A severe case of neuroleukemiosis caused by B cell chronic lymphocytic leukemia, presenting as mononeuritis multiplex.

AIMS: To report an exceptional case of nerve infiltration by an otherwise benign chronic B cell leukemia, inducing severe mononeuritis multiplex. METHODS: The patient underwent extensive evaluation, including nerve conduction study and myography, brain and plexus MRI, and nerve biopsy. RESULTS: The clinical and electrophysiological diagnosis was a mononeuritis multiplex with severe motor and sensory involvement; only the nerve biopsy allowed definite diagnosis and introduction of chemotherapy, leading to resolution of sensory deficit and progressive motor improvement. DISCUSSION: Neuroleukemiosis caused by chronic lymphoid leukemia is an exceptional diagnosis. The presence of other possible causes like cryoglobulinemia could induce avoidance of nerve biopsy thus undertreating patient, since steroid treatment is not expected to be efficient on lymphocytic proliferation. Our case stretches the importance of nerve biopsy and raises neuromuscular specialist's awareness of this rare entity.

Painless progressive mononeuritis multiplex secondary to AML associated neuroleukemiosis.

Neuroleukemiosis describes peripheral nerve involvement secondary to leukemic infiltration, a rare complication of leukemia with various clinical presentations, leading to diagnostic challenges for hematologists and neurologists. We present two cases of painless progressive mononeuritis multiplex secondary to neuroleukemiosis. A literature review of previously reported cases of neuroleukemiosis was undertaken. Neuroleukemiosis may present as a progressive mononeuritis multiplex. The diagnosis of neuroleukemiosis requires a high index of suspicion and be aided by repeated CSF analysis.

Neuropatía múltiple como manifestación clínica de una recidiva de leucemia / Isolated multiple neuropathy as clinical manifestation of leukemia relapse

Introducció La neuroleucemiosis es una rara enfermedad del sistema nervioso periférico producida por la infiltración por células leucémicas. Caso clínico: Presentamos el caso de una paciente de 34 años con antecedente de una leucemia mielomonoblástica aguda en remisión, que presentaba una parálisis progresiva del nervio mediano derecho, del facial bilateral y del peroneal izquierdo. El electromiograma confirmó el diagnóstico de una neuropatía múltiple. La tomografía por emisión de positrones-tomografía computarizada mostró un hipermetabolismo de ambos nervios ciáticos, facial bilateral y mediano derecho. La biopsia de médula ósea confirmó la recidiva de la leucemia, por lo que se inició un nuevo ciclo de quimioterapia con mejoría de los déficits neurológicos. Conclusión: La infiltración del sistema nervioso periférico por células leucémicas puede simular múltiples síndromes neurológicos dependiendo de las estructuras afectadas. La barrera hematonerviosa actúa como defensa de las células leucémicas contra la quimioterapia y el sistema inmunitario, por lo que el sistema nervioso periférico constituye un reservorio de las células leucémicas. Por ello, la neuroleucemia debe considerarse en pacientes con antecedentes de leucemia que presenten síntomas aislados de afectación del sistema nervioso periférico.(AU)

Introduction: Neuroleukemia is a rare disorder of the peripheral nervous system due to leukemic cell infiltration. Case report: We present the case of a 34-year-old patient with history of acute myelomonoblastic leukemia in remission that presented progressive paresis of the right median, bilateral facial, and left peroneal nerves. The electromyogram confirmed the diagnosis of multineuropathy. A PET-CT showed hypermetabolism of both sciatic, facial, and right median nerves. A bone marrow aspirate confirmed the leukemia relapse so a new round of chemotherapy was performed with improvement of the neurological deficit. Conclusion: Peripheral nervous system infiltration by leukemic cells can mimic multiple syndromes depending on the structures involved. The nerve-blood barrier acts as a defense of leukemic cells against chemotherapy and the immune system. Thus, the peripheral nervous system constitutes a reservoir of leukemic cells. Neuroleukemia should be considered in patients with history of acute leukemia who have isolated symptoms of the peripheral nerve.(AU)

Neurologic complications of acute myeloid leukemia. Diagnostic approach and therapeutic modalities.

Acute myeloid leukemia (AML) comprises a heterogeneous group of aggressive blood malignancies that arise from clonal expansion of malignant hematopoietic precursor cells in the bone marrow. Neurologic manifestations of these malignancies are manifolds. AML is the most common form of acute leukemia in adults and this review describes the neurologic complications in this age group. Neurologic symptoms and signs may develop in AML either from a direct neoplastic involvement of the central or the peripheral nervous system or as an indirect effect of the disease process. Direct involvement of the nervous system includes invasion of the central or the peripheral nervous system (parenchymal and leptomeningeal dissemination, myeloid sarcoma, neuroleukemiosis). Thrombotic and hemorrhagic events are common manifestations of indirect involvement of the nervous system and they are the outcome of hyperleukocytosis, thrombocytopenia and coagulopathy. Many neurologic complications are iatrogenic and include diverse categories such as lumbar puncture and intrathecal or systemic chemotherapy and targeted therapies, radiotherapy and allogeneic stem cell transplantation. Most neurologic manifestations require urgent treatment and confer a poor prognosis. This review describes the neurologic complications of acute myeloid malignancies in the era of contemporary treatment. Those manifestations require expert consideration of their origin as they are being identified with increasing frequency as patients survive longer.

Nerve biopsy in acquired neuropathies.

A diagnosis of neuropathy can typically be determined through clinical assessment and focused investigation. With technological advances, including significant progress in genomics, the role of nerve biopsy has receded over recent years. However, making a specific and, in some cases, tissue-based diagnosis is essential across a wide array of potentially treatable acquired peripheral neuropathies. When laboratory investigations do not suggest a definitive diagnosis, nerve biopsy remains the final step to ascertain the etiology of the disease. The present review highlights the utility of nerve biopsy in confirming a diagnosis, while further illustrating the importance of a tissue-based diagnosis in relation to treatment strategies, particularly when linked to long-term immunosuppressive therapies.

Neuroleukemiosis: Diagnosis and management.

An exceedingly rare manifestation of leukemia, termed neuroleukemiosis, involves peripheral nerve infiltration by leukemic cells. Patients with neuroleukemiosis typically present with a peripheral neuropathy and/or chloromatous masses. The diagnosis is supported by, and established with, electrophysiologic testing, imaging, histopathology, and immunophenotyping. We present the case of 21 year old male with multiply relapsed M4 type of acute myelogenous leukemia (AML) who presented with extremity pain and was subsequently found to have multiple cervical, thoracic, and lumbosacral nerve root masses. A diagnosis of neuroleukemiosis was established via CT-guided biopsy and immunophenotyping. The patient's neuroleukemiosis responded well to chemotherapy, donor lymphocyte infusions, and spinal irradiation. The literature is reviewed regarding this interesting and rare clinical condition.

Neuroleukemiosis: Two Case Reports.

Extramedullary tumors composed of myeloblasts or monoblasts can present in various locations. Patients with a history of acute myeloid leukemia (AML) can present with neuropathic pain and no evidence of relapse of their leukemia. Neuroleukemiosis is a form of extramedullary tumor present in the peripheral nervous systems (PNS) of leukemia patients. We report two AML patients who were in remission and later presented with neurological symptoms due to neuroleukemiosis with negative bone marrow biopsies.

Diagnosing neuroleukemiosis: Is there a role for 18F-FDG-PET/CT?

An imaging case is presented on a patient referred to our department for an 18F-FDG-PET/CT, as a paraneoplastic syndrome was suspected due to his clinical situation. He had a history of acute myeloid leukemia (AML) treated two years earlier, with sustained complete remission to date. 18F-FDG-PET/CT findings revealed hypermetabolism in almost all nerve roots, suggesting meningeal spread, consistent with the subsequent MRI findings. Cerebrospinal fluid (CSF) findings confirmed a leptomeningeal reactivation of AML. Although not many studies have evaluated the role of 18F-FDG-PET/CT in leukemia, it is a noninvasive tool for detecting extramedullary sites of disease and a good imaging alternative for those patients on whom an MRI cannot be performed.

Isolated leukemic infiltration of peripheral nervous system.

INTRODUCTION: Isolated leukemic infiltration of peripheral nerves without central nervous system involvement, or isolated neuroleukemiosis, is an extremely rare complication of leukemia. METHODS: We report the case of a patient with isolated neuroleukemiosis and review the pertinent literature. RESULTS: A man with a 12-year history of acute monoblastic leukemia presented with median mononeuropathy. Magnetic resonance imaging revealed a thickened median nerve. Nerve biopsy confirmed the diagnosis of leukemic infiltration. Clinical resolution was achieved through local radiation. Only 10 cases of isolated neuroleukemiosis have been reported. Most were in remission from leukemia. Our patient is the only one who was considered clinically cured of leukemia. CONCLUSIONS: The presumed pathophysiology of isolated neuroleukemiosis is hematogenous spread of leukemic cells into the peripheral nervous system across the blood-nerve barrier. It should be considered in the differential diagnosis in patients with leukemia who present with neuropathy, even when they are considered to be clinically cured of leukemia.