Paraneoplastic necrotizing myelopathy

Clinical features

This is a rare neurological paraneoplastic syndrome of unknown aetiology. It can only be accepted as such, if all other possible factors as radiation damage, chemotherapy and intramedullary carcinoma metastasis can be ruled out. This syndrome has been described in solid tumors and in hematological malignancies. Necrotizing myelopathy has been observed also in non-cancer patients.

The clinical features are those of a spinal cord lesion, which may develop either insidiously or rapidly. Depending on the extent of necrosis paraparesis or paraplegia progress into a complete transverse lesion. Mancall and Rosales (1962) described two cases and analyzed nine reports from literature (not all of them fit the definition, due to methodological reasons). Most reports consist of single or few cases. Diagnosis is best obtained with MRI scanning, showing the necrosis and, in the acute stage, edema. (figure 1)

Figure 1: MR image of a necrotizing myelopathy (a) and neuropathological correlation (b,c). (courtesy of Dr. W. Grisold)

Two neuropathological patterns have been described:

• A massive necrosis of the thoracic cord presenting as a transverse lesion.
• Patchy multifocal necrosis scattered through segments of the spinal cord, affecting predominately white matter. (figure 1 and 2)
  

Figure 2: Distribution of necrosis and demyelination in a case of paraneoplastic transverse myelopathy. The maximum of necrosis is at T 8 level. Additional to demyelination in upper and lower parts of the spinal cord, a column-like necrosis extends caudally (T 12). (courtesy of Dr. W. Grisold)

The primary differential diagnosis in a cancer patient is spinal cord compression. The ascending subacute evolution can resemble rare cases of meningeal carcinomatosis or ascending polyradiculopathy (as in polyradiculitis). Intramedullary metastasis, although very rare, and the possible influence of prior treatment with focal radiation (radiation myelopathy), intrathecal chemotherapy (MTX, AraC), or a combination must be considered. Viral infections also have been described as cause of the syndrome.

Associated antibodies: there is no association with onconeural antibodies.

Treatment: no known treatment

Selected references:

1. Gieron M.A., Margraf L.R., Korthals J.K., et al. Progressive necrotizing myelopathy associated with leukaemia: clinical, pathologic, and MRI correlation. J Child Neurol 1987; 2: 44-9.

2. Grignani G., Gobbi P.G., Piccolo G., et al.Progressive necrotic myelopathy as a paraneoplastic syndrome: report of a case and some pathogenetic considerations. J Intern.Med, 1992; 231: 81-5.

3. Grisold W., Lutz D.,Wolf D.: Necrotizing myelopathy associated with acute lymphoblastic leukemia. Acta Neuropathol (Berl) 1980; 49,231-5.

4. Iwamasa T., Utsumi Y., Sakuda H., et al:Two cases of necrotizing myelopathy associated with malignancy caused by herpes simplex virus type 2.Acta Neuropathol.(Berl), 1989; 78: 252-7.

5. Mancall E.L., Rosales R.K.: Acute ascending necrotizing myelopathy associated with visceral carcinoma. Brain 1965;87:639-56.

6. Ojeda V.J.: Necrotizing myelopathy associated with malignancy. A clinicopathologic study of two cases and literature review. Cancer 1984; 53: 1115-23.

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