Chief Complaint: The patient is a 28 year old female with autoimmune hepatitis, who presented to an emergency room in East Texas with acute, severe headache, accompanied by neck stiffness, left eyelid droop and difficulty moving her left eye.
Present Illness: The patient was in her usual state of health until one and a half weeks prior to admission, when she developed acute, severe right parietal and bitemporal headache. This headache was accompanied by photophobia and neck stiffness, and was described by the patient as the worst headache of her life. She went to an emergency room about one week later, and received a shot to treat her headache. Records are not available from that evaluation.
One day later, the patient developed a left-sided eyelid droop, and transient speech disturbance. Her husband relates that she occasionally appears to "space out," losing track of a conversation or abruptly changing the subject, but still able to speak coherently during episodes. She was re-evaluated at the local emergency room, and was seen by a neurologist. A CT scan of the head was suboptimal due to motion artifact, but showed no gross hemorrhage, mass or other abnormality. Due to clinical suspicion for aneurysmal subarachnoid hemorrhage, the patient was transferred to the Methodist Hospital for further diagnosis and management. While awaiting transfer, the patient developed a fever, and received an empiric dose of intravenous ceftriaxone.
The Neurology Service at the Methodist Hospital was consulted.
Past Medical History: The patient has autoimmune hepatitis diagnosed 10 years ago with liver biopsy, complicated by liver cirrhosis, portal hypertension and grade 2 esophageal varices. Splenomegaly has been noted in the past. Screens for ceruloplasmin, alpha-1 antitrypsin, iron storage disorders, and infectious causes of hepatitis have been negative. Her liver disease has been treated intermittently with prednisone and azathioprine in the past. Azathioprine was recently restarted, and the dosage is being advanced with the goal of eventual reduction in prednisone dosage. Initial screens were recently requested by the Hepatology service as part of a pre-transplant screening evaluation. The patient has a history of ulcer disease, with well healed duodenal ulcerations seen at endoscopy two months prior to admission. There is a remote history of blood transfusion, but details were not available.
Past Surgical History: She has had no surgical procedures.
Allergies: Keflex and Compazine.
Medications: Azathioprine 50 mg b.i.d., prednisone 20 mg/day, calcium tablets with vitamin D daily, spironolactone 25 mg b.i.d., rabeprazole 20 mg b.i.d., propranolol 20 mg b.i.d.
Social History: The patient is a former smoker whose last cigarette was five years ago. She denies the use of alcohol or drugs. She is married, with one daughter.
Family History: There is no history of neurological diseases or liver disease in the family.
Review of Systems: There has been no weight loss, chest pain or dyspnea, no dizziness or blurred vision, no pruritus, and no arthralgias. There is no history of viral hepatitis. The patient denies diarrhea, vomiting, melena, hematemesis, or dysphagia.
General: The patient is an awake, African-American female in no acute distress, with obvious left ptosis.
Vital Signs: Temperature 101 F, Pulse 97/min., Respirations 18/min., Blood Pressure 122/70 mmHg.
HEENT: Normocephalic, atraumatic head. Sclerae anicteric, irises normal; tympanic membranes clear, mucosal membranes moist, oropharynx clear.
Neck: Head is normocephalic, without evidence of trauma. There is minimal scleral icterus. The oropharynx is clear.
Chest: Clear to auscultation bilaterally.
Cardiovascular: Regular rate and rhythm, no murmurs, rubs or gallops heard. There is no jugular venous distension.
Abdomen: Mildly distended, non-tender, no definite ascites present. Bowel sounds present. No hepatosplenomegaly.
Extremities: No edema, clubbing or cyanosis are evident. SKIN: No rashes, ecchymoses or angiomata observed.
Skin: One 3 cm hyperpigmented spot near midline on the right side of the back, as well as a smaller hypopigmented spot on the upper buttock.
Mental Status: The patient was alert and oriented to time, place, person and situation. She was somewhat irritable during the examination, and at times agitated. She had difficulty cooperating with the examination. Spontaneous speech was fluent, without labial, lingual or guttural dysarthria on single syllables. The patient had difficulty pronouncing complex phrases, such as "Methodist Episcopal," which she tended to slur.
Cranial Nerves:
Cranial Nerves | Findings |
|---|---|
| I | Not tested. |
| II | Left pupil is reactive from 5 mm to 4 mm. Right pupil is 3 mm, reactive to 2 mm. Fundoscopic exam revealed normal discs without papilledema. Visual fields full to confrontation bilaterally. |
| III / IV / VI | Left ptosis, with limited upgaze in the left eye, limited downward gaze in the left eye, inability to adduct the left eye. Right eye has normal extraocular movements. |
| V | Intact facial sensation, intact bulk and tone of masseters and temporalis bilaterally. Normal corneal responses bilaterally. |
| VII | Normal strength of facial musculature and symmetry of facial expression. |
| VIII | Hearing intact to finger rub bilaterally. |
| IX / X | Palate elevates symmetrically on the midline; gag reflex present. |
| XI | Normal strength of sternocleidomastoids and trapezius muscles bilaterally. |
| XII | Tongue protrudes in the midline, with no atrophy, no fibrillations. |
Motor: The patient's strength is 5/5 throughout the major muscle groups.
Reflexes: Tendon reflexes are symmetric, 2+ in the upper extremities. Symmetric and 3+ with distal spread and crossed adduction at the knees. Tendon reflexes are 3+ left ankle, 4+ at the right ankle with non-sustained clonus. There is a right extensor plantar response. The left plantar response is flexor. No abnormal jaw jerk, glabellar tap or palmomental responses were seen.
Sensation: Intact to pinprick, light touch, vibration, and proprioception throughout.
Coordination: Normal finger to nose and heel to shin maneuvers bilaterally. Normal rapid alternating movements. No loss of check of flexion movements, and no titubation.
Gait: Unsteady, wide based. The patient could toe and heel walk. She had particular difficulty with tandem gait.
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