Eosinophilic Fasciitis Responds Well to Steroids and Methotrexate.

Eosinophilic fasciitis (EF) is a rare systemic inflammatory disease with an unknown etiology. Making a diagnosis in such a case is always a challenge as it is a rare disease and mimics scleroderma and scleroderma-like syndrome but should be kept in mind as it carries a high mortality. Furthermore, it is a treatable disease. Here, we report a 41-year-old woman who presented to the rheumatology clinic at the Royal Hospital, Muscat, Oman, with a one-month history of bilateral swelling of the forearms along with skin tightness and fingers contraction. Her history and physical examination along with histopathological examination and magnetic resonance imaging findings were consistent with EF. She showed an excellent response to steroids and methotrexate which is not a combination therapy that has been tried or mentioned previously.

Rigid Spine Syndrome among Children in Oman.

OBJECTIVES: Rigidity of the spine is common in adults but is rarely observed in children. The aim of this study was to report on rigid spine syndrome (RSS) among children in Oman. METHODS: Data on children diagnosed with RSS were collected consecutively at presentation between 1996 and 2014 at the Sultan Qaboos University Hospital (SQUH) in Muscat, Oman. A diagnosis of RSS was based on the patient's history, clinical examination, biochemical investigations, electrophysiological findings, neuro-imaging and muscle biopsy. Atrophy of the paraspinal muscles, particularly the erector spinae, was the diagnostic feature; this was noted using magnetic resonance imaging of the spine. Children with disease onset in the paraspinal muscles were labelled as having primary RSS or rigid spinal muscular dystrophy. Secondary RSS was classified as RSS due to the late involvement of other muscle diseases. RESULTS: Over the 18-year period, 12 children were included in the study, with a male-to-female ratio of 9:3. A total of 10 children were found to have primary RSS or rigid spinal muscular dystrophy syndrome while two had secondary RSS. Onset of the disease ranged from birth to 18 months of age. A family history was noted, with two siblings from one family and three siblings from another (n = 5). On examination, children with primary RSS had typical features of severe spine rigidity at onset, with the rest of the neurological examination being normal. CONCLUSION: RSS is a rare disease with only 12 reported cases found at SQUH during the study period. Cases of primary RSS should be differentiated from the secondary type.

Progressive neuropsychiatric symptoms and motor impairment.

A 42-year-old white man presented with cognitive impairment and behavioral changes followed by rapidly progressive motor and gait impairment. Magnetic resonance imaging revealed striking multifocal white matter signal change, areas of restricted diffusion, diffuse callosal signal change, and atrophy and hyperintensity of the corticospinal tracts. A broad range of etiologies warrant consideration in this case, including degenerative, vascular, inflammatory, metabolic, and neoplastic diseases.

Rhabdoid meningioma presenting with subependymal and diffuse meningeal involvement but no mass lesion.

Rhabdoid meningiomas are rare, aggressive tumours of the meninges that have a generally poor prognosis. We report a 49-year-old man with a background history of sarcoidosis who presented with nausea and vomiting. Imaging showed generalised leptomeningeal and subependymal enhancement suggestive of chronic meningitis. He had multiple lumbar punctures and a brain biopsy, none of which led to a pathological diagnosis. He died within months, and a postmortem examination was performed. At this stage, a diagnosis of rhabdoid meningioma was made. The clinical and radiological presentation of rhabdoid meningioma as a diffuse leptomeningeal process without a mass lesion is unique. All other published cases of rhabdoid meningioma have been of a discrete lesion. This highlights the importance of a tissue diagnosis in patients where the imaging is non-specific. A brief review of rhabdoid meningioma follows.