Psychological factors in the diagnosis and pathogenesis of stiff-man syndrome

Abstract

Retrospective psychological evaluation of nine patients with stiff-man syndrome (SMS), seven of whom evidenced autoimmune disease, revealed a characteristic set of psychological symptoms or features: Major stressful life events preceded the development of permanent symptoms by 6 months or less (seven patients); transient motor symptoms occurred in emotionally distressing situations months or even years before the onset of a permanent motor deficit (five patients); after onset, similar situations specifically precipitated or augmented stiffness and spasms (five patients). We also found task-specific fear resembling agoraphobia (six patients) and loss or invalidation of one or both parents, or loss of home, in childhood (seven patients). Eight patients were initially misdiagnosed as having psychogenic movement disorder. We conclude that the common misdiagnosis of SMS as a psychogenic movement disorder is due to the compelling association of a set of salient psychological features, bizarre and fluctuating motor symptoms, and lack of approved neurologic signs.

A 47-Year Old Female with Muscular Rigidity, New-Onset Diabetes and Hypothyroidism

“…4 In some cases, individuals can identify a major stressful life event that preceded the onset of their psychological and neuromuscular symptoms. 5 The association between the exacerbation of the patient's symptoms and emotional stress often leads to psychiatric evaluation. Early in the course of the disease, a patient's neuromuscular symptoms may be non-specific, and the psychological features of anxiety and depression may dominate the clinical picture.…”

Stiff person syndrome (SPS): Literature review and case report

“…[41] Symptoms are frequently preceded by psychiatric conditions, months or even years before the onset of the neurological symptoms. [18] SPS initially presents as muscle stiffness, which is progressive and usually involves rigidity of the axial muscles, including the paraspinal thoracic, lumbar and proximal leg muscles. [33] Another debilitating feature is hyperlordosis, which can lead to difficulty rising from a chair or rising from a bed due to the simultaneous contractures of both the paraspinal and abdominal muscles.…”

“…[18] The prominence of psychiatric symptoms often leads to a misdiagnosis of conversion disorder. Bizarre and fluctuating movement disturbance and the presence of psychological factors contribute to the misdiagnosis.…”

“…Bizarre and fluctuating movement disturbance and the presence of psychological factors contribute to the misdiagnosis. [18] Patients with SPS are often seen by psychiatrists before they come to the attention of neurologists. [7] Various mechanisms have been proposed to explain the high rate of psychiatric comorbidity in SPS.…”

Stiff person syndrome (SPS): Literature review and case report

“…[14,17] More than 60% of patients diagnosed with SPS have at least one psychiatric diagnosis. [18] In 1998, Solimena et al [19] were the first to associate SPS with antibodies against the enzyme glutamic acid decarboxylase (GAD). GAD is an antigen found in 60 -80% of the sera of patients with SPS.…”