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CASE REPORT |
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| Year : 2006 | Volume
: 1
| Issue : 2 | Page : 60-62 |
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Multiphasic disseminated encephalomyelitis, uncommon cause for central demyelination in children
Deepak Goel1, Anil Singhal1, Rajender K Srivastava2, Rahul Jain1
1 Department of Neurology, Himalayan Institute of Medical Sciences, Dehradun, UP, India
2 Department of Radiology, Himalayan Institute of Medical Sciences, Dehradun, UP, India
Correspondence Address:
Deepak Goel
Department of Neurology, HIMS, Doiwala, Dehradun - 249 014
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1817-1745.27455
 Abstract | | |
This case report is presented with the aim of discussing differential diagnosis of CNS demyelinating diseases in children below 15 years of age. The various diagnoses range from acute disseminated demyelinating disease to multiple sclerosis. In between the two extremes, we have multiphasic demyelinating encephalomyelitis. We had discussed the role of oligoclonal band in cerebrospinal fluid, auto antibodies and magnetic resonance imaging to differentiate these diagnoses.
Keywords: Childhood, encephalomyelitis, multiphasic, multiple sclerosis.
How to cite this article:
Goel D, Singhal A, Srivastava RK, Jain R. Multiphasic disseminated encephalomyelitis, uncommon cause for central demyelination in children. J Pediatr Neurosci 2006;1:60-2 |
| Introduction | |  |
Acute onset CNS demyelination in childhood (below 15 years of age) is uncommon but is an important group of disorder. Spectrum ranges from focal diseases {optic neuritis and transverse myelitis} to diffuse involvement {acute disseminated encephalomyelitis (ADEM), multiphasic disseminated encephalomyelitis (MDEM), neuro-myelitis optica and multiple sclerosis (MS)}.[1] ADEM is characterized by monophasic illness; therefore, it is confused only with the first attack of MS. MDEM is a multiphasic variant of ADEM. The diagnostic differentiation between ADEM/MDEM and MS is important mainly for prognostic reasons. Children with ADEM are generally expected to do well, whereas children with MS are more likely to develop significant disability. The ability to reduce MS disease progression with immunomodulatory drugs further emphasizes the importance of a prompt and accurate diagnosis. We present an interesting case with different aspects for diagnosis.
| Case Report | | |
A girl presented with a history of sudden visual loss in the right eye with peri-orbital pain at 11 years of age, which had recovered completely on oral steroids within a month. Nine months later, she developed paresthesia both lower limbs, followed by acute onset asymmetric quadriparesis, dysarthria and dysphasia, incoordination of hands, hesitancy and urgency of urine. There was history of repetitive febrile illness prior to each event. Thereafter, she was evaluated at a laboratory (not at our hospital), and investigations revealed leukocytes - 10,200/mm 3 , ESR - 08 mm/h. Blood urea, creatinine, bilirubin, ALT, AST, X-ray chest, and abdomen ultrasound were normal. Cerebrospinal fluid (CSF) analysis showed protien - 33 mg/dl, sugar - 66 mg/dl, no cells, negative gram and acid fast staining, sterile culture and positive oligoclonal band (OCB). Serology for ANA, ANCA; rheumatoid factor; VDRL; TPHA; and HIV were negative. Visual evoked potential (VEP) was delayed on right side.
Plain and contrast CT brain showed a large hypodense mass lesion located adjacent to the body of left lateral ventricle in the frontal region, with enhancement limited to medial margin of the lesion. An additional non-enhancing white matter hypodensity was also seen in right parieto-occipital region. T2-weighted TSE axial MR images through the above region demonstrated prolonged T2 relaxation time in the lesion that appeared hyperintense. The mass effect was mild in relation to the size of the lesion [Figure - 1](A-C).
In view of multiple episodes of neurological deficit, white matter lesions on MRI, abnormal unilateral VEP, no cells in CSF, positive oligoclonal band and negative antibodies against myelin basic protein (MBP), she was diagnosed a case of multiple sclerosis. She received five pulses of IV methyl prednisolone. She had partial recovery with treatment. At the age of 14, she was referred to our neurology unit with residual weakness without new attack. The presenting symptoms were weakness and incoordination of hands with walking difficulty.
On examination this time, the patient was conscious, oriented and euphoric. Mini mental score was normal. Visual acuity was decreased on right side with disk pallor. There were positive bilateral pyramidal and cerebellar signs. Right upper limb was slipping into involuntary posturing with grade IV grasping (groping response). Left upper limb had postural tremors. Ankle clonus was present in the left foot. Light touch and pinprick were preserved. Vibration and position sense were impaired in both lower limbs. She was not able to stand and walk. Her 'activity of daily living' score was 8/14.
Although she had plenty of evidence in favor of MS, we found few odd points. She was just 11 years old at onset of symptoms, and MRI lesions were atypical for MS. So this was an uncommon presentation of an uncommon disease and therefore resulted in search for other causes. We had done repeat MRI and CSF studies. MRI showed partial resolution of old lesions without any new lesion appearing [Figure - 1](D), and CSF - OCB was negative. The final diagnosis retained was MDEM. She is in our follow-up for more than the last 2 years without any relapse.
| Discussion | | |
While ADEM and MDEM are diseases mainly of the pediatric age group, MS is a disorder of adolescence and adulthood (15-60 years). Frequency of MS onset below the age of 15 years is 2.7-5% of all cases.[2]
Most important tools for investigating in patients of focal demyelination for predictability of future development of MS are CSF analysis for OB, auditory and visual evoked potentials, auto antibodies against (MBP) and myelin oligodendrocyte protein (MOG) and cerebral/spinal MRI.
CSF is the most important. A pleocytosis of more than 75 cells/mm 3 favor ADEM/MDEM over MS.[3] CSF - OCB is a very sensitive indicator but not very specific for MS and can be rarely positive in ADEM/MDEM.[4] Along with this, follow-up disappearance of OCB has been rated as a point against MS.[5] Our case also had disappearing OCB, which was creating a doubt in diagnosis of MS.
Auto antibodies to MBP and MOG in patients with focal demyelinating disease and positive CSF oligoclonal bands with white matter lesion on cerebral MRI had shown to be highly predictive for MS.[6] Both MBP and MOG were positive, with 95% chances of developing MS; and with MOG alone positive, the predictive value was 83% for developing MS. This test was not available in our setup.
In July 2000, the International Panel on the diagnosis of MS had integrated MRI into the overall diagnostic scheme.[7] Problem arises when the lesions are atypical, like in our case, which show a large demyelinating patch with necrosis and mass effect; and these type of lesions were thought to be an intermediate disease between MS and ADEM.[8],[9] In atypical cases, follow-up studies provide a better answer to the question. If no new lesions appear in six-monthly or yearly MRI, it is unlikely to be a case of MS.[10]
Our case having no clinical relapse for more than 2 years, with disappearance of CSF-OCB and with no new lesion detected on repeat study creates doubt in diagnosis of MS, and ADEM/MDEM is the most likely disease. Summary of differential diagnosis between ADEM and MS is given in [Table - 1].
This interesting case is good evidence that demyelinating disorder of CNS in children creates a challenge for clinicians; even costly sophisticated tests are noncontributory, and follow-up is the only confirmatory factor for final diagnosis.
| References | | |
| 1. | Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000;123:2407-22.  [PUBMED] [FULLTEXT] |
| 2. | Duquette P, Murray TJ, Plenes J, Ebers GC, Sadovnick D, Weldon P, et al . Multiple sclerosis in childhood: Clinical profile in 125 patients. J Pediatr 1987;111:359-63. |
| 3. | Hauser SL, Goodkin DE. In : Braunwald E et al , editors. Harrison's Principles of internal medicine, 15th ed. Mc Graw-Hill: New York; 2001. p. 2452-61. |
| 4. | Ben-Hur T, Abramsky O, River Y. The clinical significance of a single abnormal immunoglobulin band in cerebrospinal fluid electrophoresis. J Neurol Sci 1996;136:159-61. [PUBMED] [FULLTEXT] |
| 5. | Davies G, Keir G, Thompson EJ, Giovannoni G. The clinical significance of an intrathecal monoclonal immunoglobulin band: A follow-up study. Neurology 2003;60:1163-6. [PUBMED] [FULLTEXT] |
| 6. | Berger T, Rubner P, Schautzer F. Antimyelin antibodies as a predictor of clinically definite multiple sclerosis after a first demyelinating event. N Eng J Med 2003;349:139-45. |
| 7. | Mc Donald WI, Compston A, Edan G, Goodkin D, Hartung HP, Lublin FD, et al . Recommonded diagnostic criteria for multiple sclerosis: Guideline from the international panel on the diagnosis of multiple sclerosis. Ann Neurol 2001;50:121-7. |
| 8. | Kepes JJ. Large focal tumor-like demyelinating lesions of the brain: Intermediate entity between multiple sclerosis and acute disseminated encephalomyelitis: A study of 31 patients. Ann Neurol 1993;33:18-24. |
| 9. | Glasier CM, Robbins MB, Davis PC, Ceballos E, Bates SR. Clinical, neurodiagnostic and MR findings in children with spinal and brain stem multiple sclerosis. Am J Neuroradiol 1995;16:87-95. [PUBMED] [FULLTEXT] |
| 10. | Dale RC, Branson JA. Acute disseminated encephalomyelitis or multiple sclerosis: Can the initial presentation help in establishing a correct diagnosis? Arch Dis Childhood 2005;90:636-9. |
Figures
[Figure - 1] Tables
[Table - 1]
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