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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. [ABSTRACT FROM AUTHOR]

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, [ABSTRACT FROM AUTHOR]

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. • Neuroleukemiosis describes the leukemic infiltration of peripheral nerves. • Presentations include painless mono-neuropathies, formations of a chloroma and painful radiculoplexopathies. • Tissue sampling through nerve biopsy remains the gold standard for diagnosis though yield can be limited. • Neuroleukemiosis tends to portend a poor prognosis. [ABSTRACT FROM AUTHOR]

Objective: In neurolymphomatosis (NL), the affected nerves are typically described to be enlarged and hyperintense on T2W MR sequences and to avidly enhance on gadolinium-enhanced T1WI. This pattern is highly non-specific. We recently became aware of a 'tumefactive pattern' of NL, neuroleukemiosis (NLK) and neuroplasmacytoma (NPLC), which we believe is exclusive to hematologic diseases affecting peripheral nerves. Materials and methods: We defined a 'tumefactive' appearance as complex, fusiform, hyperintense on T2WI, circumferential tumor masses encasing the involved peripheral nerves. The nerves appear to be infiltrated by the tumor. Both structures show varying levels of homogenous enhancement. We reviewed our series of 52 cases of NL in search of this pattern; two extra outside cases of NL, three cases of NLK, and one case of NPLC were added to the series. Results: We identified 20 tumefactive lesions in 18 patients (14 NL, three NLK, one NPLC). The brachial plexus ( n = 7) was most commonly affected, followed by the sciatic nerve ( n = 6) and lumbosacral plexus ( n = 3). Four patients had involvement of other nerves. All were proven by biopsy: the diagnosis was high-grade lymphoma ( n = 12), low-grade lymphoma ( n = 3), acute leukemia ( n = 2), and plasmacytoma ( n = 1). Conclusions: We present a new imaging pattern of 'tumefactive' neurolymphomatosis, neuroleukemiosis, or neuroplasmacytoma in a series of 18 cases. We believe this pattern is associated with hematologic diseases directly involving the peripheral nerves. Knowledge of this association can provide a clue to clinicians in establishing the correct diagnosis. Bearing in mind that tumefactive NL, NLK, and NPLC is a newly introduced imaging pattern, we still recommend to biopsy patients with suspicion of a malignancy. [ABSTRACT FROM AUTHOR]

ABSTRACT 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. Muscle Nerve 51: 290-293, 2015 [ABSTRACT FROM AUTHOR]

Leukemia may initially present as a peripheral neuropathy, leading to a delay in diagnosis. Leukemic infiltration of peripheral nerves, or neuroleukemiosis (NL), is exceedingly rare, with no established diagnostic or therapeutic guidelines. Five cases are presented. All patients were men with a median age of 68 years (range 46-72). Three patients had acute myeloid leukemia (AML) and two had chronic lymphocytic leukemia (CLL). In two patients, leukemia presented with peripheral nerve involvement and both were found to have positive cerebrospinal fluid (CSF) cytology, making the diagnosis AML, despite negative bone marrow and peripheral smear. All patients had painful, progressive, motor and sensory deficits. Clinical patterns were mononeuropathy ( n =1), multiple mononeuropathies ( n =1) and plexopathy ( n =3). Magnetic resonance imaging (MRI) detected mass lesions in 4/5 cases, with avid fluorodeoxyglucose (FDG) uptake on positron emission tomography (PET) useful in all of these for following clinical disease progression. Three cases of nerve biopsy were performed, two of which were diagnostic of leukemic infiltration. Radiation treatment rapidly relieved pain in patients with mass lesions, in combination with chemotherapy. Four patients had disease relapse, four systemic and one also in peripheral nerves. These cases are discussed in the context of the broader literature. [ABSTRACT FROM AUTHOR]