title: Primary lateral sclerosis: predicting functional outcome.
abstract: Our objective was to investigate functional outcome in primary lateral sclerosis (PLS). We followed a group of 24 patients with PLS. Clinical (ALSFRS), respiratory, and neurophysiological (electromyography and transcranial magnetic stimulation) evaluations were performed at entry and regularly over the follow-up period. The time taken for a greater than 10% decrease in ALSFRS compared to the first assessment was defined as the dichotomous outcome; prognostic factors were evaluated using the Cox proportional hazard model. Results demonstrated that the median age at symptom onset was 54 years (range 28-75 years). In 46% symptoms began in the lower limbs, in 21% in the upper limbs and in 33% in bulbar muscles. The median symptom duration at first visit was 3.1 years (range 0.9-11.7 years). At last follow-up the median disease duration was 9.9 years (range 4.2-17.6 years). The median follow-up time was 4.6 years (range 2.1-11 years). We excluded from final analysis three patients with positive family history. Older age at onset (p = 0.019) was related to more rapid functional impairment; gender, forced vital capacity, region of onset and neurophysiological changes did not predict outcome. In conclusion, age is the most critical prognostic factor for functional outcome in PLS.
authors.affiliation: Department of Neurosciences, Hospital de Santa Maria-CHLN, Av. Prof. Egas Moniz, Lisbon, Portugal.
authors.forename: Vânia
authors.initials: V
authors.lastname: Almeida
authors.forename: Mamede
authors.initials: M
authors.lastname: de Carvalho
authors.forename: Manuel
authors.initials: M
authors.lastname: Scotto
authors.forename: Susana
authors.initials: S
authors.lastname: Pinto
authors.forename: Anabela
authors.initials: A
authors.lastname: Pinto
authors.forename: Benjamim
authors.initials: B
authors.lastname: Ohana
authors.forename: Michael
authors.initials: M
authors.lastname: Swash
mesh_terms: D000328:Adult; D017677:Age Distribution; D000368:Aged; D003661:Decision Support Techniques; D004568:Electrodiagnosis / Q000706:statistics & numerical data*; D005260:Female; D006801:Humans; D008297:Male; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000453:epidemiology*; D011174:Portugal / Q000453:epidemiology; D015995:Prevalence; D011379:Prognosis; D012129:Respiratory Function Tests / Q000706:statistics & numerical data*; D012307:Risk Factors