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Cortical function in progressive lower motor neuron disorders and amyotrophic lateral sclerosis: a comparative PET
J J Kew1, D J Brooks, R E Passingham
1MRC Cyclotron Unit, Hammersmith Hospital, London, UK.
Neurology
|June 1, 1994
Summary
Amyotrophic lateral sclerosis (ALS) patients show reduced resting brain blood flow and abnormal sensorimotor cortex activation during movement, unlike lower motor neuron degeneration (LMND) patients. These findings suggest pyramidal neuron loss in ALS.
Area of Science:
- Neuroscience
- Neurology
- Medical Imaging
Background:
- Amyotrophic lateral sclerosis (ALS) and progressive lower motor neuron degeneration (LMND) are debilitating neurological conditions affecting motor function.
- Understanding the underlying cortical dysfunction in these diseases is crucial for diagnosis and treatment.
Purpose of the Study:
- To compare resting and movement-induced cortical function in patients with ALS and LMND.
- To investigate regional cerebral blood flow (rCBF) patterns in the sensorimotor cortex during motor tasks.
Main Methods:
- Positron Emission Tomography (PET) was used to measure rCBF.
- Five patients with progressive LMND, six with ALS, and six age-matched controls participated.
- rCBF was assessed at rest and during a joystick-controlled hand movement task.
Main Results:
- ALS patients exhibited significantly lower resting rCBF in the primary sensorimotor cortex (SMC) compared to controls and LMND patients.
- During movement, ALS patients showed exaggerated rCBF increases in the SMC, SII cortex, and premotor areas.
- LMND patients demonstrated increased rCBF in the anterior insular cortex during movement.
Conclusions:
- Reduced resting rCBF and abnormal sensorimotor cortex activation in ALS patients suggest pyramidal neuron loss.
- Recruitment of perisylvian areas (insular, SII cortices) during movement in both ALS and LMND indicates their role as accessory sensorimotor areas, possibly compensating for limb weakness.