Neuromuscular

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ASYMMETRIC NEUROPATHIES

  • 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
  CLL
  CNS infiltration
  DLBCL
  Hemophagocytic lymphohistiocytosis
  Hodgkin
  Non-Hodgkin
  Lymphoepithelial thymoma
  Lymphomatoid granulomatosis
  Monoclonal gammopathies (MGUS)
  PNS (Nerve) infiltration
    Metastatic (Neurolymphomatosis)
    Local
  Waldenström's macroglobulinemia
  Also see
    Diffuse infiltrative lymphocytosis syndrome (DILS)
Lymphoma-related NM syndromes
  Cryoglobulinemia
  Paraneoplastic syndromes
    Motor
    Sensory (Hu)
    Cerebellar: Tr; mGluR1
    Myelopathy, Necrotic
  Immune, Other nerve
    Amyloid
    IgM Antibody syndromes
      MAG; GM1; Sulfatide; GD1b
    POEMS
    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
  Intravascular

Spinal cord (Thoracic)
  Enlarged
  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

References
1. Clinical Neurology and Neurosurgery 2000;101:275-279; JNNP 2001;71: 696-703
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

4/2/2020