Functional Impairments in a Patient with Morvan Syndrome: A Case Presentation.

A 48-year-old man with lung squamous cell carcinoma was admitted to acute care with cognitive impairment after recent chemotherapy. He developed myoclonus, ataxia, agitation, and visual hallucinations. Morvan syndrome, a rare voltage-gated potassium channel antibody disorder characterized by neuromyotonia with central nervous system dysfunction, was eventually diagnosed. He received plasmapheresis and was admitted to inpatient rehabilitation, where he safely participated in therapies. By focusing on neuromuscular rehabilitation, balance training, fine motor skills, and cognitive retraining emphasizing skills relevant to the patient's premorbid cognitive activities, the patient demonstrated significant functional improvement, decreasing the burden of care of his caregivers. LEVEL OF EVIDENCE: V.

Are Established Methods of Physiotherapeutic Management for Long-term Neurological Conditions Applicable to 'Orphan' Conditions such as Syringomyelia?

BACKGROUND AND PURPOSE: Syringomyelia is a rare or 'orphan' condition with the potential to cause significant disability and detrimental effects to quality of life. Syringomyelia shares similar symptoms to those common in other long-term neurological conditions, including spinal cord injury and multiple sclerosis. In these more prevalent conditions, physiotherapy is utilized widely and is effective in optimizing physical, psychological and social parameters. Therefore, we theorized that physiotherapy might be transferable to, and beneficial to syringomyelia patients. As a paucity of literature exists in this area, we aimed to evaluate the existing uptake and perceived efficacy of physiotherapy. METHODS: An exploratory, mixed methodology was selected to derive sufficient qualitative data for analysis. Specifically designed questionnaires and semi-structured interviews yielded data on uptake and perceived physiotherapy efficacy. One hundred patients from a National Health Service tertiary syringomyelia service were invited to participate. RESULTS: The questionnaire and interviews were completed by 49 and 20 patients, respectively. Of the small number of patients receiving physiotherapy, the majority reported beneficial effects on pain modulation and quality of life. Stretching and hydrotherapy were deemed effective for relief of pain and stiffness. Additionally, physiotherapy was reported to provide similar benefits to surgical intervention. DISCUSSION: Syringomyelia patients report physiotherapy to provide benefits for symptom management and quality of life. Such findings suggest that established rehabilitation techniques in more common conditions may be transferable to those less prevalent. Uptake of physiotherapy was limited, seemingly because of inadequate information, knowledge and resources. To address these deficiencies, further studies should be planned investigating the effectiveness of physiotherapy modalities, such as hydrotherapy, in parallel or in conjunction with surgery and/or pharmacology. Additionally, syringomyelia could be represented alongside similar pathologies in research trials to initiate further research questions and drivers for funding.

Conservative treatment of a patient with syringomyelia using chiropractic biophysics protocols.

OBJECTIVE: To present a case of a 41-year-old man with syringomyelia and intractable pain and the subsequent reduction of symptoms. CLINICAL FEATURES: This patient acquired a traumatically induced syrinx in his upper cervical spinal cord after he fell approximately 9 feet and landed on his head, upper back, and neck 9 years before presenting for care. He was diagnosed with a spinal cord cyst (syrinx), located at approximately C2 through C4 after magnetic resonance imaging. In 1995, the patient underwent occipitoatlantal decompression surgery, which improved his symptoms for a short time. INTERVENTION AND OUTCOMES: The patient was treated using Clinical Biomechanics of Posture protocol. The patient was seen 26 times over the course of 3 weeks. His scale for pain severity decreased 50% and other subjective complaints decreased. His posture improved based upon pretreatment and posttreatment lateral cervical radiographs, showing a change from a 10 degrees lordosis with midcervical kyphosis to a 30 degrees lordosis. One-year follow-up examination showed stable improvement in the cervical lordosis and pain intensity. CONCLUSION: This case represents a change in subjective and objective measurements after conservative chiropractic care. This case provides an example that structural rehabilitation may have a positive effect on symptoms of a patient with syringomyelia.

The treatment dilemma in post-traumatic syringomyelia.

PURPOSE: The aim of the present study was to compare the functional effect of conservative and surgical treatment in post-traumatic syringomyelia. METHOD: The files of 10 male patients treated for posttraumatic syringomyelia were retrospectively reviewed from 1986 to 1996. RESULTS: The spinal lesion was complete in five patients and incomplete in five. All patients underwent rehabilitation, five of them following surgery. The operative procedures included drainage by syringosubarachnoid shunting (four patients) and decompressive laminectomy (one patient). Rehabilitation alone improved the functional status in all five patients so treated. After surgery, function deteriorated in four of the five operated patients, and rehabilitation failed to restore the preoperative functional status in any of them. CONCLUSION: In view of the results it is suggested that patients with post-traumatic syringomyelia undergo rehabilitation with very close clinical and magnetic resonance imaging follow-up.

Intrathecal baclofen administration for control of severe spinal spasticity: functional improvement and long-term follow-up.

OBJECTIVES: To assess long-term efficacy and functional benefits of intrathecal baclofen for severe spinal spasticity. DESIGN: A prospective before-after trial. SETTING: A neurological rehabilitation department of a university hospital. Pump implantation was realized in neurosurgery; follow-up was carried out mostly on an outpatient basis. PATIENTS: Eighteen patients with severe and disabling spinal spasticity received intrathecal baclofen by an implantable pump; average follow-up was 37.4 months (range, 9 to 72). MAIN OUTCOME MEASURES: Spasticity (Ashworth and spasms frequency scores); disability (Functional Independence Measure [FIM]). RESULTS: A significant decrease in tone and spasms was observed in all patients. Tolerance appeared during the first 6 to 9 months. Later on, efficacy remained stable, except in cases of mechanical problems of the pump or catheter. Functional assessment found a highly significant (p < .001) increase of FIM score (particularly for bathing, dressing lower body, transfers, and in some cases, locomotion). This was particularly marked in patients with thoracic spinal cord lesion. In cases of severe upper limb dysfunction, FIM was only improved for wheelchair displacements, due to a better sitting position, but nursing became easier and life comfort was enhanced. Severe side effects (overdose) were observed in two cases. CONCLUSION: Efficacy remained stable after 6 to 9 months. Marked improvement of functional independence was observed in paraplegic patients. Improvement was less spectacular in patients with severe upper limb dysfunction, but nevertheless appreciable in terms of life comfort and use of attendants.

Post-traumatic syringomyelia: a review of the literature.

The need for increased awareness and a high index of suspicion for post traumatic syringomyelia is emphasised. Early clinical diagnosis confirmed by MRI and early treatment can avert or minimise the potentially devastating effects of post traumatic syringomyelia. The regular and frequent follow up of the patient on a yearly or alternate year basis to monitor the patient with spinal injury for this complication, as well as other complications, is the best way to ensure that post traumatic syringomyelia is diagnosed and managed early in order to avoid further disability.

Rehabilitative neurosurgery.

The neurosurgical role in rehabilitation rehabilitation was studied. Over a 5-year period, 850 individuals were referred to a rehabilitation center after initial acute care. Surgery was indicated for 66 patients. There were 28 quadriplegic or paraplegic individuals with intractable spasticity. Percutaneous radiofrequency foramenal rhizotomies were found to be 98% effective in relieving posttraumatic spasticity. In 14 patients with cognitive impairment, intellectual improvement had reached a plateau level. These persons underwent computed tomography scanning and cisternography, revealing significant communicating hydrocephalus. After surgical shunt therapy, cognitive improvement was noted in 86%. Nineteen individuals were sent for rehabilitation following spine fracture or progressive quadriplegia; 17 were found to have persistent spinal instability requiring surgical stabilization by fusion. This was successful in all cases without complications. Two persons required decompressive spinal operations, resulting in neurological stabilization or improvement. Five patients developed pain, spasticity, ascending neurological deficit, or autonomic dysreflexia due to posttraumatic syrinx. These symptoms were stabilized or improved following syringosubarachnoid shunting. The authors submit that comprehensive neurosurgical reevaluation is desirable in patients received for rehabilitation. Periodic neurosurgical follow-up is recommended. The neurosurgeon's role is not limited to the acute process.