Contributions
Abstract: EP1490
Type: ePoster
Abstract Category: Therapy - disease modifying - Immunomodulation/Immunosuppression
Background: Cyclophosphamide is a cytotoxic agent occasionally used to treat inflammatory conditions of the nervous system. We present an exceptional case of its successful use to treat a first presentation of life threatening Multiple Sclerosis (MS).
Case: A 31 year old Caucasian female, with background of opiate addiction presented with six month history of unsteadiness, falls and confusion. Initial examination demonstrated pyramidal weakness, hyper-reflexia, cerebellar signs, and cognitive impairment. Six days into admission she suffered respiratory compromise requiring intubation and mechanical ventilation. Magnetic Resonance Imaging demonstrated global cerebral atrophy and multiple widespread, confluent, partially-enhancing T2 and FLAIR white matter hyperintensities throughout both cerebral hemispheres, brainstem, cerebellum and cervical spinal cord. This was felt to most likely represent a demyelinating pathology. Investigations for other inflammatory, infective, toxic, metabolic, neurodegenerative or autoimmune conditions were negative. Cerebrospinal fluid was acellular but significant for the presence of oligoclonal bands.
A clinical diagnosis of demyelinating disease, likely MS, was obtained. She was initially treated with high dose steroids (pulsed intravenous methylprednisolone for a total for eight days and then subsequently oral prednisolone) and five cycles of plasma exchange achieving only mild improvement. She remained in high dependency care 27 days into admission, still requiring tracheostomy, enteral feeding and fully dependant on nursing care; equating to Expanded Disability Status Scale (EDSS) of 9.5. Immunosuppression was escalated to six cycles of cyclophosphamide (15mg/kg) in two week intervals. Subsequently there was a dramatic improvement; she began to talk fluently on day 51 and walk independently after day 91. Currently, she remains globally cognitively impaired (Addenbrooke"s Cognitive Examination Score 42/100) and has subtle pyramidal weakness in her right arm only; giving EDSS 4.5. To date, she has not exhibited signs of cyclophosphamide toxicity and there has been no progression of radiological findings.
Conclusion: This case highlights the remarkable outcome that can be achieved from using potentially toxic immunosuppression in the context of first presentation of highly aggressive MS, even when initiated in a critical care setting.
Disclosure:
Kumar: nothing to disclose
Sanchez: nothing to disclose
Brex: nothing to disclose
Coles: nothing to disclose
Harikrishnan: nothing to disclose
Abstract: EP1490
Type: ePoster
Abstract Category: Therapy - disease modifying - Immunomodulation/Immunosuppression
Background: Cyclophosphamide is a cytotoxic agent occasionally used to treat inflammatory conditions of the nervous system. We present an exceptional case of its successful use to treat a first presentation of life threatening Multiple Sclerosis (MS).
Case: A 31 year old Caucasian female, with background of opiate addiction presented with six month history of unsteadiness, falls and confusion. Initial examination demonstrated pyramidal weakness, hyper-reflexia, cerebellar signs, and cognitive impairment. Six days into admission she suffered respiratory compromise requiring intubation and mechanical ventilation. Magnetic Resonance Imaging demonstrated global cerebral atrophy and multiple widespread, confluent, partially-enhancing T2 and FLAIR white matter hyperintensities throughout both cerebral hemispheres, brainstem, cerebellum and cervical spinal cord. This was felt to most likely represent a demyelinating pathology. Investigations for other inflammatory, infective, toxic, metabolic, neurodegenerative or autoimmune conditions were negative. Cerebrospinal fluid was acellular but significant for the presence of oligoclonal bands.
A clinical diagnosis of demyelinating disease, likely MS, was obtained. She was initially treated with high dose steroids (pulsed intravenous methylprednisolone for a total for eight days and then subsequently oral prednisolone) and five cycles of plasma exchange achieving only mild improvement. She remained in high dependency care 27 days into admission, still requiring tracheostomy, enteral feeding and fully dependant on nursing care; equating to Expanded Disability Status Scale (EDSS) of 9.5. Immunosuppression was escalated to six cycles of cyclophosphamide (15mg/kg) in two week intervals. Subsequently there was a dramatic improvement; she began to talk fluently on day 51 and walk independently after day 91. Currently, she remains globally cognitively impaired (Addenbrooke"s Cognitive Examination Score 42/100) and has subtle pyramidal weakness in her right arm only; giving EDSS 4.5. To date, she has not exhibited signs of cyclophosphamide toxicity and there has been no progression of radiological findings.
Conclusion: This case highlights the remarkable outcome that can be achieved from using potentially toxic immunosuppression in the context of first presentation of highly aggressive MS, even when initiated in a critical care setting.
Disclosure:
Kumar: nothing to disclose
Sanchez: nothing to disclose
Brex: nothing to disclose
Coles: nothing to disclose
Harikrishnan: nothing to disclose