Benign Partial Epilepsy Of Infancy.



1- Benign Familial Infantile Convulsions (BFIC) :

Described First By VIGEVANO ON :1992 .
The Seizures Occur In neurologically Normal Infants between 3 And 8 Months Of Age. they have Autosomal Dominant Inheritance. The Seizures often Occur In Clusters And They Are Marked By: Psychomotor Arrest, Cyanosis, Head And Eye Deviation To One Side, Diffuse Tonic Contraction And Bilateral Clonic Jerks. The Side Of Head Deviation May Change In The Same Patient. The Interictal EEG Is Normal. The Ictal EEG Shows Typical Localized Spike Discharges Arising From One Or The Other Hemisphere, Usually In The Posterior Regions. The Seizures Disappear In All Infants Within A Few Days, Weeks Or Months. The Outcome Is Normal. A Locus BFIC1 is Mapped To Chromosome 19q. A Second Locus BFIC2 Is Mapped To 16p12-q12 And A Third BFIC3 To 2q 24.



2- Benign Partial Epilepsy Of Infancy With Complex Partial Seizures :

Described first by WATANABE et al On : 1987.
The Age Of Onset Is Younger Than 1 year. The Seizures Occur 1 To 10 Times Daily In clusters For Periods Of 1-3 Days. They Could Recur In The Same Form Within 1-8 Weeks Later. They Are Brief And They Appear In Wakefulness And Sleep. They consist Of: Motion Arrest, Decreased Responsiveness, Staring, Simple Automatisms Of Limbs Or A Change In Facial Expression, Eye Deviation Or Head Rotation, Mild clonic Jerks Of The Face And Limbs And Increase Limb Tone. The Interictal EEG Is Normal. The Ictal EEG Disclose Focal Discharges Of Repetitive Sharp Alpha Or Theta Waves Of Increasing Amplitude And Decreasing Frequency With Gradual Or Rapid Spread To Other Regions. The Initial Site Of Discharge Is Usually Temporal. Treatment Is Discontinued After 1.5-5 Years. The Later Development Is normal .

In Some Infants These Complex Partial Seizures Are Followed By Generalized Tonic - Clonic Convulsions. The EEG Shows A Focal Origin Of The Seizures. Recurrences Are Limited To The First 1 To 5 Months After The Onset. The Outcome Still Excellent.

CAPOVILLA et al(1998) Reported A Subgroup Of Patients With Seizures Onset Between 13 Months And 2.5 Years Of Age. Seizures Are Marked By: Apnea, Staring, Motion Arrest, Cyanosis Of The Face And Loss Of Consciousness. The Outcome Is Normal. With The Final Attack Being no Later Than 3 Years 7 Months Of Age .The Interictal EEG Recordings Were Consistently Normal. The Ictal EEGs Recorded Paroxysmal Discharges In The Temporal or Occipital Areas.

Another Subgroup Of Benign Infantile Convulsions Associated With Vertex Spikes On Interictal EEGs Has Been Described By CAPOVILLA And BECCARIA(2000).

The Relationship Between These Syndromes Appear To Be close .
Fukuyama In 1962 Described A Non Familial Subgroup Of Benign Infantile Convulsions, Before 2 Years Of Age, As Generalized Symmetric Tonic-Clonic Convulsions Lasting 1 To 2 Minutes. There Was, No Recognized Etiologies . A Normal Interictal EEG And Benign Course .

However Very Similar Cases With A Definite Family History Of Convulsions Were Reported By LEE et al (1992) . Interestingly Several Of The Infants In This Series Had A Mild Gastroenteritis at the time of the convulsive Clusters .
In Japan ABE et al (2000) Described Similar Cases, Where Gastroenteritis Were Caused By Rotavirus And Small Round Viruses.
Thus One Or Several Syndromes Of Benign Partial Seizures May Exist in Infancy.

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