Contributions
Abstract: EP1292
Type: ePoster
Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis
Introduction: Multiple sclerosis and neuromyelitis optica are the most common inflammatory disorders of the central nervous system (CNS) and commonly initially present as brainstem syndromes or myelites. They can be mimicked by other inflammatory systemic diseases. We report a fatal case of Chronic Adult T-Cell Leukemia/Lymphoma (ATLL) which initially manifested as a brainstem syndrome associated to a longitudinally extensive transverse myelitis (LETM).
Case presentation: A 38 year old previously healthy female was admitted to our service with a one and a half month history of corticosteroid unresponsive tetraparesis with a sensory level and brainstem symptoms. She had a family history of HTLV-I-associated myelopathy/tropical spastic paraparesis.
Blood workup revealed significant leukocytosis, positive human T-lymphotropic virus (HTLV) serum screening test and normal cerebrospinal fluid analysis. Magnetic resonance imaging displayed multiple tumefactive lesions with contrast enhancement primarily in the pons with extension to the remaining of the brainstem, cervical and thoracic spine. High dose intravenous methylprednisolone was initiated with no clinical response. In spite of treatment the patient developed a locked in syndrome with acute ventilatory failure within days of admission. Plasmapheresis was initiated. Subsequently, a peripheral blood smear found the presence of “flower cells” with a positive CD25 marker - a diagnose of Chronic Adult T-Cell Leukemia/Lymphoma (ATLL) was performed and specific treatment (interpheron alpha-2b and zidovudine) was offered. Despite our efforts the patient passed away due to severe dysautonomia. Post mortem analysis revealed nonspecific perivascular and parenchymal lymphocytic infiltrates with no positivity to antiaquaporine-4 or lymphoma markers.
Discussion: Worldwide approximately 10-20 million people are infected with HLTV. The risk of development of ATLL amongst infected patients is 2- 5%, few of which will ever present neurologic symptoms. There are few reports of ATLL with CNS involvement as an initial manifestation- it is even rarer in the chronic subtype. To the best of our knowledge this is the first case of chronic ATLL opening with a rapidly progressive corticosteroid unresponsive brainstem syndrome.
Conclusion: Inflammatory disorders of the CNS comprise a range of differential diagnosis. A high level of suspicion is needed in order to perform timely differential diagnosis and offer treatment.
Disclosure:
Ana Beatriz A G R Gomes: nothing to disclose.
Herval Ribeiro Soares Neto: nothing to disclose.
Marcelo Delboni Lemos: nothing to disclose.
Dagoberto Callegaro: received grants related to congress meetings and preceptorship from Genzyme, Biogen and Roche,
Samira Luísa Apóstolos Pereira: received grants related to congress meetings and preceptorship from Genzyme and Roche.
Abstract: EP1292
Type: ePoster
Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis
Introduction: Multiple sclerosis and neuromyelitis optica are the most common inflammatory disorders of the central nervous system (CNS) and commonly initially present as brainstem syndromes or myelites. They can be mimicked by other inflammatory systemic diseases. We report a fatal case of Chronic Adult T-Cell Leukemia/Lymphoma (ATLL) which initially manifested as a brainstem syndrome associated to a longitudinally extensive transverse myelitis (LETM).
Case presentation: A 38 year old previously healthy female was admitted to our service with a one and a half month history of corticosteroid unresponsive tetraparesis with a sensory level and brainstem symptoms. She had a family history of HTLV-I-associated myelopathy/tropical spastic paraparesis.
Blood workup revealed significant leukocytosis, positive human T-lymphotropic virus (HTLV) serum screening test and normal cerebrospinal fluid analysis. Magnetic resonance imaging displayed multiple tumefactive lesions with contrast enhancement primarily in the pons with extension to the remaining of the brainstem, cervical and thoracic spine. High dose intravenous methylprednisolone was initiated with no clinical response. In spite of treatment the patient developed a locked in syndrome with acute ventilatory failure within days of admission. Plasmapheresis was initiated. Subsequently, a peripheral blood smear found the presence of “flower cells” with a positive CD25 marker - a diagnose of Chronic Adult T-Cell Leukemia/Lymphoma (ATLL) was performed and specific treatment (interpheron alpha-2b and zidovudine) was offered. Despite our efforts the patient passed away due to severe dysautonomia. Post mortem analysis revealed nonspecific perivascular and parenchymal lymphocytic infiltrates with no positivity to antiaquaporine-4 or lymphoma markers.
Discussion: Worldwide approximately 10-20 million people are infected with HLTV. The risk of development of ATLL amongst infected patients is 2- 5%, few of which will ever present neurologic symptoms. There are few reports of ATLL with CNS involvement as an initial manifestation- it is even rarer in the chronic subtype. To the best of our knowledge this is the first case of chronic ATLL opening with a rapidly progressive corticosteroid unresponsive brainstem syndrome.
Conclusion: Inflammatory disorders of the CNS comprise a range of differential diagnosis. A high level of suspicion is needed in order to perform timely differential diagnosis and offer treatment.
Disclosure:
Ana Beatriz A G R Gomes: nothing to disclose.
Herval Ribeiro Soares Neto: nothing to disclose.
Marcelo Delboni Lemos: nothing to disclose.
Dagoberto Callegaro: received grants related to congress meetings and preceptorship from Genzyme, Biogen and Roche,
Samira Luísa Apóstolos Pereira: received grants related to congress meetings and preceptorship from Genzyme and Roche.