It is part of the upper motor neuron
syndrome and many neurological diseases and disorders.
Age, y/sex Signs Pattern MRI EEG 1 12/F Yes Yes Yes No 2 4/M Yes Yes Yes No 3 12/M Yes No Yes Yes 4 12/M Yes No Yes No 5 78/F Yes No Yes - 6 8/M No Yes Yes - 7 13/M No Yes - Yes 8 7/F No No - - 9 12/F No No - - At time of follow-up investigation Upper motor neuron
([dagger]) Case- Reported patient no.
In summary, adult polyglucosan body disease should be suspected in patients with a late-onset progressive disorder of the peripheral and central nervous system, especially when upper motor neuron
signs, dementia, or bladder impairment is present.
ALS causes upper motor neurons
, originating from the top of the brain, and lower motor neurons, originating from the lower part of the brain and the spinal cord, to gradually disintegrate, preventing them from delivering chemical signals and essential nourishment that muscles depend on for normal function.
The definition of spasticity most often used notes that spasticity is a motor disorder, with increased excitability of the stretch reflex, leading to a velocity-dependent increase in tone as part of the upper motor neuron
Recent laboratory-based research performed at our institution has supported the hypothesis that women with complete spinal cord injuries and upper motor neuron
injuries affecting the sacral spinal segments will maintain the capacity for reflex lubrication while losing the capacity for psychogenic lubrication (Sipski and Alexander, 1995a).
Bulbar involvement can be lower motor neuron (Bulbar palsy), upper motor neuron
(Pseudobulbar palsy), or both.
Upper versus lower motor neuron weakness Upper motor neuron
weakness Lower motor neuron weakness Muscle weakness Muscle weakness Less atrophy of muscles (may be Atrophy of muscles disuse) Increased tone, spasticity Low muscle tone Increased deep tendon reflexes Diminished or absent deep tendon reflexes No fasciculation Fasciculation Hemiplegic, quadriplegic or Specific distribution of paraplegic distribution of weakness, i.e.
Idiopathic MND is sometimes used to describe all primary degenerative lower motor neuron and all upper motor neuron
disorders (Swash, 2000).
Subjects covered include: epidemiology, patholophysiology, and surgical management of SCI; clinical and functional evaluation; initial rehabilitation medicine consultation; bladder and bowel management; pressure ulcers; contracture management; autonomic dysfunction; upper motor neuron
syndrome; heterotropic ossificans; psychological adaptation; sexuality; aging; wheelchair mobility; environmental modifications; and community reintegration.
The simultaneous combination of upper motor neuron
and lower motor neuron dysfunction in clinical manifestation strongly suggests the clinical diagnosis of ALS.[sup] However, early in the course of ALS, the clinical manifestations may be atypical, and muscle weakness and atrophy may be limited to the small hand muscles of ALS patients.
On examination he had uniform wasting of right sided upper and lower limb muscles with right sided facial nerve palsy of upper motor neuron
type, power was 4/5 in both upper and lower limbs, he had slurring of speech, and his gait was circumductory.