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  • Many asymmetric sensory-motor neuropathies are treatable.
  • They are a major indication for biopsies of nerve and muscle.
CMAP from Falck

Mononeuritis Multiplex
Other asymmetric neuropathies Cytopathies

Also see: Asymmetric myopathies

Lymphoma 5

Lymphoma & Disease subtypes
  Anaplastic large cell (ALCL)
  Angiotropic (Intravascular)
  Ataxia telangectasia
  Castleman disease
  CNS infiltration
  Hemophagocytic lymphohistiocytosis
  Lymphoepithelial thymoma
  Lymphomatoid granulomatosis
  Monoclonal gammopathies (MGUS)
  PNS (Nerve) infiltration
    Metastatic (Neurolymphomatosis)
  Waldenström's macroglobulinemia
  Also see
    Diffuse infiltrative lymphocytosis syndrome (DILS)
Lymphoma-related NM syndromes
  Paraneoplastic syndromes
    Sensory (Hu)
    Cerebellar: Tr; mGluR1
    Myelopathy, Necrotic
  Immune, Other nerve
    IgM Antibody syndromes
      MAG; GM1; Sulfatide; GD1b
    Weak association
      CIDP (Varied lymphoma types)
      GBS (Hodgkin & CLL)
  Muscle & NMJ
    Myasthenia gravis
    Inflammatory myopathy
  Toxic: Chemotherapy

Lymphoma Features Lymphoma Types
Infiltration of Nerve (Neurolymphomatosis) 8
Infiltrates primarily in spinal cord & CNS 1
  • Angiotropic large B-cell lymphoma
      (Malignant angioendotheliomatosis; Intravascular B-cell lymphoma)
    • General: Proliferation of neoplastic lymphoid cells within blood vessels
    • Onset: Especially 7th & 8th decade
    • Neurological features
      • Presenting feature in 54%
      • CNS lesions: Multifocal; "Stroke-like"
        • Encephalopathy: Sub-acute; Dementia; Coma
        • Cortical lesions: Multi-Focal; Hemiparesis
        • Myelopathy
          • Transverse or Diffuse
          • Clinical
            • Paraparesis
            • Sensory loss
            • Urinary dysfunction
          • Pathology: Multifocal infarction
        • Deafness
      • Peripheral nerve involvement: Occasional
        • Mononeuritis: Sudden onset
        • Mononeuritis multiplex
        • Cranial nerves
          • Most common: VII & Hearing loss
          • Also EOM, Trigeminal
        • Radiculopathy: Pain; Sensory loss; Weakness
    • Organ involvement
      • Eye: Visual loss; Papilledema; Retinal & Choroid vessels involved
      • Skin
        • Most common; Rash - Macular
        • Other: Papular, Nodular, Plaquelike, or Pigmented
      • Bone marrow: with More diffuse lymphoma
      • Muscle: Asymptomatic; Intravascular
      • Adrenal: Enlargement
      • Renal failure
      • Liver failure
      • Respiratory failure
      • General: Fever, Malaise
    • Disease course
      • Untreated patients: High mortality rate; Up to 80% at 1 year
      • Treatment: May be curable with early chemotherapy (40%)
    • Histopathology
      • Neoplasm features
        • Noncohesive masses of large, pleomorphic lymphoid cells
        • Location: Capillaries, venules & small arterioles
        • Neoplastic cell accumulations
          • May occlude vessels
          • Rarely extravasate into the neighboring tissues
      • Cell features
        • Nuclei
          • Contours: Irregular
          • Chromatin: Coarse
          • Nucleoli: Two or Multiple
        • Cytoplasm: Moderate amounts; Basophilic; Vacuolated
        • Antigens
          • Common: bcl-2 & CD-20 proteins
          • Other: CD10, bcl-6 & CD5 few
      • Lymphoma cell locations
        • Present in small vessels in:
            Skin; Brain; Kidney; Spleen; Liver; Bone marrow; Heart; Muscle
        • No mass
      • Diagnosis may be made on skin, muscle or nerve biopsy
    • Laboratory
      • Hematologic
        • Anemia: Mild; Hemolytic
        • Peripheral blood: Pancytopenia with leukoerythroblastic picture
      • Erythrocyte sedimentation rate: High
      • Serum lactate dehydrogenase: Markedly elevated
      • Coagulopathy: Occasional patients; Thrombocytopenia pancytopenia
      • CSF: Normal
      • LDH: Very high
      • Tissue biopsy: CD20+ cells in vascular lumen and walls

B-cell Lymphoma

Spinal cord (Thoracic)
  Increased T2 signal

Hodgkins Lymphoma
Non-Hodgkins Lymphoma
Lymphomatoid granulomatosis
Chronic lymphocytic leukemia (CLL/SLL)
Diffuse, Large B-cell lymphoma (DLBCL)

Lymphoma-related syndromes: Other

Cholesterol emboli

Hemophilia A & B

Idiopathic Thrombocytopenic Purpura 6

Wartenberg's Migrant Sensory Neuritis 2

Sickle Cell Disease 4

Return to Polyneuropathy Index

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2. Muscle Nerve 2001;24:438-443
3. J Clin Neuromusc Dis 2001;3:63-69
4. Ann Neurol 2002;51:543–552, Handb Clin Neurol 2014;120:1015-1025
5. Muscle Nerve 2005;31:301–313, J Neurol Sci 2015; Jun 3
6. J Peripheral Nervous System 2007;12:286–289
7. European Journal of Neurology 2009;16:814–818
8. J Peripher Nerv Syst 2018 Dec 16