Motor Neuron symptoms with Cervical Cord lesion

by

Michael H. Rivner, M.D.

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.

EMG and Nerve Conduction Studies

Nerve Conductions

Nerve	Stim	Record	Amp	Latency	CV	Dist
R Ulnar Sensory	Palm	Wrist	33	1.7		8.0
R Ulnar Motor
	Elbow	Hypothenar	1.4	10.0	50.9	28.5
	Wrist	Hypothenar	1.6	4.4		7.0
R Median Motor
	Elbow	Thenar	4.9	8.5	57.0	25.1
	Wrist	Thenar	4.9	4.1		7.0
L Ulnar Motor
	Elbow	Hypothenar	1.9	8.3	59.2	29.0
	Wrist	Hypothenar	2.0	3.4		7.0
L Peroneal Motor
	Knee	EDB	8.1	10.9	47.0	28.2
	Ankle	EDB	8.7	4.9		7.0

F-waves

Nerve	Latency	Max Normal Latency
R Ulnar	NR	33.3
R median	NR	32.2
L Ulnar	46.0	31.8
L Peroneal	50.5	51.0

Needle Examination

Muscle	Insertional Act	Fibs	Fasc	Vol Motor Unit Potentials
R Deltoid	Normal	0	0	Normal
L Biceps Brachii	Normal	0	0	Polyphasic Increased Size Decreased Recruitment
L Triceps	Increased Positive Waves	1+	0	Decreased Recruitment Increased Size
L 1st Dorsal Int	Increased Positive Waves	3+	0	Marked Decr Recruitment Increased Size
L Abd Pol Brevis	Increased	0	0	Decreased Recruitment Increased Size
L Tib Anterior	Normal	0	0	Normal
L Iliopsoas	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 Positive Waves	3+	0	Increased Firing freq Markedly Decr Recruitment Increased Size
R Flex Carp Rad	Increased	2+	0	Decreased Recruitment Increased Size
R Brachioradialis	Normal	0	0	Normal

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