Good outcome of brain stem progressive multifocal leukoencephalopathy in an immunosuppressed renal transplant patient: Importance of early detection and rapid immune reconstitution.
Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic and often fatal disease of the CNS which may occur under immunosuppression in transplant patients. Brain stem PML is associated with a particularly bad prognosis. Here, we present a case of a renal transplant patient treated with mycophenolate mofetil (MMF) and tacrolimus who developed brain stem PML with limb ataxia, dysarthria and dysphagia. Diagnosis was established by typical MRI features and detection of JCV-DNA in the CSF. Immune reconstitution after stopping MMF and tacrolimus led to a complete and sustained remission of symptoms with improvement of the brain stem lesion over a follow-up over 20months. In summary, early detection of PML and consequent treatment may improve neurological outcomes even in brain stem disease with a notorious bad prognosis.
Department of Neurology, Friedrich Alexander University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany.
Roland
R
Sauer
Neuroradiology Section, Friedrich Alexander University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany.
Philipp
P
Gölitz
Department of Nephrology, Friedrich Alexander University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany.
Johannes
J
Jacobi
Department of Neurology, Friedrich Alexander University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany.
Stefan
S
Schwab
Department of Neurology, Friedrich Alexander University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany.
Ralf A
RA
Linker
Department of Neurology, Friedrich Alexander University of Erlangen-Nuremberg, Schwabachanlage 6, 91054 Erlangen, Germany. Electronic address: de-hyung.lee@uk-erlangen.de.
De-Hyung
DH
Lee
D000328:Adult; D000904:Antibiotics, Antitubercular / Q000627:therapeutic use; D001933:Brain Stem / Q000000981:diagnostic imaging / Q000473:pathology*; D004247:DNA / Q000134:cerebrospinal fluid; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007577:JC Virus / Q000235:genetics / Q000276:immunology; D007676:Kidney Failure, Chronic / Q000188:drug therapy / Q000601:surgery; D016030:Kidney Transplantation / Q000009:adverse effects*; D007968:Leukoencephalopathy, Progressive Multifocal / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000209:etiology* / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D009173:Mycophenolic Acid / Q000627:therapeutic use; D016559:Tacrolimus / Q000627:therapeutic use
D000904:Antibiotics, Antitubercular; D007166:Immunosuppressive Agents; D004247:DNA; D009173:Mycophenolic Acid; D016559:Tacrolimus
Immunosuppression; Mycophenolate mofetil; Outcome; Polyoma virus; Progressive multifocal leukoencephalopathy; Renal transplantation; Tacrolimus