8th International Congress Of The Union
Of The Arab Pediatric Societies
Beirut 23-25 Nov. 2000

Hemiplegic form of Landau- Kleffner Syndrome:
a New Presentation.

Purpose: The Purpose Of This Abstract Is: First, To Describe A New Form Of Landau- Kleffner Syndrome LKS: The Hemiplegic Form; Second, To Reconfirm The Effectiveness of Immunoglobulins In The Treatment Of LKS, And To Suggest, For The First Time, That A Single Injection Of Immunoglobulins May Be Sufficient.

Introduction: Landau- Kleffner Syndrome Is Characterized By: Acquired Epileptic Aphasia With Severe Language Comprehension And Expression Disturbance; And Epilepsy Associating EEG Spikes With Posterior Temporal Predominance Activated By Sleep But With Rare Epileptic Seizures .

Method: A Right Handed, Four Years And A Half Boy With Uneventful Personal And Familial Histories. Exactly The Child Did Make Sentences of 3 Elements At 3 Years of Age. At Four Years And A Half of Age, The Child Starts To Have Acute Regression of Speech Then Appearance Of Rare Hemiclonic Seizures. The Speech Disappeared Completely After Two Months, Following Right Afebrile Hemiclonic Status Epilepticus That Was Followed Also By Right Hemiparesis With Complete Loss Of Function Of The Right Hand . Then Voluntarily He Became Left Handed. The Child Displayed Autistic Behavior: He, Didin't Excecute Orders, Was Not Interested In The Environment, Neither In Toys Nor In TV, He Was Unable To Distinguish His Parents; Even The Non Verbal Communication Disappeared. there was Also An Appearance Of Megaphagia And Drooling. Finally He Refused To Walk During Two Weeks. It Is Important To Mention That He Has No Ataxia, Vomiting, Abnormal Movement or Organomegalies. Three Months After The Onset of The Disease And Exactly After The First Dose of Intravenous Immunoglobulins, The Patient Started To Resume Progressively His Previous Normal Speech And His Previous Normal Psychomotor Development Starting First By Comprehension. It Is Important Also To Mention The Disappearance of Hemiparesis, And The Child, Surprisingly , Resumed His Old Right Handedness. The Verbal Expression Was The Last Function Recuperated, Finally The Child Regained His Normal Status After Two Months of Treatment, And after four years of followup He Is very Well Scolarized . The Sleep Interictal EEG Showed Bitemporal Spikes And Sharp Waves .

The Following Laboratory Tests Are All Normal: Auditory Evoked Potentials, Brain MRI And CT SCAN ,CBCD , FBS, Lactate Pyruvate, Ketone Bodies, Transaminases, Aminoacids In Plasma And Urine, Organic Acid In Urine ,Lumbar Puncture Including Gammaglobulins In The C.S.F. Since The Patient Is Improving Rapidly After The First Dose Of Immunoglobulins And Due To The Financial Problems Encountered By The Parents, The Patient Received Just Three Doses Each One Month Apart From The Other And Every Dose Was 2 gr /Kg.

Discussion: This Is A New Additional Case of LKS That Confirms The Effectiveness of Immunoglobulins In LKS. The Particularity of This Case Is Its Rapid Improvement After The First Injection And The Complete Recovery of Language Just After 3 Injections Which Lead To Suggest That A Single Injection May Eventually Be Sufficient. But Larger Studies Are Needed. LKS Develops During A Period of Cortical Synaptogenesis When The Basic Functional Circuitry Is Being Established (Age 1-8 Years); Hence The Hemiplegia of The Present Case Could Be Induced By The Extension of Epileptic Discharges Beyond The Temporal Regions.

This Hemiplegia Could Not Be Explained By Todd's Phenomenon. Because :
1- The Loss of The Right Hand's Function Was Complete Despite Preservation of Power At 4/5.
2- It Lasted A Long Period of Time Time (3 Months).
3- Its Improvement Was Parallel To The Improvement of Speech. on The other Hand The Normalcy of The Neurologic Exam Before The Onset And After The End Of The Disease Suggests That This Hemiplegia Is Functional.

The Severity Of The Disease Which Is Evident By The Presence Of Complete Mutism, The Absence Of Communication Even Non Verbal, The Transient Loss Of Walking That Is Caused By The Abnormal Behavior, The Complete Hemiparesis And The Sphincter Disorders, Does Not Prohibit The Rapidity Of Recovery.