Objectives: This article reviews literature examining the psychological adjustment to Spinal Cord Injury (SCI) and illustrates the applicability of a Stress Appraisal and Coping model (SAC) for rehabilitation of this population. Method: Articles concerning psychological adjustment to SCI from the previous three decades have been reviewed and critiqued. When possible the articles have been discussed in a SAC framework. Results: The literature indicates that psychological adjustment to SCI is largely predictable from psychological variables including coping, appraisal and psychosocial resources. Recent literature has suggested psychological intervention can promote positive psychological adjustment following SCI for those individuals at risk of developing clinical levels of depression. Conclusion:The SAC model provides a comprehensive formulation to incorporate the heterogeneity of populations with SCI. Suggestions for future research include developing assessment and treatment regimes speci®cally tailored to the strengths and weaknesses of an individual as highlighted in the model. Spinal Cord (2001) 39, 615 ± 627

Background/Aims: Since the polio epidemic in Ireland in the 1950s, most polio survivors are approaching into the 6th and 7th decade of their lives. There is little data about bone density and risk of fractures in these patients. In 2006, we undertook an audit of post-polio patients attending rheumatology and neurology outpatient clinics in a university teaching hospital. Our aim was to determine the prevalence of osteoporosis (OP), falls and fractures and to evaluate the association of bone density with other potential contributing factors to OP. Methods: Over a 6-month period, 50 post-polio patients attending outpatient clinics completed a questionnaire, and subsequently their medical records were reviewed. Demographic data and details of treatment were extracted. The patients underwent a dual-energy X-ray absorptiometry scanning to quantify bone mineral density. Results: Thirty subjects (60%) were females (26 were postmenopausal). The average age of females was 60 ± 13.4 years and of men 59 ± 16.8 years. Overall, 41 (82%) of the patients had experienced falls in the last 5 years and 32 (64%) in the last 6 months. Nineteen (38%) of the patients had experienced a bone fracture in the last 5 years. Based on the bone mineral density data, 28 (56%) of the patients were diagnosed with OP and 20 (40%) had osteopenia, but only 8 (16%) received anti-resorptive therapy. Of the 19 patients who had a fracture, 14 (74%) had OP and 5 (26%) had osteopenia, of whom only 6 (32%) received anti-resorptive therapy. Eight out of 9 fractures of the neck of femur occurred in the weaker leg. Conclusions: Post-polio patients are a high-risk group for fracture, and thus bone density assessment, review of falls risk and therapeutic intervention should be considered for all patients. Both osteopenia and OP are associated with increased fracture risk.

4D CT has higher sensitivity than scintigraphy. Missed lesions are more likely to occur with multigland disease for both modalities and in smaller lesions for scintigraphy.

Pelizaeus-Merzbacher Disease (PMD) is a rare X-linked recessive leukodystrophy caused by mutations in the proteolipid protein 1 gene on the Xq22 chromosome. PMD is a dysmyelinating disorder characterized by variable clinical presentation and course. Symptoms range from mild motor deficits to progressive spasticity and neurologic decline resulting in death at an early age. There is no definitive curative treatment. This report presents the clinical course of 2 young boys with PMD who are the first known patients to receive umbilical cord blood transplantation as a therapeutic intervention to stabilize disease progression. Pretransplantation evaluation revealed that both patients had significant motor deficits as well as delayed cognitive function as compared with age-matched peers. Brain imaging revealed varying degrees of hypomyelination. Both patients received myeloablative chemotherapy followed by an unrelated donor umbilical cord blood infusion, which they tolerated well with no major transplantation-related complications. At 7-years and 1-year posttransplantation, respectively, both boys are making slow neurocognitive improvements and show no evidence of functional decline. Imaging results show stable or improving myelination. Although the results of unrelated donor umbilical cord blood transplantation in these 2 boys with PMD are encouraging, longerterm follow-up will be necessary to assess the effect of this treatment on the variable natural disease course.

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Figure 1: A) CT Brain of LM showing a left fronto-temporal ICH (arrow). B) CT Brain of LM with bilateral occipital gyral calcifications (arrows)from the department of neurology (tM, aJC, SB, lC, JtM), department of neuroradiology (lg), department of neuropathology (Maf, fMB), Beaumont hospital and royal College of Surgeons ireland, dublin, ireland; Section of neuropathology, department of neurology (hVV), uCla Medical Center, los angeles, California, uSa.

DESCRIPTIONA 64-year-old woman presented with progressive hearing loss. A pure-tone audiography confirmed bilateral high-frequency sensorineural hearing loss. Her medical and surgical history was unremarkable except for long-standing back pain. Neurological examination revealed mild dysmetria and gait ataxia. There was no leg weakness or sphincteric dysfunction.MRI revealed superficial siderosis lining the cerebellum and brainstem, which prompted further imaging of the entire neuraxis in order to exclude an underlying cause. A myxopapillary ependymoma seen at the level of the conus medullaris was felt to be responsible for recurrent haemorrhage within the subarachnoid space.Excision of the spinal ependymoma was performed and the patient regained near-normal hearing following a cochlear implant. DISCUSSIONSuperficial siderosis presents with slowly progressive sensorineural hearing loss, gait ataxia and cerebellar dysarthria. Involvement of other cranial nerves has also been described. Clinical history and examination often suggest a neurodegenerative disorder.1 Superficial siderosis results from haemosiderin deposition in the subpial layers of the neuraxis, and complicates chronic haemorrhage within the subarachnoid space. The posterior fossa is preferentially affected, which may in part be explained by the presence of Bergmann glia in the cerebellum that display increased ferritin synthesis.2 The long glial segment of the vestibulocochlear nerve makes it more vulnerable to axonal damage by iron deposition.1 Bilateral sensorineural hearing loss is present in 95% of affected individuals.3 Figure 1 Linear hypointensity is seen along the cerebellar folia and superior vermis on the axial gradient echo images (A) compatible with extensive haemosiderin deposition. There is also hypointense lining of the midbrain tectum and pons. The sagittal T1-weighted image (B) reveals low signal intensity of the upper cerebellum due to extensive haemosiderin preferentially deposited in the upper cerebellum and superior vermis. The linear hypointensities are also appreciated on the axial T2 sequence (C) where they clearly outline the cerebellar folia and ventral pons and reflect the characteristic subpial haemosiderin deposition. Mild underlying cerebellar atrophy is also appreciated. The radiological findings are pathognomonic of superficial siderosis.