Management of infantile spasm
is challenging one since standard anticonvulsant drugs (AEDs) are not effective.
Characteristics of the Patients with Tuberous Sclerosis Number of patients (n) 36 Age (year) (median, range) 6 (0-17) Sex (M/F) 18/18 Presentation findings (n) Epilepsy 28 (78%) (5 infantile spasm
) Prenatal 4 Age (year) at the time of 6 years (2 days-17 years) diagnosis (median, range) Familial history 11 (%31) Skin findings (n) Hypomelanocytic macules 30 "Shagreen" patch 10 Facial angiofibroma 19 Brain findings (n) 31 (23 patients MMR) Subependymal nodules 27 Cortical tuber 16 SGCA 7 Heart involvement (n) 11 Renal involvement (n) 28 MMR: mental motor retardation; SGCA: subependymal giant cell astrocytoma Table 3.
Long-term prognosis after infantile spasms
: a statistical study of prognostic factors in 200 cases.
But when she experienced one of her infantile spasms
in hospital, it caused doctors in the Accident and Emergency department of Warrington Hospital to take action.
Cognitive dysfunction is observed more frequently in patients with infantile spasm
or resistant seizures (24).
Key words: Dravet syndrome, epilepsy, infantile spasm
, ketogenic diet, myoclonic astatic epilepsy
KARACHI -- Infantile spasms
are prevalent but are rare in Pakistani population.
The report provides comprehensive information on the therapeutics under development for Infantile Spasm
(West Syndrome), complete with analysis by stage of development, drug target, mechanism of action (MoA), route of administration (RoA) and molecule type.
As mentioned in the introduction of the article, the three main characteristics of West syndrome include :1) infantile spasms
, 2) regression and retardation in psychomotor development and 3) appearance of hypsarryhtmia on electroencephalogram.
To determine the risk factors which may affect the prognosis of seizures the patients who were classified as good and poor prognosis groups were statistically compared in terms of the age of onset of seizures, a history of status, presence of infantile spasm
, initial EEG findings, location of the epileptic activity on EEG, intellectual and cognitive development levels, the number and locations of cortical tubers and presence of astrocytoma.
An infantile spasm
is a specific type of seizure seen in an epilepsy syndrome of infancy and childhood known as West Syndrome.
This condition which was described by West in 1841 for the first time is characterized by typical seizures which are called infantile spasm
, finding of hypsarythmia on EEG and psychomotor retardation (23).