Progressive encephalomyelitis with rigidity presenting as a stiff-person syndrome

• Published in: Parkinsonism & related disorders Vol. 8; no. 4; pp. 285 - 288
• Main Authors: Gouider-Khouja, Neziha, Mekaouar, Aouatef, Larnaout, Abdelmajid, Miladi, Najoua, Khelifa, Fethi Ben, Hentati, Fayçal
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01-03-2002
• Subjects: Anti-Inflammatory Agents - therapeutic use; Baclofen - therapeutic use; Corticosteroids; Diagnosis, Differential; Diazepam - therapeutic use; Drug Therapy, Combination; Electromyography; Encephalomyelitis - complications; Encephalomyelitis - diagnosis; Encephalomyelitis - drug therapy; Evoked Potentials, Somatosensory; Female; Humans; Methylprednisolone - therapeutic use; Middle Aged; Muscle Relaxants, Central - therapeutic use; Muscle Rigidity - diagnosis; Muscle Rigidity - drug therapy; Muscle Rigidity - etiology; Muscle Rigidity - pathology; Muscle Rigidity - physiopathology; Progressive encephalomyelitis; Segmental rigidity; Stiff-person syndrome; Stiff-Person Syndrome - diagnosis; Thyroiditis; Thyroiditis; Corticosteroids; Segmental rigidity; Progressive encephalomyelitis; Stiff-person syndrome
• ISSN: 1353-8020; 1873-5126
• DOI: 10.1016/S1353-8020(01)00047-5

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.