History and Physical — Patient 78
Present Illness: A 55 year old, right-handed Caucasian male presented to the Veterans Affairs Medical Center in Houston, complaining of worsening clumsiness. The patient was in his usual state of health until 6 months prior to evaluation, when he developed a tingling sensation on the right side of his body. This symptom was followed by rapidly progressive clumsiness, more on the right than on the left, together with unsteady gait, multiple falls, blurring of vision with diplopia, and dysarthria. By one month into his illness, he was impaired to the point that he could no longer work on his dairy farm. By two months into his illness, he was dependent on a wheelchair for mobility. At that time the patient was living in the upper Midwest, where he was admitted to a local community hospital for evaluation. He underwent a reportedly unremarkable MRI study of the brain, as well as negative CT scans of the chest and abdomen, and had negative Lyme serology. Based on a report of elevated serum anti-gliadin IgA titers, the patient was diagnosed with celiac sprue and placed on a strict gluten-free diet. Despite strict dietary measures, his condition continued to decline, requiring him to sell his farm and move to Houston to live with family members. Following his arrival in Houston, his relatives noted mild impairment of cognitive function with periods of confusion, as well as personality changes with paranoid ideation.
Past Medical History: Otherwise healthy. No past surgical procedures.
Allergies: No known drug allergies.
Medications: Folate 1 mg per day, amitriptyline 10 mg per day.
Social History: Tobacco use of 2-3 packs/day for more than 35 years. No alcohol or illicit substance abuse. Prior to his illness, the patient had worked as an independent dairy farmer in the Midwest. He denied any unusual chemical exposures.
Family History: His mother died of lung cancer, and his father had diabetes mellitus.
Review of Systems: The patient denied fever, hemoptysis, and night sweats. He did complain of a non-productive cough. He had lost weight since the onset of illness, but denied abdominal pain, diarrhea or constipation. He did complain of nausea and occasional vomiting in the morning, and of dysphagia for liquids.
General: Cachectic elderly appearing male, appearing older than stated age, but otherwise in no acute distress.
Vital Signs: Temperature 97.3 F, pulse 48/min., respirations 18/min., blood pressure 135/85 mmHg.
HEENT: Normocephalic, no evidence of trauma. Sclerae were anicteric, nares and oropharynx were clear, mucous membranes were dry.
Neck: Supple, no lymphadenopathy, no carotid bruits heard.
Chest: Lungs were clear to auscultation bilaterally, with good air movement.
Cardiovascular: Regular heart rate and rhythm, no murmurs. No jugular venous distention.
Abdomen: Soft, non-tender to palpation, no masses or hepatosplenomegaly felt.
Extremities: Dorsalis pedis and posterior tibial pulses were 2+ and symmetric. There was no cyanosis or edema, and no supraclavicular, axillary or inguinal lymphadenopathy.
Mental Status: Awake and alert, Folstein Mini-Mental Status Exam score 27/28 (missed one point for orientation to date; written tasks not scored because of severe dysmetria). The patient exhibited mild motor impersistence during the examination.
Speech: Speech was fluent but dysrhythmic, with varying intensity. There was also moderate dysarthria to all phonemes, most pronounced with guttural sounds. In addition, the patient's voice had a hypernasal quality, but did not appear to fatigue.
|II||Visual acuity by near card was 20/20 bilaterally with correction. Visual fields were full to confrontation. Pupils were equal, round and reactive to light and accommodation. Funduscopic exam was normal.|
|III / IV / VI||Patient reports blurring of vision that resolves with closing one eye; however, no diplopia was elicited either with gaze or with red lens testing. No diplopia or ptosis were noted with sustained upgaze at 1 minute. Extraocular movements were full range. Saccades were coarse, with notable ocular dysmetria.|
|V||Facial sensation was intact to light touch, temperature, and pinprick. Corneal reflex was intact bilaterally. Strength in the masseters and temporalis muscles was intact.|
|VII||Facial motor strength and expression were full.|
|VIII||Hearing was intact to finger rub bilaterally.|
|IX / X||Palatal elevation was symmetric, and the uvula midline. There was no palatal tremor. Gag reflex was intact.|
|XI||Sternocleidomastoid strength was intact bilaterally.|
|XII||Tongue protruded in the midline without atrophy or fibrillations.|
Motor: Strength was 5/5 throughout in the extremities, with the notable exception of 4/5 strength in the deltoids bilaterally. There was no detectable fatiguability of the deltoid or triceps with repetitive contractions. Tone was within normal limits.
Reflexes: Tendon reflexes were 2/4, brisk and symmetric at the biceps, triceps, brachioradialis, patella, and Achilles. Babinski response was flexor bilaterally. Hoffman sign was absent. Glabellar response was negative. A snout reflex was present.
Sensation: Sensation was intact to light touch, temperature and pin prick throughout. Vibratory sense was decreased in the toes bilaterally. Proprioception was intact.
Coordination: Marked dysmetria on finger-to-nose and heel-to-shin maneuvers was present bilaterally, with bilateral inability to check flexion movements. Rapid alternating movements were slow, irregular in rhythm and inaccurate.
Gait: Unable to stand because of postural instability; Romberg maneuver and gait could not be tested.