Glutamic acid decarboxylase antibody-positive paraneoplastic stiff limb syndrome associated with carcinoma of the breast.

Stiff limb syndrome (SLS) is a rare "focal" variant of stiff person syndrome which presents with rigidity and painful spasms of a distal limb, and abnormal fixed foot or hand postures. Anti-glutamic acid decarboxylase antibodies (GAD-Ab) are variably present in most cases. Most reported cases of SLS are unassociated with cancer. We describe a patient with SLS as a paraneoplastic manifestation of breast carcinoma, in whom GAD-Ab was present. The patient responded very well to oral diazepam, baclofen and steroids.This is the third reported case of SLS as a paraneoplastic accompaniment to cancer.

Primary central nervous system lymphoma: a profile of 26 cases from Western India.

BACKGROUND: Primary central nervous system (CNS) lymphoma (PCNSL) is a rare malignant non-Hodgkin's lymphoma and it accounts for 1% of all intracranial tumors. Only a few PCNSL studies have been reported from India, and studies on prognostic factors determining outcome, or evaluation of the response to currently accepted treatment, are lacking. AIMS: This study attempts to further delineate the clinical, radiological and pathological profile of PCNSL in India, to evaluate response to treatment and to assess usefulness of the International Extranodal Lymphoma Study Group (IELSG) score. SETTINGS AND DESIGN: All patients with pathologically proven PCNSL admitted over three years at a large tertiary care institution were studied. MATERIALS AND METHODS: Clinical features, IELSG prognostic score, imaging and pathological features, and response to treatment were evaluated. Results were analyzed using chi 2 test. RESULTS: Of 26 patients found, all except two were immunocompetent. Median age at diagnosis was 59 years. Focal deficits (76.9%) and neuropsychiatric symptoms (57.6%) were the commonest presenting complaints. Except for one case, at least some contrast enhancement was seen in brain lesions of all patients. Pathological studies showed high grade diffuse large B-cell (DLBCL) histology in 96.2% of patients. Of 22 patients who received methotrexate (MTX) based chemotherapy with/without radiotherapy; six died, with a response rate of 72.7%. Median survival was 10 months. Median follow-up duration was 14.5 months. Four patients developed treatment-related cognitive decline. All six patients with IELSG score of 4/5 died, while all 16 patients with a score of 0-3 survived. CONCLUSIONS: PCNSL presents most commonly in the sixth decade with focal neurological deficit, behavioral symptoms and cognitive decline. High grade DLBCL is the commonest histological subtype. Steroids should ideally be withheld until biopsy as they may confound the diagnosis. Most immunocompetent patients respond well to high dose MTX-based chemotherapy with/without radiation. High IELSG scores correlate with worse prognosis in patients with PCNSL.

Dermoid of the conus medullaris.

Dermoids are rare midline tumours. When involving the spinal cord they usually present early, in the first decade of life. We present a case of an elderly male with paraparesis. His MRI showed cord expansion at the tip of the conus medullaris; contrast enhancement with Gadolinium revealed a cystic lesion and histopathology confirmed the diagnosis of a dermoid cyst.

Neurofibromatosis type I presenting with urinary retention and lung collapse.

A 26-year-old male presented with urinary retention, spastic paraparesis and a swelling in the right side of the neck. Urinary retention was due to a neurofibroma of a lumbar spinal root compressing the urinary bladder and right ureter retroperitoneally. Paraparesis was due to an intraspinal neurofibroma causing cord compression at the cervicodorsal junction. In addition, this tumour was contiguous with the mass in the neck, and also with another large mass in the right hemithorax which was causing collapse of the right lung.