Contributions
Abstract: P985
Type: Poster
Abstract Category: Pathology and pathogenesis of MS - MS and infections
Objective: To report a case of severe brain atrophy in a 34-year-old Caucasian gentleman post infection with progressive multifocal leukoencephalopathy (PML).
Background: Progressive multifocal leukoencephalopathy is a rare but serious infection associated with immunosuppressive agents such as Nataluzimab (Tysabri). Poor survival indicators include older age, higher EDSS prior to infection and widespread involvement seen on MRI. Current treatment protocols are experimental with little supportive evidence. PML is caused by a neurotropic polyoma virus called John Cunningham virus (JC virus), which infects oligodendrocytes, astrocytes and neurons causing lysis. Very little is known about subsequent widespread atrophy from PML. However the JC virus has been demonstrated to cause cerebellar atrophy as a result of granular cell degeneration in the cerebellum and therefore may cause global atrophy when multiple lobes are involved.
Methods / Results: A 34-year-old Caucasian gentleman, with known relapsing remitting multiple sclerosis (EDSS 3.5 before Tysabri) and established on treatment with Tysabri for three years (EDSS 0) presented with a one month history of progressive neurological symptoms. MRI brain showed widespread multi-lobar changes with involvement of the brain stem and basal ganglia strongly suggestive of PML, further confirmed with the presence of JC virus on cerebrospinal fluid analysis.
He was admitted and received plasmapheresis, cidofovir, mirtazapine, mefloquine, granulocyte colony stimulation factor, maroviroc as well as a levetiracetam and sodium valproate. He deteriorated and ultimately required sedation for myoclonic status epilepticus. He continued with treatment and stabilised but has been left with permanent neurological deficits (EDSS 6).
Sequential MRI brain imaging has shown improvement in the signal change from PML but a gradual and significant loss of the cerebral cortical volume globally (see images).
Conclusions: This case highlights that gross brain atrophy may develop in severe, cases of multi-lobar PML. Additionally it demonstrates a possible approach to treating life threatening PML.
Disclosure: Dr. Sonia Kumari: nothing to disclose
Dr. Gordon Mazibrada: has served at advisory boards for Biogen, Merck, Teva, Novartis, Genzyme and Roche and our MS department has received funding from those companies for research projects and service developments.
Abstract: P985
Type: Poster
Abstract Category: Pathology and pathogenesis of MS - MS and infections
Objective: To report a case of severe brain atrophy in a 34-year-old Caucasian gentleman post infection with progressive multifocal leukoencephalopathy (PML).
Background: Progressive multifocal leukoencephalopathy is a rare but serious infection associated with immunosuppressive agents such as Nataluzimab (Tysabri). Poor survival indicators include older age, higher EDSS prior to infection and widespread involvement seen on MRI. Current treatment protocols are experimental with little supportive evidence. PML is caused by a neurotropic polyoma virus called John Cunningham virus (JC virus), which infects oligodendrocytes, astrocytes and neurons causing lysis. Very little is known about subsequent widespread atrophy from PML. However the JC virus has been demonstrated to cause cerebellar atrophy as a result of granular cell degeneration in the cerebellum and therefore may cause global atrophy when multiple lobes are involved.
Methods / Results: A 34-year-old Caucasian gentleman, with known relapsing remitting multiple sclerosis (EDSS 3.5 before Tysabri) and established on treatment with Tysabri for three years (EDSS 0) presented with a one month history of progressive neurological symptoms. MRI brain showed widespread multi-lobar changes with involvement of the brain stem and basal ganglia strongly suggestive of PML, further confirmed with the presence of JC virus on cerebrospinal fluid analysis.
He was admitted and received plasmapheresis, cidofovir, mirtazapine, mefloquine, granulocyte colony stimulation factor, maroviroc as well as a levetiracetam and sodium valproate. He deteriorated and ultimately required sedation for myoclonic status epilepticus. He continued with treatment and stabilised but has been left with permanent neurological deficits (EDSS 6).
Sequential MRI brain imaging has shown improvement in the signal change from PML but a gradual and significant loss of the cerebral cortical volume globally (see images).
Conclusions: This case highlights that gross brain atrophy may develop in severe, cases of multi-lobar PML. Additionally it demonstrates a possible approach to treating life threatening PML.
Disclosure: Dr. Sonia Kumari: nothing to disclose
Dr. Gordon Mazibrada: has served at advisory boards for Biogen, Merck, Teva, Novartis, Genzyme and Roche and our MS department has received funding from those companies for research projects and service developments.