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SOFIA DEGANO - West Syndrome

Author Julia Views Posted at 2014/01/14

Sex: Female
Country: Argentina
Age: 2
Diagnoses: 1.WEST Syndrome, 2. Congenital heart disease (atrial septal defect)
Admission Date: 2012-08-10
Days Admitted to Hospital: 30

Before treatment:
SOFIA is a twin sister. The patient was born at 32 weeks of her mother��s pregnancy. The patient was found to have suffered from a cerebral hemorrhage 15 days before birth. The patient suffered from developmental delay, poor eyesight and movement disturbance. Her eyes couldn��t follow the moving object. She was sent to the local hospital in May 2005 and diagnosis of chronic advancing encephalopathy was considered. The MRI examination showed an arachnoidcyst. But the neurosurgeon didn��t suggest surgical treatment. The patient suffered from convulsion when she was 7 months old. The convulsion attacks happened dozens of times each day and lasted several seconds each time. She went to the local hospital again and received EEG and ECG examination. She was diagnosed with 1.WEST Syndrome, 2. Congenital heart disease (atrial septal defect) showed clearly. The doctor suggested temporarily not treating the congenital heart disease. The patient was regularly rechecked. At the same time, the patient received antiepileptic drugs. After treatment, the patient��s condition improved. The epilepsy was not severe and attack happened 1-3 times each day. The blood examination in November 2010 showed the level of blood cortisol was low. She received glucocorticoid for treatment. At present, the patient presented with developmental delay, poor eyesight, and a liquid diet. She, also, had motor difficulty of four limbs and epileptic seizure.
Before admission, the patient took sodium valproate, Topamax, diazepam and other anti-epileptic medication. The epilepsy was not under control. On average she experienced 2-3 epileptic seizures each day. Mainly presented with upper limbs buckling, muscle tetanic contraction, accompanied with rapid Horizontal nystagmus, the seizures would typically last for several seconds. The patient developed upper respiratory tract infection before admission.
Admission PE:
Temperature: 36.5��, Bp: 85/50mmHg; Hr: 134/min, Br: 30/min. Mental development delay. Poor nutrition. Height: 75cm, Weight: 9kg. Skin and mucous membrane were intact, with no yellow stain or petechia. There were vascular white-spots in the skin of four limbs. The pharynx was red and the tonsil was not enlarged. She had phlegm, but had difficulty coughing it up. Symmetrical thorax. the respiration in both lungs was rough; there were no dry or moist rales. Through auscultation it was determined that the heart sounds were strong. The rhythms of her heart were regular and there was no obvious pathological murmur. The abdomen sagged and was soft, with no masses touched. There was no pressing pain or rebound tenderness. The liver and spleen were not touched under the ribs.

Nervous System Examination:
SOFIA was alert and she had logagnosia. She couldn��t cooperate with the memory, calculation ability, or orientation examinations. Both pupils were equal in size and round, the diameter was about 4.0mms. Both pupils were sensitive to light reflex. Both eyes couldn��t follow moved objects. She couldn��t cooperate with the cranial nerve examination. She ate a liquid diet, no cough while swallowing. The muscle strength of the neck was poor and the neck was soft. She could swivel head in the horizontal position. She couldn��t cooperate with an examination of muscle strength. There was limited activity in the limbs. She couldn��t turn over, sit up, stand or walk. The muscle tone of both upper limbs was higher than normal. The muscle tone of both lower limbs was almost normal. The tendon reflexes of all four limbs were normal. The abdominal reflex was normal. The bilateral sucking reflex was negative. The Babinski sign was negative. She couldn��t cooperate with the sensation and coordinated movement examinations. There were no signs of meningeal irritation.

SOFIA received a complete examination and the atrial septal defect was found. The COR 19.46nmol/l. The diagnosis of 1. WEST Syndrome, 2. Congenital heart disease (atrial septal defect) and 3. hypocorticalism were clear. She received treatment to nourish the neurons, improve the blood circulation and control epilepsy. She also received treatment to reinforce nutrition, replenish glucocorticoid and other comprehensive treatment. This was accompanied with rehabilitation training.

After treatment, the upper respiratory tract infection and adrenal crisis were cured. The cardiac murmur was alleviated, but still exists. At present, the patient��s height is : 78 cm, weight: 9.5Kg. The patient��s neurological signs are improved. The epilepsy is almost under control. The muscle strength in her neck improved, which helps her to raise her head. She swivels more flexibly. The muscle strength of her four limbs is improved. After pain stimulus, the four limbs activity and posture are maintained better. The muscle tone of both upper limbs is almost normal in a resting state.

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