Present Illness: A 22 year old man, previously in good health, presented to an outside hospital with acute abdominal pain and was diagnosed with appendicitis. Based on a history of possible von Willebrand disease, the patient was treated preoperatively with DDAVP (desmopressin acetate) to avoid bleeding problems. He underwent laparoscopic appendectomy on his second hospital day, and continued to receive regular doses of intravenous DDAVP until hospital day 5. From the first post-operative day, his family noted personality changes in the patient, followed by confusion and disorientation, nausea and vomiting. Laboratory studies drawn on hospital day 6 revealed a serum sodium concentration of 114 mEq/L (normal range 136-146 mEq/L). The patient was treated with intravenous hypertonic saline. Repeat serum sodium levels were 123 mEq/L the next morning and 130 mEq/L later that day. The patient was discharged to home as his laboratory studies improved.
According to his family, however, the patient never returned to his usual level of function. He was restless, with diminished concentration, and was noted to have purposeless movements. On post-operative day 7, the patient was agitated, unable to recognize his family members, and had difficulty speaking. At times he stared and was unresponsive. The patient was taken to the emergency room of another hospital, where he received diphenhydramine (Benadryl) for sedation, with recommendations for an outpatient MRI of the brain in the following week. The next morning, the patient was increasingly unresponsive. He was transported back to the emergency department where he was observed to have generalized tonic-clonic seizure activity. He was treated with intravenous phenytoin and phenobarbital, intubated for airway protection, and admitted to the intensive care unit. An electroencephalogram showed diffuse slowing, with periodic sharp waves in both frontal regions.
The patient was transferred to The Methodist Hospital for further care.
Past Medical History: Possible von Willebrand disease, apparently diagnosed several years earlier when the patient had excessive bleeding after a tooth extraction, but not confirmed by laboratory studies available to us.
Allergies: No known drug allergies.
Medications: Fosphenytoin.
Social History: No history of tobacco, alcohol, or illicit substance abuse. The patient was a college graduate operating his own business.
Family History: A grandfather possibly had von Willebrand disease. There was no other history of bleeding disorders, and no history of epilepsy in the family.
Review of Systems: As above.
General: The patient was intubated, unresponsive, and appeared critically ill.
Vital Signs: Temperature 97.0° F, pulse 133/min., blood pressure 135/57 mm Hg.
HEENT: Normocephalic, atraumatic, sclerae were anicteric. Nares were clear. Oral endotracheal tube was in normal position.
Neck: Supple, no carotid bruits, and no lymphadenopathy.
Chest: Lungs were clear to auscultation bilaterally, with no wheezes, rales or rhonchi.
Cardiovascular: Tachycardic, regular rhythm, no murmurs heard.
Abdomen: Soft, non-tender to palpation, non-distended, with normoactive bowel sounds, no masses or hepatosplenomegaly. Healing site consistent with recent laparoscopy.
Extremities: Pulses were 2+ and symmetric in dorsalis pedis and posterior tibial arteries. No cyanosis or edema noted.
Mental Status: Obtunded. The patient would open his eyes to verbal stimuli, but would not follow commands. He made no attempts at verbalization.
Cranial Nerves:
Cranial Nerves | Findings |
|---|---|
| I | Not tested. |
| II | Patient would react to visual threat bilaterally. |
| III / IV / VI | Range of extraocular movements was grossly intact, assessed by observing spontaneous eye movements; the patient would not regard or track movement. There was no apparent internuclear ophthalmoplegia, no spontaneous nystagmus, and no forced eye deviation. |
| V / VII | Corneal reflexes were intact bilaterally. Facial appearance was grossly symmetric. |
| VIII | The patient would respond to verbal stimuli on the left and right. |
| IX / X | He was intubated. Reflex response to endotracheal tube manipulation was present. |
| XI | Not assessed. |
| XII | Tongue displaced by endotracheal tube, could not be assessed in detail. |
Motor: The patient intermittently moved all four extremities spontaneously with grossly symmetric strength. Formal strength testing could not be performed.
Reflexes: Tendon reflexes were 2/4, brisk and symmetric at the biceps, triceps, brachioradialis, patella, and Achilles. No definite upper motoneuron signs were seen.
Sensation: There was no withdrawal to nail bed pressure in any extremity.
Coordination: Unable to test.
Gait: Unable to test.
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