Abstract Details

Just another case of lower back pain – or is it?

Stiff person syndrome (SPS) is an exceptionally rare autoimmune condition and in a minority of cases a paraneoplastic phenomenon. It typically presents with episodic spasms and stiffness affecting commonly  the trunk (paraspinal muscles) and or lower limbs. Over time all four limbs may become affected.

Eight cases of sero-positive SPS with average age of 51.4 years (range: 34.9 – 74.7) have been diagnosed in Sheffield since 1994. All cases were sent for NCS/EMG. In the 1990’s, trans-magnetic stimulation and EMG were the techniques used to investigate for SPS, whilst in the past 10 years utilisation of the blink reflex to assess brainstem hyper-excitability have been adopted. The neurophysiological findings that are supportive of SPS include continuous motor unit activity on EMG and abnormal blink reflex excitability examination on paired electrical stimulation at short inter-stimulus intervals (i.e. 160ms between a conditioning and a test stimulus). The initial studies were supportive (continuous motor unit activity on EMG) in four cases; a repeat study was supportive in one further case, whilst in two patients the only abnormality detected was on blink reflex excitability studies.  In one patient, all neurophysiology remained negative.

The rarity of the disease alongside the frequent negative serology and neurophysiology often causes a significant delay in diagnosis. In this cohort of patients the mean delay in diagnosis was 4.4 years (range: 0.6 – 11.9). Given the morbidity associated with a delayed diagnosis the principal aim of this talk is to discuss the value of Neurophysiological testing in the diagnosis of SPS

TitleForenamesSurnameInstitutionLead AuthorPresenter
DrMartha HanbyDepartment of Clinical Neurology and Neurophysiology, Royal Hallamshire Hospital, Sheffield, S10 2JF
Dr AlexanderBarkerDepartment of Clinical Neurology and Neurophysiology, Royal Hallamshire Hospital, Sheffield, S10 2JF
Dr PtolemySarrigiannisDepartment of Clinical Neurology and Neurophysiology, Royal Hallamshire Hospital, Sheffield, S10 2JF
ProfessorMariosHadjivassiliouDepartment of Clinical Neurology and Neurophysiology, Royal Hallamshire Hospital, Sheffield, S10 2JF
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