Walking ability in patients with diastrophic dysplasia: a clinical, electroneurophysiological, treadmill, and MRI analysis.
Patients with diastrophic dysplasia have walking difficulties of obscure etiology; some are even wheelchair-bound. To explore the problem, physical examination, treadmill, magnetic resonance imaging (MRI), and electroneurophysiologic studies were performed on 87 patients (56 females, 31 males) with an average age of 31 (range 3-56) years. Mobility of the spine, hips, knees, and feet was diminished. Some of the patients were obese (mean body mass index 27.0 kg/m). In the treadmill study, patients were able to walk an average of 638 m (range 0-1,618 m). On MRI, five patients showed compression of neural structures; one of them also had clinical symptoms. Somatosensory evoked potentials and electroneuromyography revealed evidence of compression of neural structure in three (3%) and two (3%) patients, respectively. The walking difficulties seem to have a multifactorial etiology: flexion contractures of the knees, early and rapid osteoarthrosis, equinus or equinovarus foot deformities, and obesity, but only rarely spinal stenosis.
Remes
Ville
V
Hospital for Children and Adolescents, Helsinki University Central Hospital, Helsinki, Finland. ville.remes@helsinki.fi
Poussa
Mikko
M
Lönnqvist
Tuula
T
Puusa
Asko
A
Tervahartiala
Pekka
P
Helenius
Ilkka
I
Peltonen
Jari
J
D000293:Adolescent; D000328:Adult; D001848:Bone Diseases, Developmental / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004558:Electric Stimulation; D004576:Electromyography; D005080:Exercise Test; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016138:Walking / Q000502:physiology*