|Year : 2019 | Volume
| Issue : 2 | Page : 97-99
Idiopathic central pontine and extrapontine myelinolysis in a child
Madhav Kamath1, Sneha Manjunath2
1 Department of Pediatrics, Adichunchanagiri Institute of Medical Sciences, Mandya, Karnataka, India
2 Department of Pediatrics, Chacha Nehru Bal Chikitsalaya, New Delhi, India
|Date of Web Publication||20-Aug-2019|
Dr. Sneha Manjunath
Department of Pediatrics, Number 10, Hostel No. 16, All India Institute of Medical Sciences (AIIMS) Campus, Ansari Nagar, New Delhi 110029.
Source of Support: None, Conflict of Interest: None
| Abstract|| |
The objective of this study was to report a case of central pontine and extrapontine myelinolysis in a child, a rare neurological disorder. A diagnosis of demyelination carries important therapeutic and prognostic implications. In most cases, the diagnosis is made clinically and confirmed radiologically. Treatment should be initiated sincerely as severe cases have a dismal prognosis.
Keywords: Central pontine myelinolysis, extrapontine myelinolysis, idiopathic, osmotic demyelination syndrome
|How to cite this article:|
Kamath M, Manjunath S. Idiopathic central pontine and extrapontine myelinolysis in a child. J Pediatr Neurosci 2019;14:97-9
| Introduction|| |
Pediatric osmotic demyelination syndrome (ODS) is a rare condition with a very few cases reported in the world literature. In a recent review by Bansal and Zinkus, 106 pediatric cases were reported between 1960 and 2018, among which only 27 had combined central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM).
Most often, the etiology is electrolyte imbalance or its rapid correction but in our case, no such correlation was established. Many reports showed intravenous immunoglobulin (IVIG) and plasmapheresis as treatment but this case responded well to steroids. All these features make it a rare idiopathic presentation of CPM and EPM.
| Case History|| |
A 6-year-old previously healthy and developmentally normal girl was admitted in our hospital with a history of fever since 5 days, altered sensorium since 2 days, vomiting since 1 day, and generalized weakness and decreased activity since 1 day. She had been treated with intravenous (IV) fluids and antibiotics in peripheral hospital as an inpatient. According to her mother, she had been cooperative and able to follow complex commands before illness. On examination, she was found to be irritable and sick looking with Glasgow Coma Scale 13/15, pulse rate 66 bpm, respiratory rate 28 cpm, and blood pressure 122/70mm Hg (95th centile: 117/75mm Hg). She had generalized hypotonic posture with limbs semi-flexed, externally rotated, and abducted. The examination of central nervous system revealed hypotonia in all limbs with decreased power. Deep tendon reflexes were decreased, superficial reflexes were normal with bilateral plantar extensor. Cranial nerve examination was normal and meningeal signs were inconclusive. Cerebellar signs were positive. Other systems were normal.
Initial blood and cerebrospinal fluid (CSF) investigations were within normal range. Blood and CSF culture showed no growth. Viral markers were not conducted due to lack of facility. IV antibiotics and antiviral were given for 7 days. Non-contrast computed tomography (NCCT) brain scan showed ill-defined hypodensity in bilateral cerebral peduncles, midbrain, pons, and middle cerebellar peduncles (suggestive of infarct or demyelination) [Figure 1]. Magnetic resonance imaging (MRI) of brain was carried out, which showed T2 hyperintensity diffusion restriction involving the central pontine region, crossing the midline with perilesional edema extending to midbrain, bilateral cerebral peduncles, and bilateral cerebellar peduncles with areas of microbleeds, suggestive of CPM and EPM [Figure 2], and T1 showing trident hypodensity in pons [Figure 3]. Diagnosis of CPM and EPM was made.
|Figure 1: NCCT scan shows trident-shaped hypodensity in central pons, hypodensity in bilateral cerebral peduncles, midbrain, and middle cerebellar peduncles|
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|Figure 2: MRI T2 image showing trident-shaped hyperintensity with a small area of T2 hypointense ? hemorrhage in central pons. T2 hyperintensity diffusion restriction crossing the midline with perilesional edema extending to midbrain, bilateral cerebral peduncles, and bilateral cerebellar peduncles with areas of microbleeds|
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The patient was treated with oral prednisolone 2mg/kg/day for 5 days and tapered to 1mg/kg for the next 5 days and stopped gradually based on previous case reports. She showed a good response clinically, her gait and reflexes improved. Abnormal movements decreased and she was discharged. She is on follow-up and no residual neurological deficit was found with no recurrence of symptoms. In our case, the accurate etiology could not be identified, hence labeled as idiopathic.
The aim of this case report was to emphasize that it is imperative for physicians to remain cautious about any change in sensorium and neurological signs during the course of treatment and aggressively evaluate to rule out CPM, which can be fatal if not treated in earnest.
| Discussion|| |
Since its inception in 1959 as described by Adams et al., the concept of myelinolysis has evolved over time, transforming from a disorder that was usually diagnosed at autopsy in the 1960s, to one diagnosed by imaging and typically associated with a favorable outcome over the past two decades. Is there a change in the disorder or the treatment? Unlikely. What largely has changed is our ability to detect it early, because of advanced imaging techniques such as computed tomography (CT) and MRI.
Clinical features of the patient usually go through a biphasic clinical course, initially encephalopathic or presenting with seizures, then recovering rapidly, only to deteriorate few days later. The initial signs of the CPM, which reflect this second phase, include dysarthria and dysphagia (secondary to corticobulbar fiber involvement), a flaccid quadriparesis (from corticospinal tract involvement), which later becomes spastic, all from the involvement of the basis pontis; if the lesion extends into the tegmentum of the pons pupillary, oculomotor abnormalities may occur. There may be an apparent change in conscious level reflecting the “locked-in syndrome.”
The lesions can involve pons, cerebellum, lateral geniculate body, external capsule, extreme capsule, hippocampus, putamen, cerebral cortex/subcortex, thalamus, and caudate nucleus. Microscopically, the lesion shows degeneration and loss of oligodendrocytes with preservation of axons unless the lesion is very advanced.
CPM can be seen on CT, but MRI is frequently striking and is the imaging technique of choice, having a greater sensitivity than CT and superior capacity for the demonstration of the lesions of EPM. Hyperintense lesions are seen on T2, and hypointense lesions are seen on T1-weighted images. The lesions are non-contrast enhancing.
Previous reports do not conclude any connection between the extent of lesion on MRI and clinical severity at presentation and clinical course subsequently.
Isolated case reports suggest the role of steroids, plasmapheresis, and imidazole pyridine tartrate in the treatment of CPM. Aggressive supportive therapy should be provided for managing complications of neurologic deficit resulting from CPM.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Bansal LR, Zinkus T Osmotic demyelination syndrome in children. Pediatr Neurol 2019;97:12-17.
Chong CH, Lai GM, Lin JT Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) following concurrent chemoradiotherapy for nasopharyngeal carcinoma. J Cancer Res Pract 2016;3:58-62.
Martin RJ Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry 2004;75:iii22-8.
Brito AR, Vasconcelos MM, Cruz Júnior LC, Oliveira ME, Azevedo AR, Rocha LG, et al
. Central pontine and extrapontine myelinolysis: report of a case with a tragic outcome. J Pediatr (Rio J) 2006;82:157-60.
Menakaya JO, Wassmer E, Bradshaw K, Seri S, Whitehouse WP Idiopathic central pontine myelinolysis in childhood. Dev Med Child Neurol 2001;43:697-700.
Koul PA, Khan UH, Jan RA, Shah S, Qadri AB, Wani B, et al
. Osmotic demyelination syndrome following slow correction of hyponatremia: possible role of hypokalemia. Indian J Crit Care Med 2013;17:231-3.
] [Full text]
Adams RD, Victor M, Mancall EL. Central pontine myelinolysis. A hitherto undescribed disease occurring in alcoholic and malnourished patients. Arch. Neurol. Psychiat. (Chic.) 1959;81:154-172.
[Figure 1], [Figure 2], [Figure 3]