Neurology: Case of the Month

History and Physical — Patient 64

History

Chief Complaint: New-onset seizure.

Present Illness: The patient was a male, 13 months old at the time of consultation to the Neurology Service at the Texas Children's Hospital. History was provided by the referring doctor, who knew the patient and family well, as the patient's mother was not available for consultation. The patient was in his usual state of health until the day of consultation, when he developed seizure-like activity. The event disrupted a playful chasing match with his sisters, when the patient suddenly developed involuntary jerking of his left leg. This clonic activity quickly spread to the rest of his body, and then spontaneously aborted after about three minutes. Post-ictally, he was confused for about ten minutes, and also displayed a persistent left arm and leg hemiparesis as well as mild ataxia. Additionally, the patient was noted to run into objects as if he was at least partially blind. There was no evidence of tongue biting, and no bowel or bladder incontinence. No recent trauma, febrile illness, or any obvious precipitating cause was evident to the referring doctor. Urgent neurologic consultation was obtained.

Past Medical History: None. Immunizations up to date.

Past Surgical History: None.

Allergies: No known drug allergies.

Medications: None.

Social History: He has always been physically active and very playful. He has a caretaker for special needs. An animal living in the same neighborhood as the patient recently died, reportedly from CNS toxoplasmosis, but it is not known whether there was any direct contact with the patient or his family.

Family History: No known medical or neurological disorders affecting the patient's mother, step-father, or two half-sisters.

Developmental History: Normal birth and perinatal history. Appropriate growth for age. Gross motor coordination was reportedly excellent, with fully developed walking, running and jumping; fine motor skills were normal for age but less well developed in comparison. Visual-motor skills were reportedly normal. Language development was virtually absent, with interactions limited to vocalizations and limb movements, and no verbalizations at any time.

Review of Systems: No cough, congestion, fever, chills or any evidence of systemic illness. No history of frequent or unusual infections. The patient always has had a healthy appetite, until the onset of illness.

Physical Exam

General: Note that the patient was sedated and intubated for neuroimaging. Well-developed, well-nourished appearing male with generalized hirsutism.

Vital Signs: Temperature: 100.9 F; Blood Pressure: 110/90 mmHg; Pulse: 120/min; Respiratory Rate: 25/min.

HEENT: Brachycephalic, with no evidence of head trauma. The patient had high-set ears, macrotia, bushy eyebrows, epicanthal folds, microtelorism, a depressed nasal bridge, and macrognathia with slight macroglossia. Sclerae were anicteric. Mucosal membranes were slightly dry, and the patient was intubated.

Neck: No signs of meningimus.

Chest: Lungs were clear to auscultation bilaterally.

Cardiovascular: Tachycardia, with regular rhythm. No murmurs or gallops heard.

Abdomen: Soft, nondistended abdomen with normoactive bowel sounds. Testicles were appropriate size and descended bilaterally. The coccyx was prominent.

Extremities: Long, slender limbs, with relatively short phalanges. No signs of clubbing, cyanosis or edema. No rashes noted.

Neurological Examination

Mental Status: Deeply sedated and unable to be aroused to meaningful interaction. Response to visual threat could not be reliably assessed.

Cranial Nerves: Pupils were equally round and reactive to light. Corneal responses were present and symmetric. Oculocephalic responses were present and symmetric. Facial grimaces to noxious stimulation appeared symmetric. Gag response was depressed but present. Tongue was displaced by the endotracheal tube, but did not exhibit fibrillations.

Motor: No spontaneous activity. Good muscle bulk. The patient withdrew his right arm and leg from stimulation better than his left side, with respect to power and amplitude of movement.

Reflexes: 2+ throughout except for left patella 3+ (distal spread) and left ankle 4+ (3 beat clonus).

Sensation: Grimaced to deep pressure stimuli in extremities and face.

Plantar Responses: Extensor on left and flexor on right.

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