/facial onset sensory and motor neuronopathy

facial onset sensory and motor neuronopathy

Rare Disease

MONDO:0019405

Also Known As

FOSMN syndromefacial onset sensorimotor neuronopathy syndromefacial onset sensory and motor neuronopathy syndrome

Definition

Facial onset sensory and motor neuronopathy is characterized initially by paraesthesia and numbness in the region of the trigeminal nerve distribution, which later progresses to involve the scalp, neck, upper trunk and upper limbs. Onset of motor manifestations occurs later with cramps, fasciculations, dysphagia, dysarthria, muscle weakness and atrophy. This syndrome has been described in four males and appears to be a slowly progressive neurodegenerative disease.