Epicrisis

Of SPASIMIRA LYUBOMIROVA HRISTOVA - PIN 9004046259,
from Sofia, Slatina District, 7 "Tsv.Radoslavov" St, fl.1, ap.1
Admitted to Pediatric Neurology Clinic on 14.03.2006, discharged on 16.03.2006, history of disease file No. 1375


DIAGNOSIS: SYMPTOMATIC LENNOX-GASTAUT SYNDROME DUE TO AGENESIA OF CORPUS CALLOSUM. THERAPEUTICALLY RESISTANT EPILEPSY. INTELLECTUAL DEFICIENCY.

ANAMNESIS: The girl is known to the clinic from her many hospitalizations and long term treatment. The start of the epileptic seizures was when she was 8 months old - unwilled rotation movements of the eyes, for seconds only, 2-3 times a day; treatment with Phenobarbital was started- with no effect, followed by treatment with Nitrazepam with some improvement. From the age of 2 started myoclonic-atonic seizures varying from 40 up to 100 per day, a computer tomography of the brain was conducted at the age of 2 years 8 months revealing agenesia of the corpus callosum. Phenobarbital was replaced with Rivotril- without effect, afterwards treatment with Carbamazepin, Depakene, Suxinotin with variable effect. When treated with Depakene 600 mg/d, Nitrazepam 10 mg, Lamictal 50 mg a considerably good effect was reported- down to 1-2 seizures a day, as well as improvement in the mental status. In the beginning of 2001 Nitrazepam was replaced with Topamax- no change and due to loss of weight and kidney infection the therapy with that drug was stopped. In May 2005 Phenhydan was used- no effect. Afterwards Gabitril and Diazepam- also no effect. When increasing the dosage of Depakene and including Lamictal (2x100mg) in the treatment- temporary reduction of the generalized clonic-tonic seizures. From December 2003 Trileptal was increased to 1350 mg/d - temporary shortening the duration of and the same became less frequent, generalized clonic-tonic seizures became more frequent from 3 to 6 a day, short, very sudden/ jerky, with trauma, and daily morning series of asymmetrical short tonic seizures, consistent spread myoclonias of the arms, rare generalized myoclonic seizures and atonic with sudden squatting and standing up. Due to the apparent deteriorating of the condition Trileptal was excluded from the treatment and Keppra was included- up to 2x1000 mg per day. A significant improvement was registered- days without seizures, and subsequently- stabilizing in a certain frequency and interval of the secondary generalized clonic-tonic seizures: in 10-14 day single secondary generalized clonic-tonic seizures and tonic seizures were registered, and daily small in number (not in series) morning myoclonic and atonic seizures. Computer tomography of the brain (05.2003): evidence of disgenesia of the corpus callosum. Nonsymmetrical internal hydrocephaly, manifested mainly in the occipital cornua of the lateral ventricles. Consult with psychologist: Intellectual deficiency- medium to heavy degree, IQ=40% (Binet-Terman). Damaged superior core functions to a high degree, negativity.
Somatic status: knees in a discreet valgus position.

Neurological status: Discreetly limited extension of active movements in the knee joints with elevated muscle tone mainly in the lower limbs. Live deep tendon reflexes. Not in control of the pelvic reservoirs.

LABORATORY: Concentration of Depakene in the plasma 1500 mg/24h- 616 µmoll/l.
EEG (1776/14.03.2006): Abnormal findings with decelerated and disorganized basic activity, multifocal changes and frequent paroxysms from secondary generalized slow complexes, as well as rare tonic patterns, no clinical manifestation. No essential dynamics.

DISCUSSION: The girl has been diagnosed- Lennox-Gastaut syndrome resistant to therapy and acute course. The polymorphic epileptic seizures are not affected by the various and long term combinations of anticonvulsants. Keppra led to improving the condition, mainly in reducing the heavy traumatizing GTKP and GTP.

TREATMENT: Medications for conducting an EEG.
Status at discharge: No change.

RECOMMENDATIONS FOR TREATMENT: Keppra- 2x1500 mg., Depakene- 2x1,5 tab. 500 mg chrono, Lamictal 2x100 mg and Rivotril 2 mg - 3x1/2 tab.

RECOMMENDATIONS FOR THE GENERAL PRACTITIONERS: Control of blood count, GOT and GPT every 6 months. Conducting exams and EEG.


The current document can be produced to LEMC


Doctor in charge: /sgd. ill./
d-r P. Dimova, M.D.

Round seal of the University Hospital for active treatment in neurology and psychiatry "St. Naum"

Head of clinic: /sgd. ill./
Associated Prof. V.Bozhinova, M.D.