Progressive multifocal leukoencephalopathy in a renal transplant patient.

End-stage renal disease (ESRD) has a major impact on health and affects more than 600,000 people in the USA. The current mainstay treatments include dialysis and kidney transplantation (KT), and patients who have received KT have a higher quality of life and a lower mortality risk than those on chronic dialysis. Therefore, KT is considered the more preferred treatment modality for patients with ESRD. However, even though KT results in a higher long-term survival rate, the use of immunosuppressants is associated with various complications, including opportunistic infections and malignancies, which may lead to a higher risk of death in the first year after transplantation. Progressive multifocal leukoencephalopathy (PML) is a rare complication following KT, with an incidence of 0.027% in KT recipients. We present a case of PML following immunosuppressant therapy in a patient who received KT.

A 41-year-old female with progressive multifocal leukoencephalopathy after liver transplant.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by JC virus reactivation. Its occurrence is very rare after solid organ transplantation, especially liver transplantation. We report a patient who received liver transplantation due to liver failure resulting from autoimmune hepatitis and advanced PML presenting with aphasia. A 41-year-old female with a history of liver transplantation who received a usual immunosuppression regimen was admitted with a stroke attack resulting in right hemiplegia 2 months after liver transplantation. Surprisingly, she gradually developed dysarthria and left central facial paresis. A brain MRI showed an abnormal multifocal area with a high T2/flair signal in the deep subcortical white matter of the left hemisphere as well as the splenium of the corpus callosum. PCR evaluation of CSF for JCV was positive while other PCR results were negative. A liver transplant recipient receiving immunosuppressive treatment for a long time could develop PML due to JCV reactivation. Only eight cases of JCV infection were reported after liver transplantation by the time of reporting this case. Unfortunately, there is no definite treatment for PML.

Late relapse of progressive multifocal leucoencephalopathy postallogenic transplant in a young patient with CLL.

We describe a case of progressive multifocal leucoencephalopathy (PML) in a 39-year-old patient diagnosed with chronic lymphocytic leukaemia (CLL) who underwent two allogenic matched-sibling stem cell transplantations. PML was confirmed just after the first transplantation with cerebral MRI and by PCR in the cerebrospinal fluid. After immunosuppression withdrawal and cidofovir treatment, he achieved a reversal of clinical symptoms, John Cunningham (JC) virus positivity and MRI lesions regression. He remained asymptomatic for 5 years with no signs of infection activity, even though he received three new chemotherapy regimens due to a CLL relapse. However, after the second stem cell transplantation, new neurological symptoms began and a reactivation of the JC virus infection was detected. This time, treatment with mefloquine was started, but he experienced a progressive neurological deterioration and died 1 month after the symptoms began.

Tratamiento anestésico de un paciente diagnosticado de CADASIL (arteriopatía cerebral autosómica dominante con infartos subcorticales y leucoencefalopatía) / Anesthetic management of a patient diagnosed with CADASIL (cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy)

CADASIL (arteriopatía cerebral autosómica dominante, con infartos subcorticales y leucoencefalopatía) es una enfermedad hereditaria poco frecuente que puede tener implicaciones anestesiológicas, escasamente comunicadas. Presentamos el caso de un varón, previamente diagnosticado de CADASIL, que había sufrido un accidente cerebrovascular isquémico, con resonancia magnética compatible con leucoencefalopatía, y estaba muy limitado para las actividades diarias, con demencia, alteraciones del comportamiento, apatía e incontinencia urinaria. Entre sus antecedentes familiares, había varios parientes con síntomas psiquiátricos y accidentes cerebrovasculares, como su padre, dos hermanos y una hermana. Programado para artrodesis de la rodilla izquierda por complicaciones infecciosas tras cirugía de prótesis de rodilla, estaba tomando clopidogrel, que había suspendido 7 días antes. Se empleó anestesia combinada epidural-subaracnoidea y sedación intraoperatoria con midazolam, así como analgesia multimodal en el postoperatorio que incluyó analgesia epidural. No hubo incidencias notables. La anestesia y la analgesia epidurales permitieron mantener la estabilidad hemodinámica para una perfusión cerebral adecuada, clave para no empeorar los efectos de la arteriopatía crónica en la CADASIL(AU)

CADASIL (cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy) is an infrequent inherited disease that could have anesthetic implications. However these have rarely been reported. We present a male patient previously diagnosed with CADASIL, who had suffered an ischemic vascular cerebral accident with a MRI compatible with leukoencephalopathy, and who was dependent for daily activities, and sustained dementia, mood alterations, apathy, and urine incontinence. He had familial antecedents of psychiatric symptoms and ischemic stroke events in several relatives including his father, two brothers and one sister. He was scheduled for arthrodesis of the left knee because of multiple infectious complications of prosthetic knee surgery. He was under clopidogrel treatment which was withdrawn seven days before surgery. The procedure was performed under combined spinal-epidural anesthesia, intraoperative sedation with midazolam, and postoperative multimodal analgesia including epidural patient controlled analgesia. The perioperative management was uneventful and we outline the adequacy of managing these patients under regional anesthesia and analgesia, as these permit to maintain hemodynamic stability leading to adequate cerebral perfusion, key to avoid an increase in the effects of the chronic arteriopathy patients with CADASIL sustain(AU)

Treatment-related morbidity in atypical teratoid/rhabdoid tumor: multifocal necrotizing leukoencephalopathy.

BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive malignant brain tumor that, since it was first identified, has been treated with aggressive treatment regimens, e.g. high-dose chemotherapy with stem cell rescue and early radiotherapy. We reviewed our experience because of concerns with respect to treatment-related toxicity in our patients. METHODS: Seven patients with a median age at presentation of 18 months were diagnosed with AT/RT between 1996 and 2006. Tumor location was supratentorial in 2 patients, in the posterior fossa in 4 and spinal in 1. Gross total resection was performed in 1 patient, subtotal resection in 5 and biopsy only in 1. Adjuvant treatment consisted of chemotherapy and radiotherapy in 5 patients. RESULTS: Median progression-free survival was 4 months, and median overall survival was 7 months. Two children are alive at 44 and 102 months. Significant surgical and chemotherapy-related morbidity was seen. Biopsy-proven multifocal necrotizing leukoencephalopathy (MNL) was seen in one patient who is alive 44 months after diagnosis. Another patient who was thought to have recurrent tumor in the brainstem 9 months after diagnosis had imaging findings compatible with MNL. CONCLUSION: Although improving results are reported for AT/RT using intensive treatment regimens, treatment-related morbidity is considerable in this young patient population.

The surgical management of infections involving the cerebrum.

OBJECTIVE: Infection involving the cerebrum is a true neurosurgical emergency that requires rapid diagnosis and appropriate surgical and medical intervention to achieve good clinical outcome. METHODS: Because of the potential for devastating neurological sequelae, it is imperative that neurosurgeons be involved in the diagnosis and management of these serious conditions once an infection is suspected. With the advent of computed tomography and magnetic resonance imaging, it is now possible to detect an infectious process early in its course and follow the response to therapy. Although significantly more effective than in the past, antimicrobial therapy alone is insufficient to eradicate most intracranial infections, especially in the presence of compression or displacement of the cerebrum. Surgery remains an essential part of the management of intracranial infection because of its ability to provide immediate relief from pressure on neural structures and thereby result in clinical improvement. RESULTS: The most common infections affecting the brain, namely, cranial epidural abscess, subdural empyema, brain abscess, viral infection, tuberculosis, and neurocysticercosis, can each be associated with significant mass effect on the cerebrum that is greatly reduced through surgery. This relief, in combination with newer antimicrobial agents that have an improved ability to cross the blood brain barrier, has led to a reduction in the infection-related morbidity and mortality rates associated with intracranial infections. CONCLUSION: Combining advanced imaging and surgical techniques in the form of intraoperative magnetic resonance image-guided neurosurgery may further enhance clinical outcomes in these once uniformly fatal diseases.

Reversal of CNS-prophylaxis-related leukoencephalopathy after CSF shunting: case histories of identical twins.

We report the case histories of identical twin brothers who developed concordant acute lymphoblastic leukemia at the age of 4 years and who later developed leukoencephalopathy and hydrocephalus related to central nervous system prophylaxis by, in the first case intrathecally administered methotrexate and, in the second by intrathecally administered methotrexate and cranial irradiation. The delayed encephalopathy developed 9 and 22 months respectively after the first dose of intrathecal methotrexate. Both patients underwent cerebrospinal fluid shunting mainly for their hydrocephalus. The imaging studies have shown that both hydrocephalus and leukoencephalopathy have improved significantly after ventriculoperitoneal shunt insertion. We review the pathophysiology of prophylaxis-related encephalopathy in leukemia patients and summarize its clinical, histological, and radiological characteristics. We also discuss the possible contribution of altered cerebrospinal fluid dynamics in the development of leukoencephalopathy.