Finger extension weakness and downbeat nystagmus motor neuron disease syndrome: A novel motor neuron disorder?
Muscle Nerve
; 56(6): 1164-1168, 2017 Dec.
Article
in En
| MEDLINE
| ID: mdl-28440863
ABSTRACT
INTRODUCTION:
Disturbances of eye movements are infrequently encountered in motor neuron diseases (MNDs) or motor neuropathies, and there is no known syndrome that combines progressive muscle weakness with downbeat nystagmus.METHODS:
To describe the core clinical features of a syndrome of MND associated with downbeat nystagmus, clinical features were collected from 6 patients.RESULTS:
All patients had slowly progressive muscle weakness and wasting in combination with downbeat nystagmus, which was clinically most obvious in downward and lateral gaze. Onset was in the second to fourth decade with finger extension weakness, progressing to other distal and sometimes more proximal muscles. Visual complaints were not always present. Electrodiagnostic testing showed signs of regional motor axonal loss in all patients.DISCUSSION:
The etiology of this syndrome remains elusive. Because finger extension weakness and downbeat nystagmus are the discriminating clinical features of this MND, we propose the name FEWDON-MND syndrome. Muscle Nerve 56 1164-1168, 2017.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Nystagmus, Pathologic
/
Motor Neuron Disease
/
Muscle Weakness
Type of study:
Observational_studies
/
Risk_factors_studies
Limits:
Adolescent
/
Adult
/
Female
/
Humans
/
Male
Language:
En
Journal:
Muscle Nerve
Year:
2017
Document type:
Article
Affiliation country:
Belgium