http://web.b.ebscohost.com/abstract?sit ... d144209763
Background: Fulminant multiple sclerosis (MS) is rare, and the approach to treatment beyond first-line therapies (high-dose steroids, plasma exchange, and intravenous [IV] immunoglobulins) varies. Single case reports of diagnosis and treatment of fulminant MS can provide helpful resources for the clinician.
Cyclophosphamide is an alkylating agent that suppresses T- and B-cell function, interleukin 12, and T-helper type 1 (Th1) responses, thereby enhancing Th2, Th3 responses. Review of published reports suggests that cyclophosphamide has utility in early stages of MS during which inflammation predominates over degenerative processes in the central nervous system as seen by gadolinium (Gd)--enhancing lesions.
Objectives: To describe the clinical course of a case of acute MS successfully treated with cyclophosphamide. Methods: Case study.
Results: A 41-year-old woman with no medical history developed facial paresthesia, imbalance, and dizziness. Neurologic examination showed hyperreflexia. Magnetic resonance imaging (MRI) showed numerous Gd-enhancing white matter lesions throughout the brain and cervical and thoracic spinal cord. Initial treatment with 1000 mg of IV methylprednisolone did not result in symptom improvement but decreased the burden of enhancing lesions on repeat MRI. On hospital day 8, she clinically deteriorated, and plasma exchange was initiated. She continued to worsen and developed respiratory failure requiring mechanical ventilation, cranial nerve palsies, and quadriplegia. IV immunoglobulin was given with no clinical response. A brain biopsy was obtained due to concern for lymphoma, but it showed severe demyelination with relative axonal preservation. Due to significant clinical deterioration, we proceeded with induction treatment with cyclophosphamide (600 mg/m2 daily for 4 doses). Treatment resulted in gradual clinical and radiologic improvement. For maintenance therapy, she received 1 dose of IV rituximab 1000 mg and was discharged to a long-term acute care facility. Subsequently she was weaned off mechanical ventilation, significantly restored upper limb functions, and is able to walk for more than 40 ft with assistance. At day 104 from initial presentation, she continues to improve with no new relapse activity. Follow-up brain and spinal cord MRI 21 days after discharge demonstrated resolution of most of the lesions, interval improvement of some, and no new or enhancing lesions identified.
Conclusions: Induction therapy with cyclophosphamide combined with supportive care can help improve acute fulminant demyelination.