|Year : 2019 | Volume
| Issue : 2 | Page : 105-108
Primary progressive multiple sclerosis in a 10-year-old Nigerian boy
Jane Owo Anyiam, Sani Musa, Hadiza Aliyu, Robinson Daniel Wammanda
Department of Paediatrics, Ahmadu Bello University Teaching Hospital, Shika, Zaria, Nigeria
|Date of Submission||05-Mar-2018|
|Date of Acceptance||14-Jun-2018|
|Date of Web Publication||20-Jun-2019|
Dr. Sani Musa
Department of Paediatrics, Ahmadu Bello University Teaching Hospital, Shika, Zaria
Multiple sclerosis (MS) is generally believed to be extremely rare in black African children, and the primary progressive form is the rarest type. We describe a case of primary progressive multiple sclerosis (PPMS) in a 10-year-old Nigerian boy and highlight the challenges of management in a resource-limited setting. He presented with 2 years history of inability to neither walk nor use the left upper limb, and a year history of progressive loss of vision. The symptoms were insidious in onset and associated with nasal and slurred speech, hesitancy, incomplete voiding, and occasional jerky movement of the lower limbs. Examination revealed a cheerful child with good memory and intelligence. There were reduced muscle power and lead-pipe rigidity in all the limbs except the right upper limb which was normal. Fundoscopy revealed bilateral optic atrophy. The diagnosis of PPMS was made by the typical clinical presentation, and brain and spinal cord magnetic resonance imaging (MRI). We had no facilities for cerebrospinal uid monoclonal IgG. This case highlights the need for pediatricians in the tropics to have a high index of suspicion and for healthcare systems to be equipped with facilities to accurately diagnose pediatric MS.
Keywords: Children, multiple sclerosis, resource-limited setting
|How to cite this article:|
Anyiam JO, Musa S, Aliyu H, Wammanda RD. Primary progressive multiple sclerosis in a 10-year-old Nigerian boy. Sahel Med J 2019;22:105-8
| Introduction|| |
Multiple sclerosis (MS) is a rare neurodegenerative disorder, characterized by inflammatory, demyelination of the central nervous system (CNS). It is generally believed to be extremely rare in black African children, and the primary progressive form is the rarest type. In Nigeria, it was initially thought that this disease does not occur, until very recently when some cases were reported in adults. Less than 5% of all MS occur in the pediatric age group and tends to be more common in girls than boys. MS has a variable mode of presentation but, usually, runs a relapsing–remitting course. At the moment, the mainstay of its diagnoses depends on the presence of typical clinical and magnetic resonance imaging (MRI) features disseminated in time and space. It is more difficult to diagnose MS in younger children because the presentation in them tends to be atypical and has a wider range of potential differential diagnoses. This is particularly so in the sub-Saharan Africa where the index of suspicion is low, and the facilities for accurate diagnosis are limited. This paper aims to report a case of primary progressive multiple sclerosis (PPMS) in a 10-year-old Nigerian boy that presented at the Ahmadu Bello University Teaching Hospital, Shika, Zaria. Ethical approval was obtained (December 20, 2016) before the report was written.
| Case Report|| |
A 10-year-old boy presented with 2 years' history of inability to neither walk nor use the left upper limb and a year's history of progressive loss of vision. The illness started with limping of the right lower limb which progressed to involve the left lower limb within 6 months. The weakness progressed gradually over 12 months to culminate in the boy's inability to neither walk nor use the left upper limb. A year before presentation, he developed progressive visual impairment which leads to his inability to see with both eyes at the time of presentation. These symptoms were insidious in onset and associated with nasal and slurred speech, hesitancy and incomplete voiding, and occasional jerky movement of the lower limbs.
Systemic review revealed no fever, jaundice, weight loss, and paleness of the body. Neurological symptoms such as headache, convulsions, loss of consciousness, hypoesthesia, or paresthesia were not present. He had no changes in his personality and handwriting or decline in school performance. There was no hearing impairment, incontinence of feces or the urine, reddening, tearing, or discharge from the eyes. On his academic performance rating, he was among the first ten, although he was in primary 2 at 10 years due to late school enrollment. He has, however, dropped out of the school for up to a year on account of these illnesses. The perinatal history was essentially normal and he had normal development. He is the first of five children and had a measles-like illness at the age of 5, which was managed at home. One of the siblings had a history of febrile convulsions. Parents had no formal education; the father is a fisherman whereas the mother is not gainfully employed.
On examination, he was not ill looking, afebrile, and not pale and had no lymphadenopathy. He had normal anthropometry for age. Cardiorespiratory findings were normal. He was cheerful with good memory and intelligence but had dysarthria, and a visual acuity limited to perception of hand movement, normal pupillary reflexes but had bilateral optic atrophy on funduscopy. No evidence of muscle atrophy, muscle tone, and power was normal in the right upper limb.
However, muscle power was 2/5 in both lower limbs and 3/5 in the left upper limb with associated lead-pipe rigidity. There was a generalized hyperreflexia, with bilateral sustained ankle clonus and Babinski response. He had normal sensations.
An assessment of neurodegenerative disease with differentials of intracranial space-occupying lesion, subsclerosing panencephalitis, and vasculitic stroke was made. His hemoglobin electrophoresis pattern was AA, and full blood count revealed moderate eosinophilia. Cerebrospinal fluid (CSF) microscopy, culture, and sensitivity were normal. Microscopy of urine and stool showed ova of Schistosoma hematobium and cysts of entamoeba coli, respectively. Biochemical investigations revealed normal urea, electrolytes and creatinine, mildly elevated CSF protein (389 mg/L), moderately elevated alkaline phosphatase, and mild hypocalcemia with normal serum phosphorus and albumin.
Brain MRI [Figure 1] revealed multiple ovoid lesions of varying sizes in the periventricular area of both posterior horns of the lateral ventricles. It is isointense on T1-weighted and hyperintense on T2 FLAIR with mixed intensity on T2-weighted. It shows no enhancement following contrast administration. The splenium of the corpus callosum shows a markedly reduced size and signal intensity. There is the prominence of the sulci, basal cisterns with enlargement of the third ventricle. Both globes and optic nerves were within normal limits. There were scattered hyperintense lesions in the lumbosacral region of the spinal cord [Figure 2].
|Figure 1: Brain magnetic resonance imaging showing features of multiple sclerosis|
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|Figure 2: Magnified portion of spinal cord magnetic resonance imaging showing hyperintense lesions|
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The patient was managed with daily physiotherapy on admission, prednisolone at 2 m/kg/day 6 hourly, and multivitamins and had an appreciable improvement in muscle power before discharged. The patient, however, was lost to follow-up after discharge despite appropriate counseling.
| Discussion|| |
This 10-year-old boy had the opticospinal form of PPMS with spastic triplegia and bilateral optic neuritis. This is the rarest form of MS that seldom present in boys <16 years, especially Africans. The age and sex of the case being reported show that PPMS occurs in children, and clinicians should have high index of suspicion for evaluating patients with similar symptoms as mentioned above. The revised, simplified Mc Donald criteria for the diagnosis of MS require that the MRI lesions are separate in time and space. In this case, typical lesions of MS were demonstrated in the brain and spinal cord; however, the patient presented late and could not afford the repeat brain MRI to demonstrate the separation of the lesions in time.
However, in the progressive form of the disease, the lesions may not be distinctively separated in time because the definitive lesions are hardly formed as the initial inflammation is active and persistent. This is not so in the nonprogressive type, where there is usually complete healing in one affected neural lesion before the occurrence of another. The lesions usually occur as a result of healing by gliosis that produces areas of dense tissues which appear as hyperintense lesions of CNS on the MRI. This is in addition to the fact that the lesions in children tend to vanish quickly.
The facilities for CSF monoclonal IgG were lacking in the center, and so he did not have the test. Clinically, however, the features of the disease were separated in space and time and had progressed for more than 1 year.
It is generally difficult to clinically diagnose MS in children because of its substantial variability of symptoms in this age group. It is also challenging to confirm the diagnosis by way of imaging which depends solely on the classical MRI features disseminated in time and space.
Although Ibrahim et al. have reported five MRI confirmed cases of MS from our center last year, they were all adult cases. This is, therefore, the first likely case of pediatric MS reported from our center.
The challenges encountered in the management of this patient were the late presentation, extreme poverty, and ignorance on the part of the patient and inadequate diagnostic facilities on the part of the hospital. The parents' ignorance could explain why the patient became lost to follow-up despite appropriate counseling. This is what is usually obtained in Sub-Saharan Africa and other resource-limited settings.
| Conclusion and Recommendations|| |
This is a rare case of an opticospinal form of PPMS in a 10-year-old boy. It highlights the need for pediatricians in the tropics to have a high index of suspicion for MS and for healthcare systems to be equipped with facilities to accurately diagnose noncommunicable diseases such as MS. There is also the need to create public awareness to improve health-seeking behavior which will reduce late presentation to health facilities.
Declaration of patient consent
The authors certify that we have obtained written informed patient consent. The patient has given his consent for his images and other clinical information to be reported in the journal. The patients understand that his names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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