History and Physical — Patient 37

History

Present Illness: A 10-year-old Mexican boy was admitted to the hospital in May 1999, with status epilepticus. He had experienced several previous seizures, most recently one month prior to admission. His first seizure occurred at three years of age, beginning with his head jerking to the left, and followed by generalized convulsive activity lasting one minute. He was not treated at that time. His second seizure occurred when he was nine years old, in March 1998. During this episode, his head turned to the left, and he subsequently experienced generalized tonic-clonic activity lasting about 12 minutes. He was treated acutely in Mexico with Phenobarbital and Valium, but not continued on any antiepileptic drug after discharge. He had no further seizures until April 1999, when he was admitted to Texas Children's Hospital in status epilepticus. He was treated with benzodiazepines and phenytoin acutely, and then maintained on phenobarbital. Several weeks after discharge, he developed a rash and was advised by his pediatrician to stop the phenobarbital. This led to resolution of the rash. However, two weeks after stopping phenobarbital, he experienced a generalized tonic-clonic seizure which continued for about 25 minutes before his arrival in the ER. Upon arrival at the ER, he intubated due to apnea. He required numerous doses of benzodiazepines, phenytoin, and phenobarbital to abort the seizure activity.

His parents noted that he had met his early developmental milestones appropriately. At the age of five years, he was doing well in kindergarten. He could count, write all his letters, and articulate well. By the age of six, his development had slowed markedly, and his parents reported little acquisition of new skills since then, although he has learned to read in the last two years. In the interim, he has lost several skills. He can no longer write letters clearly, and his speech has become slower and more difficult to understand. Within the past few months, he has experienced increasing difficulty with walking, and he can no longer run.

He denies any headaches, visual changes, sensory abnormalities, or muscle stiffness.

Past Medical History: He was the second child born to his 32-year-old G7P6A1 mother. The pregnancy was uncomplicated. His mother denied smoking, alcohol, or drug use during the pregnancy. The delivery was an uncomplicated spontaneous vaginal delivery at 42 weeks. There were no neonatal problems. There was no history of early trauma or infection.

Medications: At the time of consultation: ranitidine, phenobarbital 75 mg IV q 12 hours, fosphenytoin 100 mg IV q 8 hours, cefotaxime, and vancomycin. Subsequently, phenobarbital and fosphenytoin were discontinued because of the appearance of a rash, and treatment with valproic acid was initiated.

Family History: No consanguinity. Both parents are of Mexican nationality. Neither parent nor anyone outside the immediate family has had seizures or cognitive delay. He has four brothers and one sister. His 13-year-old brother has had several seizures, and has mild cognitive deficits. The next oldest child, a 7-year-old brother, had one seizure at three years of age. The other children are healthy.

Social History: The family recently moved to Houston from Mexico. There was no history of travel to other areas, or of known exposure to infections or toxic substances.

Physical Exam

Vital Signs: B.P. 95/45; pulse 140; temperature 102.6 F; respiration 12 (on an IMV setting of 12); weight: 30 kg (25-50%); height 134 cm (25-50%); FOC 54 cm (50-75%).

HEENT: Normocephalic and atraumatic. No dysmorphic facial features. Funduscopic exam reveals sharp disks with no hemorrhages.

Neck: Supple without adenopathy.

Chest: Clear to auscultation bilaterally.

Cardiovascular: Regular rate and rhythm, no murmurs.

Abdomen: Normal bowel sounds. No hepatosplenomegaly.

Genitourinary: Bilateral descended testes. Normal male genitalia.

Initial Neurological Examination

Mental Status: Unresponsive to verbal command. Withdraws to painful stimuli sluggishly in the four extremities. Does not open his eyes to noxious stimuli.

Cranial Nerves: Pupils are 5 mm and reactive bilaterally. Doll's eye response is normal. Corneal and gag reflexes intact. Face appears symmetric.

Motor/Sensory: Withdraws slowly to pain in the four extremities. Hypotonic throughout.

Reflexes: 1+ bilaterally throughout, except absent at the ankles. Plantar responses are flexor.

Baseline Neurologic Exam Prior to Discharge

Mental Status: Alert and oriented to person, place, and time. Speaks fluently in Spanish, though his speech is slower than expected. Is able to count to 20. Can recite the days of the week and months of the year. His writing is slow, characterized by poorly formed letters and frequent misspellings. He was not noted to exhibit irritability or an excessive startle response.

Cranial Nerves: Nerves II-XII intact.

Motor: Decreased tone throughout, more so in the lower extremities. No dystonic posturing. He has mild diffuse weakness rated as 5-/5, proximally and distally, bilaterally in the upper and lower extremities.

Reflexes: 1+ throughout, except reflexes at the ankles cannot be elicited. Plantar responses are flexor.

Sensation: Intact to pinprick, light touch, temperature, vibration, and proprioception throughout. No difficulty with graphesthesia or stereognosis.

Coordination: Intact finger to nose and heel to shin movements bilaterally.

Gait: Mildly wide-based and unsteady.

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