Hereditary Motor Syndromes with Multisystem Involvement

http://neuromuscular.wustl.edu/synmot.html

Hexosaminidase A (Late onset GM2 Gangliosidosis - Tay Sach's disease)
Motor neuropathy +/- CNS (LMN dominant)
Cramps, paresthesias at onset
Symmetric proximal > distal weakness
Fasciculations
+/- CNS involvement - spasticity, ataxia, dementia, dystonia

Adult onset Polyglucosan body disease
CNS + Neuropathy
Dementia in 2/3rd
Cord - spasticity
Nerve - Motor and sensory