History and Physical — Patient 63
Chief Complaint: ALS evaluation
Present Illness: The patient is a 74-year-old farmer referred to the MDA/ALS Center at Baylor College of Medicine for evaluation of progressive bulbar weakness. His first symptom was speech difficulty described as slurring, beginning two years before our evaluation. At that time he was told that he had a transient ischemic attack. His symptoms subsequently improved until ten months prior to referral, at which point his voice became more difficult to understand, and was decreased in volume. Although he regularly went into town in the early morning, he related that he had stopped going into town due to embarrassment at his inability to "get the words out." He began to have progressive swallowing difficulty, associated with choking spells, and increasing sialorrhea, even carrying a towel to wipe away saliva. He also began to experience left arm weakness at this time. He was diagnosed with amyotrophic lateral sclerosis six months prior to our evaluation. Due to progressive difficulty with swallowing, a gastrostomy tube was placed four months before our evaluation.
The patient denied any sensory loss. At the time of evaluation, he was continuing to be active on his farm, but reported excessive generalized fatigue as the day progressed, with some improvement after a mid-afternoon nap. He had experienced progressive head drop, requiring a neck brace for support. He noted accelerating weight loss since his symptoms began to worsen, and at the time of evaluation was taking four cans of supplemental nutrition by bolus feeding through the gastrostomy tube, supplemented by additional water. The day prior to evaluation he fell and sustained a laceration to his forehead. He denied any other falls, headaches, bowel or bladder changes, or head or spine trauma.
Past Medical History: Rheumatoid arthritis. Left shoulder pain, chronic. Anhydrous ammonia pneumonitis in the 1980s.
Past Surgical History: Left knee replacement (remote). Gastrostomy tube placement.
Allergies: No known drug allergies.
Medications: Amitriptyline 25 mg, 1 pill at night for sleep; celecoxib 200 mg for arthritic pain; zolpidem 5 mg h.s.; docusate 100 mg, 1 pill twice daily for constipation; saline eye drops daily for dry eyes.
Social History: Married; living with his wife in Kansas who had been ill recently, requiring multiple surgical procedures. The patient has been working on his farm with more than 250 cattle as well as cotton crops. He quit tobacco use in the 1950s, reported occasional alcohol and coffee use. No history of illicit drug use. No reported exposure to heavy metals.
Family History: The patient's father died in a farming accident in the 1980s. His mother died at age 90 of unknown causes. His brother died in a motor vehicle accident. There is no known family history of muscle or neurologic disease, cancer, cardiac disease, diabetes, or hypertension.
Review of Systems: He denied fever, chills, nausea, vomiting, or diarrhea. He experienced significant weight loss recently. He noted some bright red blood mixed in with stool recently, but denies melena or change in stool caliber. There was no chest pain, shortness of breath, abdominal pain, or urinary symptoms including blood in urine. He did report very dry eyes for the last 6 months, but denied dry mouth symptoms. The family reported emotional lability with episodes of crying recently. His mood has been depressed, due to his decreased ability to work on the farm and to physically care for his wife.
General: No apparent distress, but wiping saliva from mouth constantly. Cachectic, with temporal muscle wasting, and exhibiting a severe head drop.
Vital Signs: Temperature = 96.7 (F); Pulse = 80/min; Respirations = 16/min, unlabored; Blood pressure = 118/78 mm Hg.
HEENT: Left forehead laceration. Sclerae anicteric. Mucous membranes moist.
Neck: No carotid bruits. No lymphadenopathy.
Chest: Clear to auscultation bilaterally with decreased diaphragmatic excursion.
Cardiovascular: Regular rate and rhythm without murmurs.
Abdomen: Gastrostomy tube in left upper quadrant without erythema or exudates around the insertion site. Soft, nontender, nondistended abdomen without organomegaly. Normal bowel sounds.
Extremities: No clubbing, cyanosis, or edema.
Spine: Marked kyphosis present, accentuated by his head drop.
Skin: Skin abrasion on the left forearm.
Mental Status: Folstein Mini-Mental Status Exam score: 24/30, 1 off for date, 0/3 recall at 5 minutes, 2 off for complex commands. Effort was variable during this evaluation, and affect appeared to be depressed.
Speech: Fluent with pronounced nasal quality. Repetition and comprehension were intact. The patient had difficulty with articulating labial, lingual, and guttural sounds.
|II||Pupils 3 mm, equal, round, and reactive to light. Visual fields full to confrontation.|
|III / IV / VI||There was apparently full range of motion on extraocular movements, without nystagmus. V - Sensation of the face to light touch, pinprick, and temperature was intact. Corneal reflex present with incomplete closure of eyes bilaterally.|
|VII||Marked bilateral weakness of the orbicularis oris and orbicularis oculi, with incomplete eyelid closure. Minimal facial expression, with a vertical smile.|
|VIII||Hearing intact to finger rubs bilaterally.|
|IX / X||Symmetric but decreased elevation of the palate. The gag reflex is decreased but present.|
|XI||Sternocleidomastoid strength 3/5 bilaterally.|
|XII||Decreased tongue bulk with few fibrillations noted. There was a tri-grooved appearance of the tongue.|
Motor: Decreased bulk of both distal and proximal muscles. No cogwheel rigidity. Neck flexor strength 4-/5, neck extensor strength 3/5. Strength in major muscle groups of the arm were as below:
Strength in the legs was symmetric, and 4+ to 5-/5 in all major muscle groups. No fasciculations were evident at rest.
Reflexes: Triceps 2+ bilaterally; Biceps 2+ right, 3+ left; Brachioradialis 2+ bilaterally; Patellar 3+ bilaterally (distal spread, R > L); Achilles 1+ right, 2+ left. No crossed adduction noted. Plantar responses were flexor. Hoffman, jaw jerk, palmomental, and snout responses were negative. Myerson sign was present.
Sensation: Intact to pinprick, temperature, light touch, vibration, and proprioception.
Coordination: Finger to nose, rapid alternating movements, and heel to shin maneuvers were normal for level of weakness.
Gait: Steady with full arm swing. Able to toe, heel, and tandem walk without difficulty.