Abstract
Aim:To report a case of sudden onset vertical diplopia, blurred vision, and muscle spasms.Methods:This is a case report of a 57-year-old female who presented to the accident and emergency department with a one day history of vertical diplopia and a two week history of lower limb spasticity secondary to muscle spasms.Results:The patient had no significant medical or ocular history. Orthoptic investigation initially revealed a left inferior rectus (IR) underaction. Possible diagnoses at this point were thought to be isolated IR weakness, myasthenia gravis or skew deviation. An urgent MRI scan was arranged and blood tests were taken. MRI showed no abnormalities. Blood tests were normal, however, the acetylcholine receptor antibody serum test (ACH-R) was 0.43 nmol/L, which is at the high end of normal. At the follow-up visit, the IR weakness had deteriorated and the patient had also developed gaze-evoked nystagmus. An appointment with the neurologist and neuro-ophthalmologist was expedited. When the patient returned, she reported that her neurologist had diagnosed her with stiff-person syndrome (SPS). The patient had also developed a laterally alternating skew deviation and reported that she had undergone a course of intravenous immunoglobulin (IVIG). The patient was prescribed diazepam and gabapentin. Due to the lack of recovery, persistent diplopia and oscillopsia, monthly IVIG have been prescribed.Conclusion:There is currently a paucity of literature relating to ophthalmic problems with SPS and how they are best treated. Previous reports have established that there is a link with myasthenia gravis, with many patients going on to develop myasthenia. Treatment of SPS is lacking large evidence-based studies. However, treatment with muscle relaxants and anticonvulsants has provided a good outcome for some. Further research is required to develop an evidence-based approach to treating the ophthalmological problems patients with SPS experience. This case report highlights the importance and value of orthoptists in investigating and monitoring patients with stiff-person syndrome.
Highlights
Stiff-person syndrome (SPS) is a rare neurological disorder which affects the nervous system and affects less than one million people worldwide (Brown & Marsden 1999; Hadavi et al 2011; Lorish, Thorsteinsson & Howard 1989)
stiff-person syndrome (SPS) is rather unique as it lacks significant similarity to any other neurological disorder, it has the features of an autoimmune disorder as the majority of patients have glutamic acid decarboxylase (GAD) antibodies (Brown & Marsden 1999; Hadavi et al 2011; Lorish, Thorsteinsson & Howard 1989)
We describe a patient who presented with complaints of muscles spasms and vertical diplopia that was subsequently found to have SPS
Summary
Stiff-person syndrome (SPS) is a rare neurological disorder which affects the nervous system and affects less than one million people worldwide (Brown & Marsden 1999; Hadavi et al 2011; Lorish, Thorsteinsson & Howard 1989). SPS is rather unique as it lacks significant similarity to any other neurological disorder, it has the features of an autoimmune disorder as the majority of patients have glutamic acid decarboxylase (GAD) antibodies (Brown & Marsden 1999; Hadavi et al 2011; Lorish, Thorsteinsson & Howard 1989). The cause of SPS is relatively unclear (Dalakas 2008, Hadavi et al 2011; Lorish, Thorsteinsson & Howard 1989). SPS is characterised by progressive rigidity and stiffness, accompanied by muscle spasms, commonly affecting the trunk and/or limbs
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