Neurology: Case of the Month

History and Physical — Patient 51

History

Present Illness: A 40 year old homemaker, previously in good health, experienced a transient episode of left-sided face, arm, and leg numbness lasting about 10 minutes. The following day, she developed recurrent left-sided numbness, with speech disturbance described as difficulty with articulating words. She was taken to a nearby hospital, but on arrival, her symptoms of numbness and dysarthria had spontaneously resolved. A computed tomographic scan of the head was reported as normal, and the patient was admitted for observation. The following morning at 2 a.m., she developed a seizure described by a nurse as "grand mal," lasting about 30 seconds with tongue biting and bladder incontinence, and followed by transient confusion and aphasia. Electrolytes and a complete blood count were within normal limits. A lumbar puncture was performed, revealing clear, colorless cerebrospinal fluid with glucose 72 mg/dl, protein 55 mg/dl, 0 white blood cells, and 0 red blood cells). An MRI study of the head with diffusion-weighted and FLAIR sequences was read as normal, and an electroencephalogram likewise showed no abnormalities. Phenytoin was initiated, and the patient returned to her baseline mental status.

On the third hospital day, the patient developed a low-grade fever (temperature to 102 F) and delirium with psychotic features. She was able to follow commands. A chest x-ray revealed patchy infiltrates in the left lower lobe. She was treated with ceftriaxone and clindamycin for a presumed aspiration pneumonia, and with olanzapine for management of psychotic symptoms. Her fever persisted, and she became aphasic, with diminished responsiveness. Blood and urine cultures showed no growth. Doxycycline, acyclovir and methylprednisolone were added to her medical regimen, and the patient was intubated for airway protection. A second lumbar puncture was performed, revealing clear, colorless cerebrospinal fluid with an opening pressure of 26 cm H2O, with glucose 81 mg/dl, protein 80 mg/dl, 0 white blood cells, and 0 red blood cells. A repeat EEG showed diffuse slowing with the development of a burst-suppression pattern. On the sixth day of her hospitalization, the patient was transferred to the Ben Taub General Hospital for further evaluation and management.

On initial examination in the ICU, the patient exhibited intermittent forced eye deviation to the right, with subtle, rhythmic twitching of the right arm. The serum phenytoin level was 9.7 µg/ml, and the patient was loaded with additional phenytoin, with transient improvement in the level of consciousness and disappearance of spontaneous motor manifestations. The Neurology Service was consulted for further evaluation, and noted that the patient's level of consciousness had again declined, without evident seizure activity.

Past Medical History: Previously healthy. The patient's mother reported an apparent insect bite on the neck about five days prior to her hospital admission. No history of surgeries or blood transfusions.

Allergies: No known drug allergies.

Medications: At home — Ibuprofen as needed for occasional headache. In hospital — Aspirin 325 mg q.d., fosphenytoin 100mg q8h, amlodipine 5 mg q.d., famotidine 20 mg q12h, acyclovir 600 mg q8h, digoxin 0.25 mg q.d., methylprednisolone 250 mg q6h, clonidine 0.1 mg q6h.

Family History: Diabetes mellitus in a paternal aunt and maternal grandmother. Her father had hypertension, and died of congestive heart failure at age of 78. A second cousin died at age 30 of breast cancer.

Social History: The patient was born in India. She spent 12 years in Saudi Arabia before becoming a Texan (12 years in Dallas and three years in Houston). She is married with three healthy teenage children. There is no history of smoking, alcohol or illicit drug use. There was no history of recent travel to foreign countries or outside the Houston area. There was no history of swimming in freshwater lakes. Peak temperatures in Houston in October ranged from 80-85 F (27-29 C), and the weather had been unusually dry.

Physical Exam

General: Well-developed, well-nourished appearing lady lying in supine position, orotracheally intubated and with nasogastric tubing.

Vital Signs: Temperature: 102 F (39 C). Blood Pressure: 154/70 mmHg. Pulse: 120/min, regular. Respiration: 12/min (ventilator in assist control mode). Pulse oximetry showed 99-100% saturation.

HEENT: Normocephalic, no evidence of trauma; anicteric sclerae; conjunctival injection is evident. No oropharyngeal lesions noted. Neck: Signs of meningismus are present. No lymphadenopathy evident; normal jugular venous pulsations present.

Chest: Clear to auscultation bilaterally.

Cardiovascular: Tachycardia; no murmurs, gallops, or rubs detected.

Abdomen: Soft, not distended; no organomegaly. Normally active bowel sounds.

Extremities: No clubbing, cyanosis or edema. Skin examination disclosed no rashes or other lesions.

Neurological Examination

Mental Status: Eyes opened to sternal rub and roamed preferentially toward the left side, but the patient did not regard objects or persons. There was no forced eye deviation. There was no response to visual threat.

Cranial Nerves: Both pupils responded to light, constricting from 2 mm to 1 mm. Consensual responses were present. The oculocephalic response to passive head movement was present and symmetric. There was no apparent facial asymmetry. There was no response to auditory stimuli.

Sensorimotor: Painful stimuli applied to the extremities elicited withdrawal equally in all four limbs, but no localization of stimuli or facial grimacing was observed. No spontaneous movements of the trunk or extremities were evident.

Reflexes: Symmetrically very brisk, with distal spread throughout.

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