Title : Kiza-ntenga syndrome
Abstract:
The disorder of the development of the SN during the embryonic period is at the origin of malformations of cortical development (MDC) which represent a major cause of mental and motor disabilities as well as severe eilepsy. We describe here a syndrome called Kiza-Ntenga syndrome which is of embryonic origin and which is made up of right cerebral hemiatrophy, facial asymmetry with prominence of the face ipsilateral to the cerebral hemiatrophy, right hemierythrodermin, focal secondarily generalized convulsive seizures, slight regression of speech and walking. And therefore, Kiza designates the name of the patient and Ntenga which designates the name of the author who described him.
Observation: Child K, male, aged 1 year, weighing 9 kg, received for sudden seizures lasting more than 48 hours which were managed without success in a local structure. Born at full term with a birth weight of 4,100 kg eutogic manner. One day later, he will be put in an incubator without oxygenation. No history of malformation reported in the family or epileptic seizures. His mother reports a regression in speech and walking. Objectively we note an asymmetrical head with the right hemiface more prominent than the left, a right hemicorporal erythroderma from the head to the foot taking the interior and posterior face and describing an atypia taking half of the sole of the right foot while the left hemibody is normal in color. Left hemicorporal tonic-clonic convulsive seizures which generalize secondarily and which are sudden. A treatment consisting of valproic acid syrup at 30 mg/kg and injectable dexamethasone at 1 mg/kg will make it possible to control the seizures after a failure of treatment with phenobarbital which was administered in its source structure. A sleep EEG performed shows some loacilated localized spikes and spikes in the right hemisphere. A brain CT scan shows on an axial section cerebral hemiatrophy of the right hemipsphere with a double cerebral cortex in the frontal region and polymicrogyria. A basic biological assessment carried out and not contributory. After stopping the convulsive seizures under treatment, the examination shows an attentive child, with good eye tracking, without motor deficit, well toned, able to stand up with support and capable of walking on all fours. The remainder of the examination is unremarkable. This picture describes, not only that it is out of the ordinary but deserves to be described as a syndrome in its own right from the group of epileptogenic encephalopathies; which we call Kiza – Ntenga syndrome (Kiza to designate the name of the person who suffers from it, and Ntenga to designate the name of the author who described it).
Comments: This present syndrome is congenital and secondary to a developmental anomaly of the nervous system. He makes a differential diagnosis with Sturge Weber and Rasmussen syndrome
Audience Take Away Notes:
- When the syndromic diagnosis is made, this has an advantage on a therapeutic prientation to stop the seizers wich threaten the brain, but also, on the pratical orientation. This work allows othres researchers to push their reasoning further on the pathologies or syndromes described in the books and wich are not the same.
