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The stiff leg syndrome

P Brown¹, J C Rothwell, C D Marsden

¹MRC Human Movement and Balance Unit, Institute of Neurology, London, UK.

Journal of Neurology, Neurosurgery, and Psychiatry

|January 1, 1997

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This study describes a distinct neurological disorder characterized by chronic, painful leg spasms and stiffness, separate from stiff man syndrome. Investigations suggest a potential cause of chronic spinal interneuronitis.

Area of Science:

Neurology
Neuroscience
Clinical Electrophysiology

Background:

A chronic progressive neurological disorder affecting middle-aged individuals presents with lower limb stiffness and painful spasms.
Symptoms include spontaneous, reflex, and movement-induced spasms, rigidity, and abnormal leg postures without truncal involvement.
Patients exhibit prolonged symptoms (up to 16 years) with absence of brainstem, pyramidal, or sensory dysfunction, and late-onset sphincter disturbance in one case.

Purpose of the Study:

To characterize a distinct neurological syndrome presenting with chronic progressive lower limb stiffness and spasms.
To differentiate this syndrome from known conditions like stiff man syndrome and encephalomyelitis with rigidity.
To investigate the underlying pathophysiology and potential causes of the observed symptoms.

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Main Methods:

Clinical assessment of four patients with chronic progressive lower limb disorder.
Comprehensive neuroaxis imaging and laboratory investigations, including anti-GAD antibody testing.
Electrophysiological studies to analyze motor unit activity, spasm patterns, reflexes, and long tract function.

Main Results:

Patients displayed continuous motor unit activity at rest, repetitive grouped discharge during spasms, abnormal cutaneomuscular reflexes, and minimal long tract disturbance.
No cause was identified through extensive investigations, and anti-GAD antibodies were absent.
Baclofen and diazepam provided only partial relief from spasms, with patients remaining significantly disabled.

Conclusions:

The findings suggest a characteristic syndrome distinct from stiff man syndrome and encephalomyelitis with rigidity.
The syndrome is hypothesized to result from chronic spinal interneuronitis.
Further research is needed to elucidate the precise etiology and optimal management of this condition.