Cavallero-West syndrome-(Argentina)

Name: Cavallero Baz Constantino Efrain  

Sex: Male

Country: Argentina

Age: 5 years

Diagnoses: West syndrome, Epilepsy

Before treatment:

The patient was born when his mother was 38 weeks pregnant through cesarean. The weight at birth was 2.3Kg. The patient stayed in incubator for 3 days for suffocation at birth. He had swallow difficulty after birth. He had suffocation and convulsion frequently. The patient was diagnosed with West syndrome. He received ACTH through intramuscular. The epileptic seizure was reduced. For the repeated aspiration, result in repeated infection of lungs. The patient maintained gastrostomy when he was 1 year old. At the same time, the patient received ketogenic diet for treatment. But the epilepsy still attacked often. The patient received botulinum toxin for treatment. He received autologous stem cells for treatment 1 year ago. The pulmonary infection was alleviated after treatment. The patient does three times rehabilitation each week. From the onset of disease, the patient had poor spirit. He had a delay in intellectual and motor development. He had much sleep. He also suffered from gatism. Before the treatment, the patient still couldn't turn over, sit-up, crawl or walk. He had speech disorders and only could pronounce the sound "A".  He still takes Zonisamide 100mg q12, Lacosamide 50mgqd 75mg qn, phenobarbital 50mg q12 for anti-epileptic.

Admission PE:

BP: 90/60mmHg, Br: 24/min; Hr: 118/min, Height: 105cms, weight: 17kgs. The patient suffered from developmental retardation. The nutrition was normal. His skin and mucosa were normal, with no yellow stains. His thorax was symmetrical. The respiratory sounds in both lungs were clear with no signs of dry or moist rales. The sound of his heartbeat was strong and the rhythm was normal. There was no obvious murmur in the valves. The abdomen was flat, with no palpable masses.  The gastrostomy tube fistula was maintained. The liver and spleen were not palpable under the ribs.

Nervous System Examination:

The patient had much drowsiness and dispirited. There was no obvious emotional response. He had speech disorder and only could produce the sound "A". He could not perform the memory, calculation abilities or orientation examinations. His comprehension was poor. The diameter of the pupils was 3.5mms. Both pupils had sensitive responses to light stimulus. The forehead wrinkle pattern was symmetrical. The bilateral nasolabial sulcus was equal in depth. The movement of neck was poor in supine position. He had difficulty supporting his head in a seated position. He was unable to turn over by himself. The muscle strength of his waist and back were poor. He was unable to maintain a seated position by himself. Both hands were unable to grasp objects. Both lower limbs had movement action. He could not lift his legs off of the surface of the bed. He was unable to complete the examination for the muscle strength of his four limbs. The muscle tone of his four limbs was higher than normal when he was in sober condition. His abdominal reflexes were normal. The tendon reflex of his four limbs was active. Bilateral Achilles tendon reflex was not elicited. Bilateral ankle clonus was positive. Bilateral sucking reflex was negative. Bilateral palm jaw reflex were negative. Bilateral Hoffmann sign was negative. The Rossolimo sign of both upper limbs was negative. Bilateral Babinski sign was positive. He could not perform the sensory and coordination movement examinations.

Treatment:

After admission, the patient received the relevant examinations and started treatment from October 4, 2013. The patient received nerve regeneration treatment and stem cell activating treatment. At same time, the patient received treatment to improve the blood circulation in order to increase the blood supply to the damaged nerves, to nourish the neurons and to enhanced immunity. This was combined with diet and rehabilitation treatment.

Post-treatment:

The patient's has grown taller and gained weight as well, height: 106cm, weight: 19Kg. We adjusted the medication of antiepileptic drug. The epileptic seizure is relieved than before. The degree of awareness is increased than before. He has better emotional response now. The salivation is reduced than before. The pharyngeal reflex is more sensitive than before. The strength of the muscles that control the head has increased than before. Right hand has acquired grasp ability and all the fingers can stretch now. The index of tendon reflex of four limbs is reduced. The patient had poor metabolizers before admission. At present, the patient has better diet. The hypoglycemia is almost corrected. The thyroid function has been restored to normal level.

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Baz has significant improvement after discharge, his parents email to us, please see the videos:

https://www.dropbox.com/s/o2wap45jxktspui/IMG_1853.MOV

https://www.dropbox.com/s/6m4rssfg9gxmm3q/llora%2520Efra.mp4

Date: 2014-08-25

Doctor buenas tardes, espero estén muy bien.
 
Queriamos comentarle que Efrain esta muy bien, ya no tiene espasmos solo al despertarse lo notamos unos minutos con su mirada perdida y unos pequeños movimientos en su pierna.

Deseabamos consultarles sobre sus medicinas.
 
Ya se nos esta terminando B- complex y Oryzanol, queríamos saber si ya podemos concluirlas.

Adicional Efrain ha subido mucho de peso últimamente esta ya en 25,700kg, Deseamos saber si continuamos con Flunarizine y Vitamina B6 o también se podrán concluir pronto.
 

Saludos y muchas gracias.
 
Analia Baz