Basic Human Neuroanatomy
A Clinically Oriented Atlas 
Case 199 Answers

1. Can this patient’s signs and symptoms be localized to one single area of the central nervous system? If so, where is the neurologic lesion located, and which side of the central nervous system is involved?

No, long tract signs (motor or sensory) do not localize a lesion well unless they are intersected by other focal signs (e.g., cortical dysfunction, cranial nerve or brain stem signs, lower motor neuron dysfunction, deep tendon reflex abnormalities, etc.) (see slide #1).

2. What regions, levels, and/or structures of the nervous system are involved with signs or symptoms of lower motor neuron (LMN) disease?

a. Mild atrophy and weakness of the thenar eminence muscles bilaterally
These signs are most likely caused by compression of the median nerves bilaterally at the wrist due to the patient’s need to support her weight with her hands on her walker (or cane) (i.e., bilateral carpal tunnel syndrome). This is supported by the pattern of the mild sensory signs and symptoms, which conforms to a median nerve distribution.

3. What regions, levels, and/or structures of the central nervous system are involved with signs or symptoms of upper motor neuron (UMN) disease?

a. Brisk jaw jerk reflex
Corticonuclear (corticobulbar) tracts above the mid-pons

b. Hyperreflexia, including Hoffmann’s signs, in both upper limbs
Corticospinal tracts above the cervical (C5) spinal cord

c. Increased muscle tone (spasticity), hyperreflexia, extensor plantar reflexes in both lower limbs
Corticospinal tracts above the lumbar spinal cord

d. Weakness in both lower limbs
Corticospinal tracts above the lumbar spinal cord. The pattern of the weakness suggests an upper motor neuron process.

4. The findings in this case represent a classic neurologic syndrome. What is the name of this syndrome?

Motor neuron disease: Primary lateral sclerosis (PLS) type

5. In general, what type of pathologic process is involved in this case?

Neurodegenerative process involving loss of UMN cell bodies (including giant pyramidal cells [Betz]) in layer 5 of the precentral gyrus and premotor area (areas 4, 6) of the frontal lobes, degeneration of the corticospinal tracts, and preservation of the alpha motor neurons (LMNs) in the cranial nerve motor nuclei and anterior horns of the spinal cord.

6. What diagnostic procedure(s) would you undertake at this point?

References:
1. Pringle CE, Hudson AJ, Munoz DG, et al. Brain 1992;115:495-520.
2. Marti-Fabregas J, Pujol J. Neurology 1990;40:1799-1800.
3. Claassen DO, Josephs KA, Peller PJ. Arch Neurol 2010;67:122-125.
4. Cosgrove J, Jamieson S, Chowdhury FU. Neurology 2015;84:e206. 


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