Present Illness: A 55 year old right-handed gentleman noticed bilateral leg weakness when trying to climb flights of stairs while on vacation in New York City. He did not have any difficulty with the first few steps but noticed fatigue after just two flights of stairs, requiring him to hold onto the handrail for support. After a few minutes of rest, he felt the return of his strength to baseline level. However, another attempt up the stairs reproduced the same effect. He attributed the symptoms to a long week of vacation and did not bring up the subject to his primary care physician after he returned home.
He continued to work full-time as a field auditor but over the next five years the weakness progressed. He began having difficulty stepping onto curbs and arising from the floor unassisted. He did not seek medical attention until his weakness began to involve his arms which impaired his line of work. During one particular job, he had to have the boxes brought down to a first floor office because he had difficulty navigating the stairs and lifting boxes above his head.
During this progressive decline, he denied additional symptoms such as difficulty with fine motor movements, sensory changes, muscle cramping, fasciculations, or bulbar symptoms. Three months prior to his visit to our clinic, he sought help at another institution and underwent a battery of testing, including: Lumbar puncture; brain and spinal imaging; nerve conduction and electromyography; and, a right leg biopsy. He did not bring the results but states he was diagnosed with a "myositis" and given five days of intravenous immunoglobulin therapy without any noticeable improvement to his weakness.
Past Medical History: Hypertension and dyslipidemia.
Past Surgical History: Cholecystectomy and right leg muscle biopsy.
Allergies: Bactrim (rash) and Sulfa (rash).
Medications: Diltiazem, hydrochlorothiazide, potassium chloride, and fish oil.
Social History: The patient denies tobacco, alcohol, and illicit drug abuse. He is married with three healthy children. He works as an auditor.
Family History: His father was diagnosed with hypertension, Alzheimer's dementia, and polymyositis. This last diagnosis was made when his father developed severe weakness to the point of being bedbound, and had to be hospitalized for an extended period before regaining his strength and was able to "walk out of the hospital." It was unclear how the diagnosis was made or what treatments were given. His mother and brother were diagnosed with osteoarthritis. His three children are healthy.
Review of Systems: A complete review of systems was performed and was negative with the exception of fatigue and loss of muscle bulk in his arms that was brought up by the examining physician.
Vital Signs: Temperature 98.2 F, Blood Pressure 150/93, Heart Rate 62, Respiratory Rate 18, Weight 164 lbs.
General: Awake, comfortable, cooperative, thin, and in no acute distress.
HEENT: Neck was supple. He has a thin facies with frontal balding.
Cardiovascular: Regular rhythm without any murmurs and with normal S1 and S2 sounds.
Chest: Clear to auscultations bilaterally. Breathing was unlabored.
Abdomen: Soft and non-tender. Normal bowel sounds. No organomegaly.
Extremities: No edema. Distal pulses were palpable.
Mental Status: Alert and oriented to person, place, and time. He followed two step commands. Language and speech were normal. Good fund of knowledge.
Cranial Nerves: Weak orbicularis oculi. Intact orbicularis oris. Pupils were equal and symmetrically reactive to light and accommodation. Fundoscopy examination showed normal discs bilaterally. Visual fields were fully intact. Extraocular movementes were full. No ptosis or lid lag. Facial sensation was intact. Palate raised symmetrically. Gag reflex was symmetric. Sternocleidomastoid and trapezius muscles were weak bilaterally rated at 4. Tongue bulk and strength assessments were normal. There were no tongue fasciculations.
Motor: Patient had to hold onto the wall to pull himself onto the examination bed. Bilateral scapular winging was noted. Overall thin muscle bulk but more pronounced at the leg adductors. Clavicles were not horizontal. He did not have the "poly-hill" sign. He showed normal muscle tone, and no fatigability was noted. He did not demonstrate pronator drift and fine movements were symmetric and normal. There were no features of fasciculations or myotonia. He was unable to arise from the ground with legs crossed without assistance.
Strength | Right | Left |
|---|---|---|
Upper Extremities | ||
| Deltoids | 3 | 3 |
| Infraspinatus | 3 | 3 |
| Biceps | 5 | 5 |
| Triceps | 5 | 5 |
| Wrist Extensors | 5 | 5 |
| Wrist Flexors | 5 | 5 |
| Finger Extensors | 5 | 5 |
| Finger Flexors | 5 | 5 |
| Hand Intrinsics | 5 | 5 |
Lower Extremities | ||
| Iliopsoas | 2 | 2 |
| Hip Adductors | 2 | 2 |
| Hip Abductors | 2 | 2 |
| Knee Extensors | 5 | 5 |
| Knee Flexors | 5 | 5 |
| Ankle Extensors | 5 | 5 |
| Ankle Flexors | 5 | 5 |
Sensation: He reported symmetrical light touch, temperature, pin prick, vibration, and proprioception.
Reflexes: 2+ symmetrical reflexes at the biceps, brachioradialis, patellar, and Achilles. The plantar response was down-going. No pathological reflexes were noted.
Coordination: Bilateral finger-to-nose and heel-to-shin testing were normal.
Gait: Swaying of the hips was noted with straight away gait. He demonstrated a mildly increased lumbar lordosis. He showed normal stride with appropriate arm swing. Turning was normal. Tandem gait was steady and normal. Romberg sign was absent.
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