Population rCharacteristics and Progressive Disability in Neuofibromatosis Type 2.

Related Articles

Population rCharacteristics and Progressive Disability in Neuofibromatosis Type 2.

World Neurosurg. 2017 Jul 15;:

Authors: Iwatate K, Yokoo T, Iwatate E, Ichikawa M, Sato T, Fujii M, Sakuma J, Saito K

Abstract
OBJECTIVE: To characterize the clinical features of the NF2 population and determine prognostic risk factors for progressive disabilities.
METHODS: In this retrospective cohort study of the Japanese national NF2 registry between 2009-2013, various clinical data (demographic, history, oncological, and neurological) of 807 patients with diagnosis of NF2 were analyzed. The overall severity of neurological disability was assessed using a comprehensive 25-point scoring system encompassing a wide variety of neurological deficits. In 587 patients in whom longitudinal disability data were available, multivariate logistic regression was performed to identify risk factors of significant disability progression.
RESULTS: The clinical characteristics of the Japanese NF2 population were heterogeneous Median onset age [range] of 24 years [1-80]; male-to-female ratio 1:1.29; initial severity score 4 [0-22] out of 25-points. Family history was present in 33%. Most frequent clinical features were bilateral cranial nerve VIII nerve sheath tumor (CN8 NST, 87%), spinal NST (80%), hearing loss (65%), spinal dysfunction (50%), intracranial meningioma (49%), and facial paresis (36%). Disability score progressed by ≥5-points in 6.1% of patients over the study period. Based on multivariate logistic regression analyses, the significant independent risk factors of progression (p-value) included: onset age <25 years (p=0.015), positive family history (p=0.007), positive treatment history (p=0.026) hearing loss (p=0.014), facial paresis (p=0.015), blindness (p=0.011), hemiparesis (p=0.025).
CONCLUSIONS: The NF2 population in Japan is heterogeneous in clinical features. Risk factors of progressive disability include younger age of onset, positive family history, positive treatment history, and specific neurological deficits.

PMID: 28720529 [PubMed - as supplied by publisher]

from #Audiology via xlomafota13 on Inoreader http://ift.tt/2thUrAy
via IFTTT