Cervical Cord Compression and ALS

by

Michael H. Rivner, M.D.

73-year-old woman who was in fairly good health until 3 years ago when she developed low back pain. She had two lumbar laminectomies that produced relief of her pain. Her low back pain was relieved following the second surgery. About 18 months ago, she noticed that she was dragging her left leg. A head MRI showed a right lacunae infarct. She developed increasing weakness of her left leg and then her right leg. More recently she developed upper extremity problems. She had been walking with a cane but now needs a walker. She has been falling a lot. She has vague sensory complaints. She also is having problems with bladder and bowel incontinence and wears Attends. She also complains of syncopal spells and feeling faint and dizzy when she stands up. She denies dysphagia and dysarthria.

Exam: Mental status, speech and language functions are normal. CN: normal except for tongue fasciculations. Motor: Wasting of bilateral FDI and left thenar eminence. She had wasting of her left thigh. LUE was 4/5. RUE was 4+/5. LLE- hip 4/5, knee 4-/5, ankle dorsiflexion 1/5, and plantar flexion 2/5. She has no movements of her toes. RLE-distally 4-/5, proximally 4+/5. Her gait was unsteady and slow. She had a left foot drop. He had fasciculations of her shoulders and tongue. Sensory exam was normal to fine touch but vibratory was diminished in both lower extremities. Her reflexes were 2+ except her ankle jerks which were absent. She has a left Babinski's sign. She has a bilateral Hoffman’s and palmar-mental signs. She is not orthostatic. She does not have spasticity.

Nerve conductions:

Motor

Nerve	Amp-prox	Amp–dist	CV	DL	F-wave
L Peroneal Motor	NR	NR	NR	NR
L Ulnar Motor	1.1	1.3	37.5	3.6
L Ulnar Motor (BE)	1.2	1.3	46.8	3.6
R Ulnar Motor	7.3	8.0	49.3	2.8	30.6
R Tibial Motor	0.3	0.5	40.2	5.7	NR

Sensory

Nerve	Amp	DL	CV	Distance
Left Sural	2.4	4.6	38.2	14.5
Left Ulnar Palmar Sen	6.5	2.3		8.0
Right Ulnar Palmar Sen	14.1	2.0		8.0
Right Sural	3.8	36	59.1	14.0

Needle Exam:

Muscle	Insert Act	Fib	Fasc	VMUP
R FDI	increased Positive Waves	3+	1+	Decreased recruitment Increased firing freq Increased Size
R Deltoid	Increased Positive Waves	1+	0	Increased Size Decreased Recruitment Increased Firing Freq
R Vastus Lateralis	Increased Positive Waves	2+	0	Increased Size Markedly decreased Recru Increased Firing Freq
R Med Gastroc	Increased Positive Waves	3+	0	Decreased Recruitment Increased Firing Freq
L EIP	Increased Positive Waves	1+	0	Decreased Recruitment
L Biceps Brachii	Increased CRDs Positive Waves	3+	0	Decreased Recruitment
L Tib Ant	Increased Positive Waves	3+	0	No Units
L Iliopsoas	Increased Positive Waves	2+	0	Increased Size
L Tongue	Increased Positive Waves	1+	0	Normal
L Anal Sphincter	Increased	1+	0	Decreased Recruitment
R Thoracic Paraspinals	Poor Relaxation	0	0	Normal
L Thoracic Paraspinals	Increased Positive Waves CRDs	1+	0	Decreased Recruitment
R Biceps Brachii	Increased Positive Waves	1+	1+	Normal
R Thoracic Paraspinals	Increased Positive Waves	2+	0	Decreased Recruitment
R Orb Oculi	Increased	+/-	0	Normal
R Mentalis	Normal	0	0	Normal

Imaging Studies:

MRI of Head: Showed a small lacunae infarction in the right posterior thalamus probably not the cause of the patient’s left leg weakness. Cortical Atrophy was also seen.

MRI of L/S spine: Multilevel lumbar degenerative disease. Arachnoiditis at L3-4 and L4-5 and neural foraminal stenosis. Sacral region not well visualized on films.

MRI of Cervical Spine:

Lumbar Puncture:

CSF protein 59

CSF Glucose 56

13 RBCs

1 WBCs

Questions: I am bothered by several things:

1) The incontinence.

2) The Cervical MRI Should it be operated on even though I believe that this cannot explain all this patients symptoms. She has L/S disease but her lower extremities are behaving more like ALS then spine disease.

3) Does anyone think this is not ALS?

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Modified March 31, 2003