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

(c) 2001 Medical College of Georgia