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1 Definition

This is a condition of edema of the spinal cord developing after vaccination with diphtheria-tetanus-acellular pertussis (DTaP) vaccination as evidenced by neurological exam and MRI imaging.

2 Incidence

The case report by Riel-Romero seems to be the only one developing acute transverse myelitis after DTap 17 days earlier, although the child had two previous vaccinations (DTaP) without reaction. Kulenkampff et al. described a case of an infant, 6-month-old, who developed flaccid quadraparesis with respiratory compromise 17 days after DTaP vaccination (1974). Other cases of acute transverse myelitis (ATM) in childhood were reported due to hepatitis B vaccination (Fonseca et al. 2003; Iniguez et al. 2000). A case of Japanese B encephalitis vaccine was followed by transverse myelitis (Matsui et al. 2002). Whittle and Robertson reported a case of a 7-month-old infant who developed flaccid paraplegia after receiving DT inoculation and oral polio vaccine (1977). Zanoni et al. in 2002, reported a 15-month-old girl who developed ATM 21 days after receiving measles-mumps-rubella vaccination; similarly, Lim et al. 2004 with a 9-year-old girl who developed ATM 16 days post measles and rubella vaccination.

3 Etiology

The cause of developing ATM after DTaP vaccination is not quite clear:

  • Riel-Romero's hypothesis is that the infant had acute respiratory infection 2 weeks before the onset of the myelitis, suggesting a viral agent. In an analysis of 33 patients of ATM post vaccination the author found, including adults, that 45 % had a preceding infection, most of them were respiratory with 73 %, gastrointestinal infection was 13 and 13 % had flu-like symptoms (Jeffery et al. 1993).

  • The offending agent triggers an autoimmune reaction to the myelin sheath of the central nervous system (CNS), mainly to the spinal cord (Kerr and Ayetey 2002). The risk of ATM as an autoimmune response may depend on genetically predisposed child.

  • Myelitis may have developed as an incidental condition.

4 Pathology

There is no pathological data in these cases; however, in hepatitis B vaccination it shows the inflammatory reaction, edema, and demyelination (Sindern et al. 2001). The disease seems to be self and time limited illness and is generally monophasic. Improvement occurs in 2–12 weeks after maximal development, but the faster the development the worse the prognosis is. Resolution occurs especially after steroid therapy; however, a scar is formed and motor recovery is minimal.

5 Clinical Picture

(From Riel-Romero 2006) An infant 7-months-old was admitted for leg weakness 17 days after DTaP vaccination. He received two injections without problems. He developed priapism and dribbling of urine. He had constipation previously, which was resolved with fleet enema. His past history was negative except for acute upper respiratory infection before admission. Examination of his systems was unremarkable. The neurological exam showed flaccid paraplegia. Noxious stimuli of the legs gave triple flexion response (no anesthesia). The lower limbs tendon reflexes were +1.

6 Diagnosis

Diagnosis is based on:

  1. 1.

    Clinical picture

  2. 2.

    Laboratory tests

    1. (a)

      Blood count shows leucocytosis 16,000 and poly 67 %

    2. (b)

      CSF showed 2 RBC/uL and 7 WBC/uL, 4 lyphocytes, and 3 monocytes

    3. (c)

      Myelin basic protein was increased at 4.9 mg/dL (normal: 0–2.3)

    4. (d)

      Other tests were within normal including IgG, and tests for viruses and bacteria were negative.

  3. 3.

    MRI: brain was negative. Spine showed diffused edema of the spinal cord with increased T2 signal within the spinal cord from C3 to T6, dorsal and ventral veins were normal and no evidence of AVM. After gadolinium injection there was a faint and patchy encroachment of the spinal cord.

The diagnosis was, from the clinical and imaging pictures, ATM 17 days after DTaP vaccination.

7 Management

Riel-Romero gave the following treatment:

  • IV methylpredinsolone: 1.5 mgm/kg/day for 7 days followed by

  • Oral predinsolone tapering at 1 mg/kg/day for 2 weeks

  • Acyclovir pending on the result of CSF, HSV-DNA PCR.

  • Bowel and bladder care

  • Rehabilitation program

Within a week priapism resolved and the lower extremities gained tone and there was a clonus. At three weeks the constipation resolved and urine stream was established but signs of pyramidal involvement persisted. Three months later MRI showed resolution of the cord edema and its signals but showed reduction of the cord caliber. Ten months after the onset spastic paraplegia persisted.