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Subacute combined degeneration of the spinal cord: a brief review.

Subacute combined degeneration of the spinal cord: a brief review. SCD: Overview. History of SCD Pathophysiology and Pathology Imaging Findings. SCD: History. aka Lichtheim’s Disease Ludwig Lichtheim described the disease in 1887

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Subacute combined degeneration of the spinal cord: a brief review.

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  1. Subacute combined degeneration of the spinal cord: a brief review.

  2. SCD: Overview • History of SCD • Pathophysiology and Pathology • Imaging Findings

  3. SCD: History • aka Lichtheim’s Disease • Ludwig Lichtheim described the disease in 1887 • Recognized that pathology of cord disease was associated with pernicious anemia and is different from tabes dorsalis.

  4. SCD: History • aka Putnam-Dana disease • Putnam: series of 8 patients describing the disease • Dana: called it “combined” because it involves the dorsal AND lateral columns

  5. SCD: Clinical Findings • Insidious subacute onset of parasthesias, sensory ataxia, hyperreflexia, & + Babinski • symmetric and progresses distal to proximal • megaloblastic anemia and low B12 • Severe disability in weeks to months

  6. SCD: Pathophysiology • B12 Deficiency • Accumulated methylmalonic acid believed to cause myelin toxicity • Possible upregulation of tumor necrosis factor and downregulation of epidermal growth factor • Nitrous oxide • in susceptible patients, B12 is inactivated

  7. SCD: Pathophysiology • Causes of B12 deficiency • GI malabsorption - pernicious anemia (lack of intrinsic factor), post-gastrectomy, Crohn disease, and celiac disease • Nutritional deficiency

  8. SCD: Pathology • Loss of myelin in the spinal cord white matter tracts: • Dorsal columns • Lateral columns • Ventral columns Typical pattern of involvement

  9. AIDS Myelopathy Similar distribution of myelopathy to SCD • Pathophysiology is uncertain • Theories include cytokines secretion from HIV infected monocytes, neurotoxic HIV proteins, inability to use B12 appropriately

  10. SCD: Imaging Findings • MRI shows, high T2 signal intensity, involving the cervical and/or the thoracic spinal cord in: • Dorsal, lateral and ventral white matter • Cord diameter is normal • No Gd enhancement

  11. SCD: Imaging Findings

  12. SCD: Imaging Findings Note involvement of thoracic and cervical cord.

  13. SCD: Treatment • In the setting of B12 deficiency, treat with B12. • Cannot recover lost axons • Avoid nitrous oxide anesthesia

  14. References • Pema et al. Myelopathy Caused by Nitous Oxide Toxicity. AJNR, 1998 19:894-896. • Ravina et al. MR findings in subacute combined degeneration of the spinal cord: a case of reversible cervical myelopathy. AJR, 2000 Mar; 174(3) 863-865. • Yamada et al. A Case of Subacute Combined Degeneration. Neuroradiology , 1998 40: 398-400.

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