|
This is a form of motor neurone disease (MND). It is the most common form of
MND and accounts for 65% to 85% of all cases of MND (1). Amyotrophic lateral
sclerosis results from lesions to the corticospinal tract and the anterior horn
cells and produces the characteristic feature of tonic atrophy - brisk reflexes
and fasciculations. - a late onset, rapidly progressive and ultimately
fatal neurological disorder, caused by the loss of motor neurons in the brain
and spinal cord (2)
- familial aggregation of ALS, with an age-dependent
but high penetrance, is a major risk factor for ALS
- familial ALS (FALS)
is clinically and genetically heterogeneous
- three genes and linkage to
four additional gene loci have been identified so far and may either predominantly
lead to ALS (ALSI-ALS6) or cause multisystem neurodegeneration with ALS as an
occasional symptom (tauopathies, ALS-dementia complex)
This form of the disease includes Progressive Bulbar Palsy (1). Corticospinal
tract degeneration in the absence of significant muscle wasting may be referred
to as primary lateral sclerosis (PLS). - PLS entity is not universally
accepted. Debate continues over whether the disease constitutes a distinct clinical
and pathological entity or whether it is a part of the spectrum of MNDs that presents
an upper motor neuron (UMN) predominant form of MND (3)
- prospective analysis
of a series of PLS case reports was in agreement with data from other studies
suggesting that pure PLS cases have a prolonged course of disease with a high
level of independence when compared to other MND (3)
Reference:
|
