptic aphasia (the Landau-Kleffner syndrome) due to neurocysticercosis. Epilepsia 1989;30:569-72.  Back to cited text no. 8
    
9.
Pascual-Castroviejo I, López Martín V, Martínez Bermejo A, Pérez Higueras A. Is cerebral arteritis the cause of the Landau-Kleffner syndrome? Four cases in childhood with angiographic study. Can J Neurol Sci 1992;19:46-52.  Back to cited text no. 9
    
10.
Perniola T, Margari L, Buttiglione M, Andreula C, Simone IL, Santostasi R. Case of Landau-Kleffner Syndrome secondary to inflammatory demyelinating disease. Epilepsia 1993;34:551-6.  Back to cited text no. 10
    
11.
Bergqvist AG, Chee CM, Lutchka LM, Brooks-Kayal AR. Treatment of acquired epileptic aphasia with the ketogenic diet. J Child Neurol 1999;14:696-701.  Back to cited text no. 11
    
12.
Gordon N. The Landau-Kleffner syndrome: Increased understanding. Brain Dev 1997;19:311-6.  Back to cited text no. 12
    
13.
Nevsímalová S, Tauberová A, Doutlík S, Kucera V, Dlouhá O. A role of autoimmunity in the etiopathogenesis of Landau-Kleffner syndrome? Brain Dev 1992;14:342-5.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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