Case report
Progressive encephalomyelitis with rigidity presenting as a stiff-person syndrome

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Abstract

Diagnosis criteria of stiff-person syndrome (SPS) include progressive, fluctuating muscular rigidity and spasms with normal neurological examination. The presence of unusual features such as prominent limb rigidity with segmental signs and contracture, evidence of brainstem dysfunction, profound autonomic disturbances, CSF pleiocytosis or MRI abnormalities in patients with SPS presentation allows to classify these patients as progressive encephalomyelitis with rigidity (PER). We report a 50 year-old woman suffering from severe painful spasms of abdominal wall and limb muscles. Neurological examination showed pyramidal signs. EMG disclosed continuous muscle activity with superimposed discharges. Treatment with high doses of diazepam and baclofen led to moderate improvement of generalised stiffness. However, the right arm became more rigid with oedema and vasomotor changes. Subsequently, bilateral nystagmus and internuclear opthalmplegia appeared. There was mild CSF pleiocytosis. Associated auto-immune thyroiditis was found with positive anti-microsome antibodies and decreased thyroid hormones. Search for profound neoplasm was negative. The patient had three subacute bouts then she improved with methylprednisolone.

The initial clinical presentation mimicking a SPS with subsequent diffuse involvement of the central nervous system and a striking localisation of a severe rigidity to one arm allowed to suspect the diagnosis of PER. The relationship between SPS and PER remains unclear because of the rarity of these disorders. The observation reported in this paper gives evidence that both the disorders are probably two clinical presentations of the same pathogenic process.

Introduction

Stiff-person syndrome (SPS) first described by Moersch and Woltman [1] is a rare acquired disorder of adult life characterised by progressive and fluctuating muscle rigidity and spasms. In 60% of cases, it is associated to antibodies against glutamic acid decarboxylase (GAD) and can sometimes be paraneoplastic [2]. In some patients with SPS presentation, atypical clinical signs (such as prominent limb rigidity with segmental signs and contracture, evidence of brainstem dysfunction, profound autonomic disturbances, CSF pleiocytosis and MRI abnormalities) were reported suggesting progressive encephalomyelitis with rigidity (PER) [3], [4]. Because of the rarity of the two disorders the relationship between SPS and PER remains unclear. We report the history of a patient with PER and discuss the link of this entity to SPS.

Section snippets

Case report

A 50 year-old woman had hypertension and dyslipidemia for three years. On April 21, 1996, she suddenly developed painful spasms and rigidity of the abdominal wall muscles. Spasms extended to the lower limbs within three days. On examination, five days after onset, there was a generalised stiffness sparing the face, and superimposed contractures aggravated by voluntary movements and external stimuli such as noise or touch. The patient was unable to walk unaided and to bend her neck forward; she

Discussion

The clinical features of the patient reported here are characterised by acute onset of limb and axial rigidity and spasms without hyperlordosis. Three subacute bouts occurred with aggravation of rigidity and more diffuse involvement of central nervous system (pyramidal, vestibular, oculomotor signs). The bouts were followed by a spontaneous slow recovery. A dramatic improvement was obtained during the last attack of the disease with high doses of methylprednisolone. In this patient, two

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