Neurology: Case of the Month

History and Physical — Patient 42

History

Present Illness: The patient is a 10.5 year old Caucasian female, previously in good health, who lives in a rural area of southeast Texas. She began to complain of diarrhea, fatigue, anorexia, and headache several days prior to hospital admission. Three days prior to her presentation, the patient was noted by family members to have facial swelling or puffiness, and dark, tea-colored urine. Her facial swelling appeared to resolve over the next day, but her anorexia and fatigue persisted. On the evening prior to admission, the patient was excessively fatigued, and went to bed early without eating dinner. That evening, she also complained of headache, but this did not provoke immediate concern, as she was known to have frequent headaches due to chronic sinus problems. She awoke at 6:45 on the morning of admission with a severe headache, followed by nausea and vomiting after she was given acetaminophen. She was not noted at any time to have a fever or skin rash. As her mother had to go to work, the patient was taken to her grandmother's home and went back to sleep. She awoke approximately 1.5 hours later, and told her grandmother that she "couldn't see." Her grandmother led her by the hand into another room, where she stated that her vision returned. A few minutes later, she complained to her grandmother that she once again could not see.

On the way to her doctor's office, the patient was noted to develop a blank stare and become extremely quiet, with muscle contractions noted on the right side of her face. She was immediately taken to the local emergency room, where she was noted to have "jerking movements" of her arms and legs and was thought to be having generalized tonic-clonic seizure activity. At this time, her blood pressure was 166/108 mmHg. While in the emergency room of the local hospital, the patient was treated with midazolam and lorazepam, loaded with phenytoin, and intubated for airway protection due to persistent vomiting and somnolence. She continued to exhibit intermittent spontaneous motor activity over the next five hours, and received during this time an undocumented amount of phenobarbital, in addition to propofol sedation. A CT scan of the head, performed at the outside hospital, was reported as normal. The patient was transferred by helicopter to the Texas Children's Hospital. During this time, she was intermittently responsive and able to recognize her father. At Texas Children's Hospital, the patient was admitted to the intensive care unit for close observation, where her mental status continuously improved, with successful extubation. Her blood pressure in the ICU was measured as high as 148/105 mmHg, and her diastolic blood pressure was controlled to the range of 80-100 mmHg with sublingual nifedipine.

The patient and her parents deny any history of urinary tract infections, fever, flank pain, rashes, or weight loss. Her headaches had remitted by the time of arrival to Texas Children's Hospital.

Past Medical History: Chronic sinus problems, affecting both maxillary sinuses and reactive airways disease.

Past Surgical History: None.

Allergies: No known drug allergies.

Medications: Unknown steroid inhaler and liquid asthma inhaler (PRN usage); nonprescription sinus medications.

Family History: No family history of seizure disorders. Menstrual migraine headaches, one episode of pyelonephritis, and idiopathic microhematuria in the patient's mother. Idiopathic, chronic microhematuria in the patient's maternal grandmother, and chronic nephritis (not otherwise specified) in a maternal great-aunt. A maternal great-grandmother was diagnosed with systemic lupus erythematosus. A maternal uncle and maternal great-grandfather had nephrolithiasis.

Social History: No similar illnesses were known in children at the patient's school or home, at the time of the patient's illness (late July). The patient lives with her mother and stepfather in rural Texas. She is a good student, with an outgoing personality and many friends. There was no recent history of family conflicts, and no history of delinquency or drug use. There are no medications in the patient's house other than her usual medications. Two dogs live in the patient's house, and two cats and a cockatoo in her grandmother's house. The patient went swimming in a nearby lake about four days prior to admission.

Review of Systems: No previous skin rashes, cardiac problems, or joint pain.

Physical Exam

Vital Signs: Temperature 99.2° F. Heart rate ~140/min, regular. Blood pressure 140/93 mmHg. Respiratory rate 30/min, unlabored. Oxygen saturation (pulse oximetry) was in high 90% range.

HEENT: Normocephalic, atraumatic. Slight periorbital edema noted. Conjunctiva mildly injected. Fundi showed no gross abnormalities. Blood was seen in the right naris. Oropharynx was clear. Good dentition. Mucous membranes moist. Sinuses non-tender to palpation. Tympanic membranes normal. Neck supple, with no meningeal signs. Shotty, <1 cm, nontender cervical lymph nodes palpable on right.

Chest: Lungs were clear to auscultation bilaterally. Heart rate was tachycardic with regular rhythm; no murmurs were heard.

Abdomen: Soft, nontender, nondistended, bowel sounds present. Tanner I female.

Extremities: No clubbing, cyanosis or edema. No lesions or rashes on skin exam.

Neurological Examination

Mental Status: The patient was somnolent but easily arousable. Speech fluency and comprehension were intact. She followed two step commands and was able to identify all of her relatives.

Cranial Nerves:

Cranial Nerves

Findings

INot tested.
IIPupils equally round and reactive to light and accommodation. No gross visual field defects noted to confrontation.
III / IV / VIExtraocular movements intact.
VSensation in all 3 divisions of the trigeminal nerve intact bilaterally.
VIIFacial movements were symmetric and strong.
VIIIHearing grossly intact bilaterally.
IX / XPalate elevated symmetrically and gag reflex was intact.
XIITongue midline, protruding normally.

 

Motor: Normal tone, bulk, and strength throughout. She was able to reach and grasp items without difficulty. No intention tremor or dysmetria noted.

Reflexes: 1+ in brachioradialis and biceps bilaterally, 2+ in triceps bilaterally, 3+ patellar bilaterally, with 2-3 beats of clonus in both ankles. Equivocal Babinski on the right, with a definite Babinski sign on the left.

Sensation: Intact throughout to pinprick, light touch and vibration. There was no evidence of a sensory level over the trunk.

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