title: Patrikios syndrome in two patients with treatable flail-leg weakness.
abstract: Flail-leg syndrome or lower limb diplegia is a form of motor neuron disease characterized by a slower progression rate. The differential diagnosis with motor neuropathy is important. We present two patients with a previous diagnosis of amyotrophic lateral sclerosis (ALS)-flail-leg syndrome, in whom neurophysiological studies suggested proximal conduction block. Both patients responded to immunomodulatory therapy, which suggested an immunologically mediated, treatable flail-leg syndrome phenotype. We stress the importance of fasciculations in the diagnosis of ALS, and the study of nerve root conduction in the differential diagnosis.
authors.affiliation: Department of Neurosciences, Hospital de Santa Maria, Avenue Prof. Egas Moniz, Lisbon 1649-028, Portugal.
authors.forename: Vânia
authors.initials: V
authors.lastname: Almeida
authors.forename: Benjamim
authors.initials: B
authors.lastname: Ohana
authors.forename: Mamede
authors.initials: M
authors.lastname: de Carvalho
authors.forename: Michael
authors.initials: M
authors.lastname: Swash
mesh_terms: D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D003937:Diagnosis, Differential; D005207:Fasciculation / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009123:Muscle Hypotonia / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D018908:Muscle Weakness / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D013577:Syndrome; D016896:Treatment Outcome