Ocular myasthenia gravis in a person with tetraplegia presenting challenges in diagnosis and management.

We report the first case of ocular myasthenia gravis (OMG) in a patient with complete tetraplegia, highlighting diagnostic and management challenges. Spinal multidisciplinary rural clinic and specialised inpatient Spinal Cord Injury Unit, NSW, Australia. A 61-year-old man with established C5 AIS A tetraplegia, presented with sudden onset of diplopia and bilateral ptosis, later diagnosed as OMG, in context of other complex co-morbidities, including a cervical cord syrinx, obstructive sleep apnoea and labile blood pressure. Clinical findings were consistent with fluctuating bilateral partial third and sixth nerve palsies. Acetylcholine receptor antibodies were negative, but electromyography demonstrated muscle fatigue. The ocular signs responded well to pyridostigmine. Medications taken before diagnosis, including solifenacin for neurogenic bladder overactivity, were ceased to avoid attenuating the anti-cholinesterase effect. However, the unopposed anti-cholinesterase activity led to frequent and painful abdominal spasms, associated with uncontrolled detrusor hyperreflexia and worsening autonomic dysreflexia (AD). A trans-vesical phenol block to treat this provided only short-lasting benefit. Pyridostigmine was ceased to avoid provoking his abdominal spasms and his regular medications were recommenced. It was decided that the most appropriate treatment for his distressing diplopia was an eye patch. After discharge home, he continued to experience problems with recurrent urinary tract infections, abdominal spasms, episodic postural hypotension and AD. After 5 months, the patient died from an acute myocardial infarction. This case report contributes new knowledge about the rare presentation of OMG in a person with chronic tetraplegia.

Issues and challenges for development of a sustainable service model for people with spinal cord injury living in rural regions.

OBJECTIVE: To develop and implement a service model for people with spinal cord injury (SCI) living in rural regions. DESIGN: Service development, pilot evaluation study. SETTING: Regional and remote areas of the state of New South Wales, Australia. PARTICIPANTS: Persons with SCI, caregivers, and health professionals. INTERVENTION: Phase 1 included initial needs analysis, followed by education and resource development tailored to needs of rural health professionals, caregivers, and persons with SCI. Phase 2 included coordination, professional support, and network development by part-time rural key worker and metropolitan-based project officer, documenting health- and service-related issues. MAIN OUTCOME MEASURES: Self-perception of confidence as a result of education as well as reported issues, adverse health events, and barriers to service provision. RESULTS: Clinician confidence in managing people with SCI improved after education. Various health-related, environmental, and psychosocial issues were reported. Limited availability of resources and health infrastructure, particularly in more isolated or smaller towns, challenged service provision. Rural key workers played a central role in supporting local clinicians and service providers, improving communication and service coordination between rural health professionals and metropolitan SCI services. CONCLUSION: Education and support for rural workforce that may be limited in numbers and capacity, and a model facilitating communication and coordination between services, are essential for improving health outcomes of rural people with SCI.

A longitudinal study of the prevalence and characteristics of pain in the first 5 years following spinal cord injury.

A longitudinal cohort study of 100 people with traumatic spinal cord injury (SCI) was performed to determine the prevalence and severity of different types of pain (musculoskeletal, visceral, neuropathic at-level, neuropathic below-level) at 5 years following SCI. Prospective data on the characteristics of pain up to 6 months following injury had been collected previously and allowed comparisons between the presence of pain at different time points. In addition, we sought to determine the relationship between the presence of pain and physical factors related to the injury such as level of lesion, completeness and clinical SCI syndrome. We also obtained information regarding mood, global self-rated health and the impact of pain on function. Of the 100 subjects in the original cohort, 73 were available for follow up. When all types of pain were included, 59 of the 73 subjects (81%) reported the presence of pain. Musculoskeletal pain was the most common type of pain experienced and was present in 43 subjects (59%), at-level neuropathic pain was present in 30 subjects (41%), below-level neuropathic pain was present in 25 subjects (34%) and visceral pain was present in four subjects (5%). Overall, 58% reported their pain as severe or excruciating and those with visceral pain were most likely to rate their pain in these categories. There was no relationship between the presence of pain overall and level or completeness of lesion, or type of injury. However, tetraplegics were more likely to report below-level neuropathic pain. This study prospectively demonstrates the differing time courses of different types of pain over the first 5 years following SCI. There was a strong correlation between the presence of both types of neuropathic pain at 5 years and earlier time points but both visceral pain and musculoskeletal pain demonstrated a poor correlation between time points. Chronic visceral pain occurs in a small percentage of patients and does not correlate with the presence of visceral pain early following injury. Those with neuropathic pain early following their injury are likely to continue to experience ongoing pain and the pain is likely to be severe. In contrast, chronic musculoskeletal pain is more common but less likely to be severe and cannot be predicted by the presence of pain in the first 6 months following injury.

Pain characteristics in patients admitted to hospital with complications after spinal cord injury.

OBJECTIVES: To determine characteristics of pain, the relation between pain and mood, the effect of pain on activities, and the perceived difficulty in coping with pain in patients hospitalized for treatment of complications associated with spinal cord injury (SCI). DESIGN: Cohort survey. SETTING: Hospital inpatient unit in Australia. PARTICIPANTS: Consecutive sample of patients (N=88) admitted to a hospital spinal injuries unit with complications after SCI. Two eligible patients declined to participate. INTERVENTION: Face-to-face interview with questionnaire. MAIN OUTCOME MEASURES: Pain severity, global self-rated health, mood (Kessler Mood Inventory), and interference with activities (Von Korff disability scale). RESULTS: Sixty-six (75%) of the 88 subjects experienced pain, with an average time of onset +/- standard deviation of 8.02+/-12.4 years; 27% of those with pain described it as severe or excruciating. Subjects with pain were less likely to rate their global health as excellent or very good when compared with those who did not have pain (22% vs 44%, respectively). Patients with pain had significantly greater levels of psychologic distress than did people with SCI and no pain. CONCLUSIONS: Pain is a common problem in people admitted to hospital with SCI for treatment of other complications. It has a significant impact on activities and is associated with a reduction in global self-rated health and higher levels of psychologic distress.