Neurolymphomatosis in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma mimicking Hansen's disease (original) (raw)
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Frontiers in Neurology, 2013
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 lymphocytes in the peripheral nerve. Infiltration of brain parenchyma, meninges or Virchow-Robin spaces is characteristic of systemic disease at autopsy. We describe a patient presenting with biopsy negative NL affecting exclusively the peripheral nervous system at autopsy.
Neurolymphomatosis of the Sciatic and Tibial Nerves
Journal of the College of Physicians and Surgeons Pakistan
Neurolymphomatosis (NL) is an uncommon clinical condition, characterised by lymphomatous infiltration of the central and/or peripheral nervous system. Most often it is caused by B-cell non-Hodgkin's lymphoma (NHL). Clinically, patients usually present with neuropathy involving the nerve roots, plexuses, peripheral or cranial nerves. NL usually occurs as a complication of prior lymphoma, but it can also present in the form of relapsed lymphoma. It is important to diagnose and start early treatment in all cases of nodal or visceral (including neural) lymphoma with chemo and/or radiation therapy. The PET-CT and MRI can help in making diagnosis. We are presenting a case of 28-year male patient, diagnosed as diffuse large B-cell lymphoma on the background of follicular lymphoma, which initially responded to treatment but then presented with NL, based on clinical history and radiological findings which were confirmed by histopathology.
Neuropathies associated with lymphoma†
Neuro-Oncology Practice, 2015
Neuropathy occurs with various manifestations as a consequence of lymphoma, and an understanding of the etiology is necessary for proper treatment. Advances in medical imaging have improved the detection of peripheral nerve involvement in lymphoma, yet tissue diagnosis is often equally important. The neoplastic involvement of the peripheral nervous system (PNS) in lymphoma can occur within the cerebrospinal fluid (CSF), inside the dura, or outside of the CSF space, affecting nerve root plexuses and peripheral nerves. The infiltration of either cranial or peripheral nerves in lymphoma is termed neurolymphomatosis (NL). These infiltrations can occur as mononeuropathy, multifocal neuropathy, symmetric neuropathies, or plexopathies. In rare cases, intravascular lymphoma (IL) can affect the PNS and an even rarer condition is the combination of NL and IL. Immune-mediated and paraneoplastic neuropathies are important considerations when treating patients with lymphoma. Demyelinating neurop...
Frequently Thickened Nerve in Hansen’s Disease
Journal of Pharmaceutical Research International
Background: Leprosy by definition is a chronic granulomatous infection of the skin and superficial nerves in the skin caused by Mycobacterium leprae and Mycobacterium lepromatosis. [1] It most commonly involves the nose, eyes, throat, and sometimes the testicles. The bacilli are most frequently transmitted via droplets, from the nose during close contact with untreated cases of leprosy. Leprosy was first mentioned in 700BC. Leprosy is common in tropical and subtropical Asia, some Pacific countries, Africa, and South America [2]. It is not known how leprosy is transmitted. Although skin lesions and nerve thickening manifest together, they also appear as separate entities. This study aims to find out the frequently thickened nerves in different spectrums of Hansen’s disease. Objectives: To determine the frequently thickened nerves in different spectrums of Hansen’s disease. Methods: This descriptive study was conducted in Chettinad Hospital and the Research Institute from June 2021 to...
Skeletal Radiology, 2015
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 Btumefactive 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 Btumefactive^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.
Clinicopathological features of neuropathy associated with lymphoma
Brain, 2013
Lymphoma causes various neurological manifestations that might affect any part of the nervous system and occur at any stage of the disease. The peripheral nervous system is one of the major constituents of the neurological involvement of lymphoma. In this study we characterized the clinical, electrophysiological and histopathological features of 32 patients with neuropathy associated with non-Hodgkin's lymphoma that were unrelated to complications resulting from treatment for lymphoma. Nine patients had pathologically-proven neurolymphomatosis with direct invasion of lymphoma cells into the peripheral nervous system. These patients showed lymphomatous cell invasion that was more prominent in the proximal portions of the nerve trunk and that induced demyelination without macrophage invasion and subsequent axonal degeneration in the portion distal from the demyelination site. Six other patients were also considered to have neurolymphomatosis because these patients showed positive signals along the peripheral nerve on fluorodeoxyglucose positron emission tomography imaging. Spontaneous pain can significantly disrupt daily activities, as frequently reported in patients diagnosed with neurolymphomatosis. In contrast, five patients were considered to have paraneoplastic neuropathy because primary peripheral nerve lesions were observed without the invasion of lymphomatous cells, with three patients showing features compatible with chronic inflammatory demyelinating polyneuropathy, one patient showing sensory ganglionopathy, and one patient showing vasculitic neuropathy. Of the other 12 patients, 10 presented with multiple mononeuropathies. These patients showed clinical and electrophysiological features similar to those of neurolymphomatosis rather than paraneoplastic neuropathy. Electrophysiological findings suggestive of demyelination were frequently observed, even in patients with neurolymphomatosis. Eleven of the 32 patients, including five patients with neurolymphomatosis, fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society electrodiagnostic criteria of definite chronic inflammatory demyelinating polyneuropathy. Some of these patients, even those with neurolymphomatosis, responded initially to immunomodulatory treatments, including the administration of intravenous immunoglobulin and steroids. Patients with lymphoma exhibit various neuropathic patterns, but neurolymphomatosis is the major cause of
Neurolymphomatosis: A Rare Cause of Multiple Mononeuropathy
Neurolymphomatosis, defined as invasion of cranial nerves and peripheral nerve roots, plexus or nerves by Non Hodgkin’s Lymphoma is a very rare clinical entity. We describe a case of 69 years old gentleman, who presented to us with asymmetric, painful sensorimotor polyneuropathy. He was admitted with 2 months history of dry cough, constitutional symptoms, paraesthesias on right side of face along with painful asymmetrical quadriparesis. Nerve conduction studies were suggestive of asymmetrical sensorimotor axonal and demyelinating neuropathy. Cerebrospinal fluid analysis revealed mild pleocytosis with raised protein. FDG-PET showed intense uptake in both adrenals, abdominal lymph nodes, sacral nerve roots and brachial plexus. Fine needle aspiration cytology of adrenal mass revealed evidence of diffuse large B cell Non Hodgkin’s Lymphoma. As patient succumbed to illness, an autopsy was done, which revealed diffuse large B cell lymphoma involving adrenals, brachial and lumbosacral plexuses. Our report provides important insights into a rare cause of painful demyelinating multiple mononeuropathy and emphasises on increasing role and diagnostic utility of PET imaging in evaluation of patients presenting with multiple mononeuropathy, especially with regards to paraneoplastic or neoplastic causes such as lymphoma.
Sonographic characteristics of median nerve neuropathy in Hansen’s disease: a case-control study
Leprosy Review, 2021
Background and objectives High-resolution ultrasonography (HRUS) has become a vital imaging tool in the management of leprosy. This case-control study analyzed the sonographic data on median nerves in leprosy and healthy controls to identify the features characteristic of leprosy. Methods Newly diagnosed and treatment naïve Hansen's patients of both sexes and aged >16 years were included in the study. The control group included prospectively enrolled healthy subjects. Nerve conduction studies were performed in all. HRUS of the median nerve was performed using a Philips HD15 machine. HRUS parameters studied included cross-sectional area (CSA) of the nerve, fascicular architecture and abnormal blood flow (by colour Doppler). CSA was measured at the wrist (S1), 5 cm above the wrist (S2), 10 cm above the wrist (S3), mid-forearm (S4) and at the elbow (S5). Results 30 median nerves from 22 cases of leprosy and 30 nerves from 15 healthy subjects were compared. Among cases, 8 were borderline tuberculoid, 9 borderline lepromatous, 3 lepromatous leprosy, and 2 indeterminate leprosy. Abnormal motor (76.7%) and sensory conduction (96.7%) was noted in the patient group. The mean CSA was 18.5 mm 2 at S1, 20.3 mm 2 at S2, 14.1 mm 2 at S3, 9.1 mm 2 at S4, and 8.1 mm 2 at S5. The CSA at S1 and S2 were significantly higher compared to other sites. CSA values were significantly higher in patients compared to controls. The fascicular architecture was distorted in patients-grade II (33.3%), III (40.0%) and IV (20%), and hypervascularity was noted in 26.6%.
Processing of nerve biopsies: A practical guide for neuropathologists
Clinical Neuropathology, 2011
Nerve biopsy is a valuable tool in the diagnostic work-up of peripheral neuropathies. Currently, major indications include interstitial pathologies such as suspected vasculitis and amyloidosis, atypical cases of inflammatory neuropathy and the differential diagnosis of hereditary neuropathies that cannot be specified otherwise. However, surgical removal of a piece of nerve causes a sensory deficit and -in some cases -chronic pain. Therefore, a nerve biopsy is usually performed only when other clinical, laboratory and electrophysiological methods have failed to clarify the cause of disease. The neuropathological work-up should include at least paraffin and resin semithin histology using a panel of conventional and immunohistochemical stains. Cryostat section staining, teased fiber preparations, electron microscopy and molecular genetic analyses are potentially useful additional methods in a subset of cases. Being performed, processed and read by experienced physicians and technicians nerve biopsies can provide important information relevant for clinical management.