16 year old right handed man who was referred to me to evaluate for ALS. He had a two year history of progressive upper extremity weakness. This started in his right thumb and progressed to involve his entire Right hand and now involves his left hand as well. He denies weakness anywhere else. He also denies sensory findings. He denies trauma or pain. No family history of similar problems.
Examination shows normal mental status, speech and language. Cranial nerves are normal. His upper extremity is normal with the exception of the distal hand. He has wasting of the intrinsic muscles of both hands. He has 3/5 strength of his intrinsic hand muscles and 4/5 in the wrist flexors and extensors. His strength is normal elsewhere He has absent upper extremity reflexes and normal lower extremity reflexes. He has normal lower extremities and no Babinski sign. His gait is normal. His rapid alternating movements are normal.
Normal B12, Folate, Thyroid functions, methyl malonic acid, and homocysteine level.
|R Ulnar Sensory||Palm||Wrist||33||1.7||8.0|
|R Ulnar Motor|
|R Median Motor|
|L Ulnar Motor|
|L Peroneal Motor|
|Nerve||Latency||Max Normal Latency|
|Muscle||Insertional Act||Fibs||Fasc||Vol Motor Unit Potentials|
|L Biceps Brachii||Normal||0||0||Polyphasic
|L 1st Dorsal Int||Increased
|3+||0||Marked Decr Recruitment
|L Abd Pol Brevis||Increased||0||0||Decreased Recruitment
|L Tib Anterior||Normal||0||0||Normal|
|L Dorsal Paraspinals||Normal||0||0||Normal|
|L Cerv Paraspinals||Increased||+/-||0||Normal|
|L Med Gastroc||Normal||0||0||Normal|
|L Vastus Lat||Normal||0||0||Normal|
|R Flex Carp Uln||Increased
|3+||0||Increased Firing freq
Markedly Decr Recruitment
|R Flex Carp Rad||Increased||2+||0||Decreased Recruitment
MRI of the Brain is Normal.
MRI of the Cervical Spine shows a lesion at C6-C8. It is not seen in T1 weighted images but is with T2. It enhances with Gad.
I do not believe that this patient has ALS but rather this cervical lesion is related to his symptoms. I am concerned about the absence of sensory findings as this lesion is posterior. What do you think this person has?
I think this may be a low grade glioma or possibly sarcoid. I plan to do a L.P. and Chest X-ray on this patient. If these do not give a diagnosis than my plan would be to go at it surgically. What are the opinions of list members.
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Revised November 22, 2001
(c) 2001 Medical College of Georgia