Spectrum of epilepsy and electroencephalogram patterns in Wolf-Hirschhorn syndrome: experience with 87 patients.

[wolf-hirschhorn syndrome]

To define the spectrum of epilepsy in Wolf-Hirschhorn syndrome (WHS ) better , we studied 87 patients (54 females , 33 males ; median age 5 .6 years ; age range 1 -25 .6 years ) with confirmed 4 p 16 .3 deletion . On the basis of clinical charts , we retrospectively analyzed the evolution of the electroencephalogram (EEG ) findings and seizures . Epilepsy occurred in 81 patients (93 %) within the first 3 years of life . Sixty out of 81 (74 %) had generalized tonic-clonic seizures , which was the only seizure pattern in 32 . Tonic spasms occurred in 15 out of 81 (18 %), complex partial seizures in 10 out of 81 (12 %), and clonic seizures in 6 out of 81 (7 %). Seizures were frequently triggered by fever (59 out of 81 ; 73 %), and occurred in clusters in 36 out of 72 (50 %). In the same 36 (50 %), unilateral or generalized clonic or tonic-clonic status epilepticus occurred during the first 3 years of life . Twenty -seven out of 81 patients (33 %) developed atypical absences between 1 and 6 years , accompanied by a myoclonic component involving the eyelids and the hands . Distinctive EEG abnormalities were observed in 73 out of 81 (90 %). Epilepsy was well controlled in 65 out of 81 (81 %), mainly with valproate and phenobarbital , and improved with age in all . Thirty -two out of 58 (55 %) are currently seizure -free . Seizures stopped at a median age of 4 years 6 months . Epilepsy represents a major clinical challenge in WHS ; however , it has a good prognosis . Early diagnosis and treatment of atypical absences , subtle and often misdiagnosed , is mandatory .

Diseases
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Diseases presenting "seizure-free" symptom

wolf-hirschhorn syndrome

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