Age: 1.09
Sex: F
Vaccine: MMR
Symptoms: Hemiplegia, Hypokinesia, Laboratory test abnormal, Muscular weakness, Musculoskeletal stiffness
Clinical narrative: Information has been received from a consumer concerning her approximately 12 to 14 month old daughter who on 1/31/03 was vaccinated with a dose of MMR (lot # 640318/1085L). The mother reported that concomitant therapy included HIB conjugate vaccine (unspecified carrier) (lot # UA791AA) which the patient received on 7/7/03. Subsequently, the patient experienced neurological problems (reported by the mother as occurring around this time frame and reported by health professionals as occurring at about one month after vaccination, at 12 months of age and 13 months of age). The mother reported that they noticed something wrong with the patient; her left hand was curling and fisting, her left leg was weak and stiff and she was unable to stand or crawl. Subsequently, the child improved and was able to crawl, stand and walk however; by November/December 2003 the child had regressed again. The mother stated that her daughter had not yet been diagnosed. Per the mother, diagnostic testing included: an MRI (4 total; first on unspecified date, second on 9/3/03, third on 12/10/03 and fourth on 2/17/04), torch test for rubella antibody and physical and occupational therapy. She stated that on all tests taken were either normal or negative, except the torch test for rubella, which the mother reported was high at 3.08. Additional information was obtained from the patient's neurologist who reported that the child was vaccinated with either MMR  or rubella virus vaccine live at about 11 months of age. The physician noted that there were no concomitant medications. He reporter that about one month after vaccination, the child began to experience difficulty using and stiffness of her hands and legs. The physician reported that an MRI showed delayed myelination. He reported that the child had been diagnosed with brain white matter problems and leukodystrophy. The patient had a spinal tap (results not reported) which required half a day in the hospital. Per the physician, lab evaluations revealed a panel for antibodies was high for rubella. The child was currently receiving physical therapy and was improving. Additional information received from the nurse practitioner at the neurologist's office who reported that the patient had the spinal tap for diagnostic reasons. She reported that the patient was an extremely complicated patient, she had a long history of motor dysfunction and the neurologist questioned if the patient had alternating hemiplegia or an acute disseminating encephalomyelitis. She reported that the patient was OK until she was 13 months old. At 13 months of age the patient experienced difficulties with hand and leg movement. The patient had improved but continued to be under the neurologist's care. Additional information received from the neurologist's office in the form of medical records. On 12/10/03 an MRI of the head MR spectroscopy was done and compared with an MRI exam dated 2/17/03. The head MRI findings revealed there was subtle gyral asymetry between the right and left cerebral hemispheres particularly towards the vertex. The gyri on the right appeared fuller and the sulci smaller. The subcortical white matter along the right vertex also appeared slightly attenuated compared to the left. The ventricles demonstrated normal size and configuration. The midline structures including the corpus callosum, pituitary gland, brain stem, cerebellar tonsils and vermis were normally situated. The craniocervical junction was unremarkable. There was no evidence for mass or mass effect. There was a focal area of increased signal intensity adjacent to the right frontal horn within the right frontal lobe. This was nonspecific in appearance and could represent an area of ischemic gliosis. Also noted was an abnormally increased flair signal along the right motor strip. No other significant areas of abnormal signal intensity or pathologic enhancement were identified. The brain stem and posterior fossa structures appeared intact and unremarkable. No sells or parasellar lesions were identified. The upper portions of the vertebral arteries, the basilar artery, the petrous and cavernous portions of the internal carotid arteries and the vessels about the circle of Willis demonstrated normal flow voids indicating patency. Normal flow voids were present within the intracranial venous sinuses. The visualized paranasal sinuses showed mild inflammatory changes. The mastoids were clear. The orbital structures were within normal limits and no significant osseous or soft tissue abnormalities were seen.  The head MR spectroscopy revealed short and long TE MR spectroscopy tracings from voxels placed in the right frontal lobe over an area of FLAIR hyperintensity, showed relatively decreased creatinine. The significance was uncertain, but it could indicate an area of diminished metabolic activity, possibly reflecting gliosis. Other voxels placed in the right basal ganglia and parasagittal lobe regions showed normal tracings. The impression reported was: Foci of nonspecific white matter abnormalities in the right frontal lobe; MR spectroscopy tracings showed decreased creatinine which could reflect ischemic gliosis; subtle asymmetry in the gyral patterns between the right and left cerebral hemispheres evident near the vertex which might represent a mild form of cortical dysplasia. On 12/16/03 the patient was seen for an EMG. It was noted that the patient was 23 months and she had a normal birth. Her early development has had progressive motor regression since 12 months of age, when the parents noted right leg and arm weakness. The weakness became bilateral and involved upper and lower extremities. The patient began walking at 14 months, but she could no longer walk. The patient has trouble holding her head up when tired, trouble lifting her arms above her head, trouble holding objects and she does not use pincer grasp well. There was associated cognitive dysfunction (she only said one word and understood a few others). The patient did not point. Clinical examination showed that the patient was able to hold her head up. Tone was spastic, bilaterally, in arms and legs. The patient reached across her body bilaterally but not over her head. She was able to extend and flex her hips and knees bilaterally against mild resistance and was able to flex and extend her ankle. She oriented to mild pinch bilaterally on arms and legs. Reflexes were symmetric, 2+ in upper extremities, 3+ at knees, continuous clonus at ankles. Plantars extensor bilaterally. The interpretation was that it was a normal but limited study. Sensory nerve conduction studies could not be done because of patient discomfort and agitation. There was no electrodiagnostic evidence of demyelinating peripheral neuropathy based on normal peroneal and median conduction velocities. On 2/17/04 the patient was seen and it was noted that she had history of spastic quadriparesis, motor impairment and white matter changes. On 2/17/04 she had an MR of the brain with MR spectroscopy and MR of cervical spine and this was compared with a head MRI with MR spectroscopy done on 12/10/03. As noted in the previous exam, there was gyral asymmetry between the right and left cerebral hemispheres and the gyri on the right side appeared fuller and the sulci smaller. The subcortical white matter along the right vertex appeared slightly attenuated compared to the left side. This difference in signal intensity was less marked as compared to the previous examination and might be due to continued myelination on the right side. These changes likely represent some migration anomaly of the brain. There was slight reduction in the focal area of increased signal intensity seen in previous examination involving the right frontal lobe, immediately anterior to the frontal horn. It demonstrated less enhancement and this was non-specific in appearance. There was decreased myelination in the bilateral inferior frontal lobe regions that was delayed for this age. Slight mucosal hypertrophy was seen in bilateral maxillary sinus and ethmoid air cells. MR spectroscopy of the brain revealed multiple single voxels were obtained and showed tracings within normal range for present age. MR imaging of the cervical spine revealed vertebrae and disc spaces were normal in height and alignment. Spinal cord in the cervical part appeared normal with no abnormal areas of intensity, cranio-vertebral junction appeared normal, no osseous or soft tissue abnormalities identified. The impression reported was: asymmetry in gyral patters between right and left cerebral hemispheres suggestive of some migration anomaly; difference noted earlier in the attenuation between the subcortical white matter along right vertex was slightly less marked and the focal area of increased signal intensity noted earlier in right frontal lobe was less marked with less enhancement; slight mucosal hypertrophy involving bilateral maxillary sinus and ethmoid sinuses were seen. Upon internal review, brain white matter problems and leukodystrophy was determined to be an other important medical event (OMIC). Additional information has been requested.