Progressive encephalomyelitis with rigidity and myoclonus (PERM) associated with anti-glycine receptor antibodies and urothelial carcinoma: a case report.

BACKGROUND: Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological condition with paraneoplastic etiology in about 20% of cases, usually presenting before or shortly after the oncological diagnosis is established. PERM associated with anti-glycine receptor antibodies is not previously reported in a patient with bladder cancer. CASE PRESENTATION: A 72-years-old Caucasian male was admitted with acute onset of dysarthria, dysphagia and trismus three years after initial surgical treatment for bladder cancer. The condition was initially diagnosed as tetanus and treated accordingly, but the diagnosis was reconsidered because of progression despite adequate treatment. Diagnostic workup on readmission revealed lung and paraaortal metastases from bladder cancer and anti-glycine receptor (anti-GlyR) antibodies both in the cerebrospinal fluid and in serum, which supplemented with the clinical presentation led to the diagnosis of PERM, presumably related to bladder cancer. The patient showed improvement and stabilization after treatment with intravenous immunoglobulin and chemotherapy against metastatic bladder cancer. CONCLUSION: To the best of our knowledge, this is the first reported case of anti-GlyR antibody positive PERM related to urothelial carcinoma. The symptoms mimicked tetanus, and responded to chemotherapy and immunotherapy.

A man in his forties with acute onset of speech impairment and unilateral paresis. / En mann i 40-årene med akutt innsettende talevansker og ensidig lammelse.

BACKGROUND: Stroke has several causes and the diagnostic investigation can be challenging. Neurosyphilis occurs when Treponema pallidum infects the central nervous system, and is a rare cause of stroke. CASE PRESENTATION: A man in in his late forties with diabetes mellitus and overweight presented with headache, speech impairment and right-sided stroke symptoms. He also had cognitive impairment and psychiatric symptoms. He underwent intravenous thrombolysis, and standardised stroke investigation was without clear findings. Cerebral MRI demonstrated non-specific subtle changes in the primary motor cortex in the left frontal lobe. However, lumbar puncture revealed elevated white blood cell count, and syphilis tests were positive. INTERPRETATION: Diagnosis of syphilis is often difficult and requires specific suspicion. Due to increasing incidence of the disease and its therapeutic consequences, alertness around the condition is important. Neurosyphilis should be suspected in young patients with stroke symptoms in the absence of risk factors and/or with cryptogenic strokes, especially in the presence of risk factors for syphilis infection and in patients from endemic areas.

Diffuse alveolar hemorrhage during alemtuzumab infusion in a patient with multiple sclerosis: a case report.

BACKGROUND: Diffuse alveolar bleeding is a potentially life-threatening condition that can be induced by several drugs. Whereas fatal cases have been reported in patients treated for other indications, no report have so far been published in a patient with multiple sclerosis treated with alemtuzumab. CASE PRESENTATION: We report a case of alemtuzumab-induced diffuse alveolar bleeding in a 29 year old woman with relapsing remitting multiple sclerosis. The patient developed acute shortness of breath, chest pain on inspiration and haemoptysis following the second infusion of alemtuzumab during the first treatment cycle. Computed tomography showed bilateral alveolar opacities. Bronchoscopy and broncho-alveolar lavage showed persistently bloody return with no evidence of infection. The symptoms resolved completely without treatment and control computed tomography performed one week later showed total resolution of pulmonary infiltrates. CONCLUSION: This is the first published report of diffuse alveolar bleeding in a patient with multiple sclerosis treated with alemtuzumab. Four similar cases in patients treated for multiple sclerosis and several fatal cases in patients treated for other conditions are registered at the World Health Organization database of suspected adverse events (VIgiBase©), underscoring that this is a serious and possibly under-recognized complication of alemtuzumab which can also occur in the treatment of multiple sclerosis. The clinician should consider the possibility of diffuse pulmonary haemorrhage in patients with sudden onset of respiratory distress and haemoptysis following administration of alemtuzumab for multiple sclerosis.

Severe inflammatory disease activity 14 months after cessation of Natalizumab in a patient with Leber's optic neuropathy and multiple sclerosis - a case report.

BACKGROUND: Leber's hereditary optic neuropathy (LHON) co-occuring with multiple sclerosis-like disease (LHON-MS) is suggested to be a separate disease entity denoted Harding's disease. Little is known about the response to initiation and discontinuation of potent immunomodulatory treatment in LHON-MS. CASE PRESENTATION: We describe a LHON-MS patient with 27 years disease duration who developed severe disease activity peaking 14 months after discontinuation of natalizumab, with extensive new inflammatory lesions throughout the brain and in the spinal cord resembling immune inflammatory reconstitution syndrome. She had previously been clinically and radiologically stable on natalizumab treatment for 6 years, and before that only experienced subtle clinical activity during 9 years on interferon beta1a. CONCLUSION: This is the first report on severe exacerbation of inflammatory disease activity after discontinuation of natalizumab in LHON-MS, and suggests that late rebound activity can occur in these patients.