Patient #26 is a 50 year old white male with progressive multifocal weakness. He first noticed weakness in 1982 when he had difficulty standing on his left heel. Within six months, he developed difficulty holding jars with his left hand. He initially sought help from a chiropractor in 1983 and was given the diagnosis of hemiparesis cruciata of unknown cause.
When the weakness continued to worsen, and he developed bilateral calf cramps, fasciculations, and wasting of the right hand, he was given a diagnosis of amyotrophic lateral sclerosis by a local neurologist. He saw no physicians from 1989 until 1997 despite continued progression of his weakness. In August of 1997 he asked to be evaluated in the ALS research clinic of the VAMC in Houston. By this time, he had developed severe right hand weakness with marked atrophy of the intrinsic hand muscles, mild left arm weakness, and a pronounced left foot drop. He denied any pain, numbness, dysarthria, dysphagia, or bowel/bladder dysfunction.
Past Medical History: Hiatal hernia and gastroesophageal reflux.
Past Surgical History: None.
Allergies: No known drug allergies.
Medications: None.
Family History: No neurological diseases.
Social History: The patient previously worked as a welder and worked with several unidentified chemicals for many years. He has been disabled for 12 years and currently lives alone. He smoked two packs of cigarettes a day for 30 years but quit two years ago. In the past he was a heavy drinker but now has only occasional binges of beer drinking. He has three children.
General: WDWN white male in no acute distress.
Vital Signs: B.P. 146/89; pulse 84; temperature 98 F; respirations 16.
HEENT: NCAT, sclerae anicteric, conjunctivae pink, oropharynx clear and moist without lesions; neck is supple without lymphadenopathy, thyromegaly, or bruits.
Chest: Lungs are clear to auscultation and percussion bilaterally; respiratory excursion is WNL bilaterally; the patient does have mild gynecomastia.
Cardiovascular: Regular rate and rhythm without rubs, gallops, or murmurs.
Abdomen: Soft, NTND without hepatosplenomegaly.
Extremities: No cyanosis, clubbing, or edema.
Skin: There are no significant hyper- or hypo- pigmented lesions.
Mental Status: The patient is alert, and oriented to person, place, time, and situation. His speech is fluent without paraphasic errors. Language is intact with normal comprehension, naming, repetition, reading, and writing. Short and long term memory are intact to bedside testing. MMSE is 30/30.
Cranial Nerves:
Cranial Nerves | Findings |
|---|---|
| I | Not tested. |
| II | Visual acuity 20/20 bilaterally; visual fields were full to confrontation; pupils were 3 mm, equal, round and reactive to light and accomodation; discs were sharp on fundoscopic exam. |
| III / IV / VI | Extraocular movements were intact in all directions; there was no nystagmus and no ptosis. |
| V | Sensation was intact to light touch, pinprick, and cold in all three divisions bilaterally; masseter and temporalis strength were intact. |
| VII | Smile was symmetrical. |
| VIII | Hearing was intact to finger rub bilaterally. |
| IX / X | Gag reflex is intact bilaterally; palate elevates in the midline; normal lingual, labial, and pharyngeal sounds. |
| XII | SCM and trapezius strength intact bilaterally. |
Motor: Tone was WNL. There was no cogwheel rigidity or tremor. There was marked atrophy of the intrinsic hand muscles and thenar and hypothenar eminences on the right side. There was moderate atrophy of the intrinsic hand muscles on the left side. There was marked atrophy of the left calf muscles. Fasciculations were present in all four extremities. Strength in the neck flexors and extensors was 5/5.
Strength | Right | Left |
|---|---|---|
Upper Extremities | ||
| Deltoid | 5 | 5 |
| Biceps | 4- | 4+ |
| Triceps | 4 | 4+ |
| Wrist Extensors | 3 | 4+ |
| Wrist Flexors | 3 | 4 |
| Finger Extensors | 2 | 4- |
| Finger Flexors | 3 | 4 |
| Hand Intrinsics | 2 | 4- |
Lower Extremities | ||
| Iliopsoas | 5 | 4+ |
| Knee Extensors | 5 | 4 |
| Knee Flexors | 5 | 4 |
| Ankle Extensors | 4+ | 2+ |
| Ankle Flexors | 4+ | 2 |
Reflexes: Jaw jerk was trace. Hoffman's and Babinski's signs were absent. There were no crossed adductors.
Reflex | Right | Left |
|---|---|---|
| Biceps | 3+ | 3+ |
| Triceps | 2+ | 2+ |
| Brachioradialis | 2+ | 2+ |
| Patellar | 2+ | 2+ |
| Ankle | 1+ | 0 |
Sensation: Intact to light touch, pinprick, temperature sense, and proprioception throughout; there was a mild decrease in vibratory sense in the lower extremities bilaterally.
Cerebellar: There was no dysmetria on finger-nose-finger; rapid alternating movements were WNL.
Gait: The patient walked with an asymmetrical steppage gait due to his left foot drop. Arm swing and pivot were normal. He could not walk on his heels and had difficulty walking on his toes. He could walk heel-to-toe with some difficulty.
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