Nervous System Lymphoma

BackgroundBackground Hodgkin’s diseaseHodgkin’s disease

Rarely involves the nervous systemRarely involves the nervous system

Non-Hodgkin’s lymphomaNon-Hodgkin’s lymphoma Involves nervous system in 10% of casesInvolves nervous system in 10% of cases Less differentiated, diffuse forms and bone marrow Less differentiated, diffuse forms and bone marrow

involvement increase risk of nervous system spreadinvolvement increase risk of nervous system spread Leptomeninges (6-8% of cases)Leptomeninges (6-8% of cases) Epidural space (5% of cases)Epidural space (5% of cases)

Neurolymphomatosis

BackgroundBackground 1907- Marek1907- Marek

Disease of Orpington roostersDisease of Orpington roosters• ““Peripheral nerves infiltrated by lymphocytes”Peripheral nerves infiltrated by lymphocytes”

1934- Lhermitte and Trelles1934- Lhermitte and Trelles ““human peripheral neurolymphomatosis”human peripheral neurolymphomatosis”

1992- Diaz-Arrastia1992- Diaz-Arrastia Reviewed literature on 39 casesReviewed literature on 39 cases

Neurolymphomatosis

BackgroundBackground Rare complication of NHLRare complication of NHL At least 50% occur in context of systemic NHLAt least 50% occur in context of systemic NHL Most cases are associated with leptomeningeal Most cases are associated with leptomeningeal

involvementinvolvement Infiltration of spinal nerve roots, dorsal root Infiltration of spinal nerve roots, dorsal root

ganglia and peripheral nervesganglia and peripheral nerves

Neurolymphomatosis

Clinical featuresClinical features Diaz-Arrastia- 39 casesDiaz-Arrastia- 39 cases

Neurology 1992; 42: 1136-1141Neurology 1992; 42: 1136-1141

HistoryHistory Subacute course over weeks most commonSubacute course over weeks most common Acute/rapid course in ~10% of casesAcute/rapid course in ~10% of cases Pain is commonPain is common Ante-mortem diagnosis in 35% of casesAnte-mortem diagnosis in 35% of cases

Neurolymphomatosis

Clinical SyndromeClinical Syndrome ExaminationExamination

Lower motor neuron syndromeLower motor neuron syndrome• Weakness, sensory loss, areflexiaWeakness, sensory loss, areflexia

OtherOther• Cranial neuropathy (18)Cranial neuropathy (18)

• Bladder or bowel incontinence (10)Bladder or bowel incontinence (10)

• Ataxia (7)Ataxia (7)

• Mental changes (5)Mental changes (5)

Neurolymphomatosis

Diagnostic testsDiagnostic tests CSFCSF

Increased protein (73%)Increased protein (73%) Lymphocytic pleocytosis (70%)Lymphocytic pleocytosis (70%) Positive cytopathology (4/7 assessed)Positive cytopathology (4/7 assessed)

ElectrophysiologyElectrophysiology Sensorimotor axonal neuropathy- most commonSensorimotor axonal neuropathy- most common Mixed axonal/demyelinating neuropathyMixed axonal/demyelinating neuropathy

Neurolymphomatosis

NeuroimagingNeuroimaging Thick, enhancing nerve roots, nervesThick, enhancing nerve roots, nerves

Nerve biopsyNerve biopsy Axonal degenerationAxonal degeneration DemyelinatingDemyelinating MixedMixed

Neurolymphomatosis

TreatmentTreatment Concomitant involvement of subarachnoid Concomitant involvement of subarachnoid

space in 2/3 cases at autopsy- need drugs which space in 2/3 cases at autopsy- need drugs which penetrate blood brain barrierpenetrate blood brain barrier

AnecdotalAnecdotal Surgery/BiopsySurgery/Biopsy RadiationRadiation ChemotherapyChemotherapy

• Systemic plus/minus intrathecalSystemic plus/minus intrathecal

Nervous System Lymphoma

BackgroundBackground CNS Lymphoma (Primary or Secondary)CNS Lymphoma (Primary or Secondary)

BrainBrain• Most are primaryMost are primary

LeptomeningealLeptomeningeal• Most are secondaryMost are secondary

Spinal CordSpinal Cord• Most secondary, from epidural compressionMost secondary, from epidural compression

Cranial nerveCranial nerve IntravascularIntravascular

Nervous System Lymphoma

BackgroundBackground PNS LymphomaPNS Lymphoma

Nerve rootNerve root PlexusPlexus Peripheral NervePeripheral Nerve

CNS + PNSCNS + PNS NeurolymphomatosisNeurolymphomatosis

• Relevant to our case reportRelevant to our case report• ““Symptoms and signs of peripheral neuropathy that are confirmed by Symptoms and signs of peripheral neuropathy that are confirmed by

histopathologic evidence of lymphomatous infiltration of the nerves as histopathologic evidence of lymphomatous infiltration of the nerves as seen by nerve biopsy or autopsy”seen by nerve biopsy or autopsy”

Neurolymphomatosis

Neurolymphomatosis

Differential Diagnosis Differential Diagnosis Mononeuropathies (nerves, roots, plexuses)Mononeuropathies (nerves, roots, plexuses)

Compression (tumor, vertebral collapse, hematoma)Compression (tumor, vertebral collapse, hematoma) Pressure palsies (loss of subcutaneous tissue)Pressure palsies (loss of subcutaneous tissue) RadiationRadiation Herpes zosterHerpes zoster Brachial neuritisBrachial neuritis

Neurolymphomatosis

Differential DiagnosisDifferential Diagnosis PolyradiculoneuropathiesPolyradiculoneuropathies

Paraneoplastic (sensory, motor, both)Paraneoplastic (sensory, motor, both) Guillain-Barre SyndromeGuillain-Barre Syndrome Chemotherapy (Vinca alkaloids)Chemotherapy (Vinca alkaloids) NutritionalNutritional Herpes zosterHerpes zoster AmyloidAmyloid