title: Differential diagnosis in progressive infantile spastic tetraparesis.
abstract: Progressive infantile spastic tetraparesis spans a wide spectrum of partially rare differential diagnoses. Based on a clinical example the differential diagnostic thoughts are discussed in detail. Though juvenile motor neuron disease is a rare entity, it has to be kept in mind for differential diagnostics in cases of slowly progressive spastic tetraparesis, especially when a pseudobulbar palsy or distal amyotrophies add to the clinical picture. Electromyography can be helpful for early detection of lower motor neuron involvement. The glutamate antagonist riluzole slows the disease progression, but a causal treatment is not available, yet. Therefore symptomatic treatment of disturbing symptoms like muscle cramps, spasticity, pseudobulbar affect, dyspnea or dysphagia are of major interest.
authors.affiliation: University of Ulm, Deparment of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany. peter.kuehnlein@uni-ulm.de
authors.forename: Peter
authors.initials: P
authors.lastname: Kühnlein
authors.forename: A-D
authors.initials: AD
authors.lastname: Sperfeld
authors.forename: H-J
authors.initials: HJ
authors.lastname: Gdynia
mesh_terms: D000328:Adult; D017668:Age of Onset; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D003937:Diagnosis, Differential; D004576:Electromyography; D020662:Guanine Nucleotide Exchange Factors / Q000235:genetics; D006801:Humans; D008297:Male; D011782:Quadriplegia / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology
chemical_list: C438187:ALS2 protein, human; D020662:Guanine Nucleotide Exchange Factors