2013
DOI: 10.3389/fneur.2013.00197
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Autopsy Proven Peripheral Nervous System Neurolymphomatosis Despite Negative Bilateral Sural Nerve Biopsy

Abstract: Neurolymphomatosis (NL) refers to a lymphomatous infiltration of peripheral nerves associated with central nervous system or systemic lymphoma, or alternatively, neurodiagnostic evidence of nerve enhancement and/or enlargement beyond the dural sleeve in the setting of primary central nervous system lymphoma or systemic lymphoma. NL is a rare complication of systemic cancer with heterogeneous clinical presentations and an elusive diagnosis. Diagnosis usually requires the demonstration of infiltrating malignant … Show more

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Cited by 11 publications
(14 citation statements)
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“…About 90% of cases of neurolymphomatosis are DLBCL (1). Similar to previous cases of neurolymphomatosis in the cauda equina (4), the present case also showed asymmetrical polyneuropathy without pain (Figure B-E). Gd-enhanced MRI and FDG-PET are useful for detecting small or insidious malignancies, but the diagnosis is still difficult in some cases (5).…”
Section: Discussionsupporting
confidence: 90%
“…About 90% of cases of neurolymphomatosis are DLBCL (1). Similar to previous cases of neurolymphomatosis in the cauda equina (4), the present case also showed asymmetrical polyneuropathy without pain (Figure B-E). Gd-enhanced MRI and FDG-PET are useful for detecting small or insidious malignancies, but the diagnosis is still difficult in some cases (5).…”
Section: Discussionsupporting
confidence: 90%
“…In one study where diagnostic uncertainty persisted, nerve biopsy (often guided by imaging findings) was performed with 88% sensitivity for neurolymphomatosis 9 . The imperfect sensitivity of nerve biopsy owes to the usually patchy distribution of malignant cells 39 . In some patients with hematological malignancies that are latent or in remission, nerve biopsy may be the only way to link the malignancy with the neuropathy 40 .…”
Section: Methodsmentioning
confidence: 99%
“… 9 The imperfect sensitivity of nerve biopsy owes to the usually patchy distribution of malignant cells. 39 In some patients with hematological malignancies that are latent or in remission, nerve biopsy may be the only way to link the malignancy with the neuropathy. 40 Polymerase chain reaction (PCR) of the lymphoid infiltrate in nerve biopsies can prove monoclonality and help to distinguish between malignant and inflammatory infiltrates.…”
Section: Methodsmentioning
confidence: 99%
“…However, it is invasive and false negative results occur due to sampling errors. Ramirez-Zamora [ 9 ] reported 1 case of NL with negative nerve biopsy that was later confirmed by autopsy. NL is easily misdiagnosed or missed due to its rarity and complex clinical manifestations.…”
Section: Discussionmentioning
confidence: 99%