/Translated from Russian to English/
/Stamp: DEPARTMENT OF HEALTH OF MOSCOW CITY
SCIENTIFIC & PRACTICAL CENTER OF MEDICAL CARE
FOR CHILDREN WITH CONGENITAL ABNORMALITY
OF CRANIOFACIAL AREA AND INHERENT DISEASES
CASE RECORD Nr. 622/10 Full name: Sidorin Georgy Vasilievich Age: 2 years old 7 month Date of birth: 09.07.2007 Settlement: Moscow city
He stayed at Psychoneurology Department of Scientific & Practical Center from 18.02.10 till 23.04.10 Current hospitalization is the first one. Clinical diagnosis: Cryptogenic localizes (sinistral, frontal) epilepsy, drug-resistant course. Infantile spasms syndrome. Secondary diagnosis: Minor cardiovascular development abnormalities (abnormal heart chorda). First degree hypertrophy of palatine tonsils. Complaints at admission: of attacks Attack type Frequency Duration Time of the day Registered Type 1: nods with 6-11 series a day Treatment at admission: Clonazepam 1,5 mg/d, Suxilep 750 mg/d, Luminal 12, 5 mg/d, Diphenylhydantoin 30 mg/d. Anamnesis and medical history: A child was born in normal pregnancy, in time. Pace of development before the attack debut according to age. 13.10.2009 he had an operation - resection of fibrous baby hamartoma of the left hand. Disease debut: at the age of 2 against the background of absolute health there appeared above mentioned attacks within the waking time, with the frequency of 1-2 per day, duration 5-10 minutes, with gradual acceleration. Since 06.01.10 there have been attacks at night. Pharmacological anamnesis: Medicine Doses mg/d Terms Concentration (mcg/ml) Results of previous examinations: MRT (10.10.09): in the paraventricle parts of the alba of the parietal lobes of both hemispheres – there are unclear areas of myelination retention. EEG (27.10.09): epileptiform activity with high grade of performance in terms of sets of “acute slow wave” with bitemporal amplitude maximum. Some signals are of regional origin from right posttemporal and left central temporal parts. Separate sets morphological y coincide with DEND. EEG (1.15.10): there is teta- and delta deceleration registered in frontal – central temporal parts of both spheres. Epiactivity is noticed in terms of several independent foci. At admission: As to somatic status the condition is satisfactory. Child’s weight is 15,5
kg. Skin and muculent is clear and pink. Heart and lung activity is satisfactory. Physiological functions are normal.
As to neurological status: There are no brain and meningeal symptoms. SN is without
focal symptoms, kinesis of limbs is ful , tone and power is sufficient. Brisk tendon reflexes, D – S, there are pathological reflexes registered. Coordinational probes are performed satisfactory, sensitivity is not disturbed. Psychic sphere: the child is cooperative, adequate. Speech – separate words, simple phrases.
Examination results: Indices of blood and urine analysis are within the age norm. Pharmacomonitoring dd. 31.03.10: Denakin chronosphere in doses of 500 mg/d: 1 probe: 68 mg/ml, 2 probe: 89 mg/ml. Video-EEG – monitoring during 12 hours with a record of night sleep dd. 22.02.10:
Significant diffuse changes of brain electrobiological activity. Hypsarrhythmia pattern. The sleep is not differentiated in stages. Physiological patterns are not traced.
Charact ac e t r e is i ti t c i of i c n of i te t r e ic i t c al t pat al h pat ologi ol c ogi al c ac al t ac i t v i i v t i y t i y n i domi dom n i ati at on i : State Localization Clinical performance: Attack features Localization
Flexor epileptical spasms of different manifestation rate, It’s difficult to determine the area
Video-EEG- monitoring within 6 hours with a record of night sleep dd. 6.04.10:
Significant diffuse changes of brain electrobiological activity. Hypsarrhythmia pattern.
Episodical y registered main rhythm corresponds to the age.
The sleep is not differentiated in stages. Physiological patterns are not traced. Charact ac e t r e is i ti t c i of i c n of i te t r e ic i t c al t pat al h pat ologi ol c ogi al c ac al t ac i t v i i v t i y t i y n i domi dom n i ati at on i : State Localization Clinical performance: Attack features Localization QTY
Flexor epileptical spasms of different manifestation rate, It’s difficult to determine the area
In comparison with the previous examination dd. 22.02.10 there is some improvement in frequency characteristics seen and indices of basic rhythm abundance growth.
• Brain MRT dd. 23.03.10: there are no pathological processes in the brain. • Psychologist’s consultation: Higher mental functions development is slightly lower the age norm.
• Speech therapist consultation: speech development delay • Ophthalmologist consultation: OU-development according to the age • Geneticist consultation, Candidate of medical sciences Zhilina S.S.: in the first
place one should exclude the group of genetic leukodystrophy (TMC)-Crabbe, met achromatic leukodystrophies, Tay-Sacks disease. Treatment applied:
In respect of ineffectiveness, Difenin, Luminal and Clonazepam are canceled. Depakin chronosphere 500 mg/d is introduced, on the back of which the number and duration of attacks has decreased. There also has been Tonamax max 6,5 mg/kg/d introduced, secondary to which the attacks modificated – the start with rhythmic low amplitude twitching of the right leg with posterior nod and shoulders uplift, the number of attacks hasn’t changed greatly. Sarbil is introduced with the doses increase up to 2000 mg/d (130 mg/kg/d), in this connection there has been a significant decrease in the number of attacks up to 3-4 times per 24-hours. He bears the treatment satisfactory. Release condition: satisfactory Dynamics in somatic and neurological statuses: is overal positive in terms of frequency characteristics improvement and indices of basic rhythm abundance growth, significant decrease of attack number, positive psycho verbal development of the child. Efficiency of antiepileptic therapy (%): decrease of attacks > 75%. There has been no contact with contagious patients. Recommendations: Denakin chronosphere 250 mg twice per 24-hours with a 12-hour interval, Sabril 0,5 2 pills twice per 24hours with a 12-hour interval There is a future Sabril doses increase up to 150 mg/kg/d possible, provided that there are still attacks but with no side effects. Antiepileptic medicine should be taken permanently, durably and at the same time without skipping the medicine taking! Doctor in charge /Ananjeva T.V./ Chief of the Department /Yavorskaya M.M./ Head of Epileptology Department /Ayvazyan S.O./
OF THE DEPARTMENT OF HEALTH OF MOSCOW CITY * PSRN 102776210237
SCIENTIFIC & PRACTICAL CENTER OF MEDICAL CARE FOR CHILDREN WITH CONGENITAL
ABNORMALITY OF CRANIOFACIAL AREA AND INHERENT DISEASES OF NERVOUS SYSTEM
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