Case reportProgressive encephalomyelitis with rigidity presenting as a stiff-person syndrome
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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