CASE 4

Clinical Presentation

A 9-year-old child presents with rapidly developing deficit of all four limbs.

Figure 4A

Radiologic Findings

The sagittal T1-weighted image (Fig. 4A1) demonstrates fusiform enlargement of the cervical spinal cord, with patent surrounding CSF spaces, however. The signal of the cord is homogeneous, but with occasional cavitation, necrosis, or hemorrhage. Sagittal T2-weighted imaging (Fig. 4A2) demonstrates a homogeneous hypersignal extending from C3 to T1, and the axial cut (Fig. 4A3) shows that the abnormality covers both sides of the cord, fitting the anatomic definition of transverse myelitis. Infusion of contrast agent (Figs. 4A4 and 4A5) demonstrates a faint enhancement only, in the periphery of the lesion on the left side.

Diagnosis

Acute transverse myelitis (ATM)

Differential Diagnosis

• Spinal cord tumor: more heterogeneous mass, occupying the whole section of the cord often associated with extended edema, cavitation and/or syringomyelia, hemorrhage, disappearance of the perimedullary space with occasional enlargement of the spinal canal, more nodular enhancements
  
• Spinal cord compression: from spinal, extradural, dural, or intradural infections or tumors
  
• Guillain-Barré syndrome (GBS, ascending polyradiculoneuritis), featuring an ascending flaccid para- then tetraplegia, with intense muscular pains, and characteristically high protein level but no cellularity in the CSF
  
• Multiple sclerosis (MS): chronic demyelinating disease. The first attack of the disease may present like ATM. The MRI appearance of the lesions of the spinal cord helps confirm the diagnosis (see Table 4-1), together with the laboratory assessment of the CSF (oligoclonal IgG bands present in MS, consistently absent in ATM). The brain as a rule is also affected. Devic’s disease (neuromyelitis optica) is assumed to be a variant of MS, affecting both the cord and the optic nerves.
  
• Necrotizing myelitis: direct infectious necrosis (usually viral) of the cord. Poliomyelitis used to be a major problem in infected areas, but it is disappearing with worldwide vaccination.
  
• Other myelitis: systemic lupus erythematosus (SLE), antiphospholipids antibody syndrome, other connective tissue diseases, paraneoplastic syndromes
  
• Rarely: hemorrhage (arteriovenous malformations, cavernomas), ischemia (usually iatrogenic)

Discussion

Background

ATM belongs, together with acute disseminated encephalomyelitis (ADEM) and GBS, to the group of postinfectious, immune-related acute demyelinating disorders. The typical clinical course starts with an acute, often seasonal infection, which is followed after a few days by a sudden neurological impairment due to an acute episode of demyelination of the nervous system. Most commonly, the entire CNS is involved with disseminated areas of demyelination, making up the ADEM. Less frequently, only the spinal cord is involved, and the patient then presents with ATM. Even less frequent, only the nerve roots and distal nerves are affected, usually in an ascending manner, which defines the GBS. Acute demyelination is provoked by the antibodies induced by the initial infection, where the myelin has an antigenicity similar to that of the infectious agent. In acute

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