Infantile Tuberculous Meningitis Complicated by West

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Case Report

Infantile Tuberculous Meningitis Complicated by West Syndrome Division of Pediatric Neurology, Department of Pediatrics, 1Department of Radiology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Abstract

Sumeet R. Dhawan, Jitendra K. Sahu, Naveen Sankhyan, Sameer Vyas1, Pratibha D. Singhi

Tuberculous meningitis rarely causes refractory epilepsy and is a rare cause of West syndrome. We describe three infants, who had tuberculous meningitis complicated by stroke and paradoxical tuberculoma, and, who later developed West syndrome. Keywords: Chronic meningitis, infantile spasm, meningeal tuberculosis, paradoxical tuberculoma, refractory epilepsy

Introduction

W

est syndrome is catastrophic epilepsy of infancy and childhood characterized by a triad of epileptic spasms, hypsarrythmia, and developmental retardation.[1] Tuberculous meningitis (TBM) is a rare cause of West syndrome.[2,3] Timely diagnosis and early initiation of treatment are crucial to morbidity and mortality in both TBM and West syndrome. We describe three infants with TBM and West syndrome.

Case Descriptions Out of 289 children with TBM in our institute from January 2011 to August 2015, three children developed West syndrome. The diagnosis of TBM was based on the clinical symptoms suggestive of TBM (fever >5 days with or without headache, vomiting, focal deficits and/ or encephalopathy, suggestive neuroradiology for TBM, suggestive cerebrospinal fluid, and/or evidence of extracranial tuberculosis). The children with West syndrome were treated with corticosteroids and followed up for outcome assessment [Table 1]. All children had basal exudates and hydrocephalous at diagnosis. Cerebrospinal fluid analysis of all three children was consistent with TBM. However, acid-fast bacilli were not isolated in any children. These children developed epileptic spasms after 3–14 months of TBM diagnosis. All three children had developmental delay and epilepsy on follow-up.

Discussion These cases highlight that epileptic spasms can complicate the course of TBM. The latency of epileptic Access this article online Quick Response Code:

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DOI: 10.4103/jpn.JPN_137_17

spasms varies from 4 to 24 months following the neurological insult.[4] This latency depends on various factors such as severity of brain injury and age at insult. The initial insult results in pathological processes such as gliosis and abnormal synaptic reorganization. Infantile TBM is frequently complicated by progressive hydrocephalous and secondary neuronal injury, progressive meningovasculitis leading to infarcts, and paradoxical tuberculoma as in index cases, which contribute to epileptogenesis in TBM.[5] There are only few reports of epileptic spasms in TBM. A 10-month-old girl with focal infantile spasm due to underlying cerebral tuberculoma has been described.[2] Zorn-Olexa et al.[3] described a child with TBM (with tuberculoma) who initially presented with refractory status epilepticus and subsequently refractory infantile spasm. The etiology was multifactorial with infarcts, tuberculomas, and paradoxical tuberculomas. Watanabe et al.[6] described a 10-month-old infant with TBM who presented with status epilepticus and developed spasms 3 months later. Thus, these cases highlight the multifactorial origin wherein stroke, porencephaly, and paradoxical tuberculoma contribute to refractory epilepsy in TBM. Refractory epilepsy is an uncommon complication with TBM. In a series, 10% of children had epilepsy, 75% of seizures in TBM were controlled with only one antiepileptic drug, and 25% required two antiepileptic Address for correspondence: Professor Pratibha Dutta Singhi, Department of Pediatric Neurology and Neurodevelopment, Medanta, The Medicity, Gurgaon, Haryana, India. E-mail: [email protected] This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected]

How to cite this article: Dhawan SR, Sahu JK, Sankhyan N, Vyas S, Singhi PD. Infantile tuberculous meningitis complicated by West syndrome. J Pediatr Neurosci 2018;13:237-40.

© 2018 Journal of Pediatric Neurosciences | Published by Wolters Kluwer - Medknow

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[Downloaded free from http://www.pediatricneurosciences.com on Monday, October 1, 2018, IP: 41.68.101.222] Dhawan, et al.: Tuberculous meningitis and West syndrome

Table 1: Demographic profile, cerebrospinal fluid analysis, neuroimaging at diagnosis, and follow-up of children with tuberculous meningitis and West syndrome Demographic profile Age at diagnosis of TBM Latency between diagnosis and infantile spasms History of contact with open case of tuberculosis Investigations (at diagnosis) Cerebrospinal fluid analysis Cell count Glucose Protein CSF AFB culture and gastric lavage for AFB culture Mantoux Chest radiograph Biochemistry (LFT, RFT), HIV Neuroimaging Neuroimaging at diagnosis

Neuroimaging at development of infantile spasm

Risk factor for infantile spasm Follow-up Duration of follow-up Neurological status at follow-up

Case 1

Case 2

Case 3

10 months 6 months

3 months 3 months

4 months 14 months

Father

Nil

Nil

110 cells (80% lymphocytes)

275 cells (93% lymphocytes)

20 mg/dL (blood glucose, 86 mg/dL) 130 mg/dL Sterile

15 mg/dL (blood glucose, 110 mg/dL) 411 mg/dL Sterile

60 cells (predominant lymphocytes) 37 mg/dL

Positive Normal Normal

Positive Normal Normal

Communicating hydrocephalous and basal exudates [Figure 1A]

Noncommunicating hydrocephalous, diffuse meningeal enhancement, and basal exudates [Figure 2] Multiple tuberculomas in Communicating hydrocephalous, porencephalic suprasellar cisterns [Figure 2C] and chronic infarct in right dilatation of right lateral lentiform nucleus [Figure 2D] ventricle, and tuberculoma in right sylvan fissure [Figure 1] Infarct, tuberculoma Tuberculoma, infarct

134 mg/dL Sterile

Negative Normal Normal

Communicating hydrocephalous and basal exudates

Right encephalomalacia

Infarct

3 years 3 years 18 months Global developmental Global developmental Developmental delay (motor delay (developmental age, delay (developmental age, age, 10 month; language, 3–4 months) 12–15 months) 5–6 months) Outcome of spasm Poor (refractory) Responded to ACTH Responded to ACTH Epilepsy burden Refractory seizures (focal, Focal and generalized seizures, Focal seizures, 3–4 per year generalized), 3–5 per week 2–3 per year ACTH = adrenocorticotropic hormone, AFB = acid-fast bacilli, CSF = cerebrospinal fluid, HIV = human immunodeficiency virus, LFT = liver function test, RFT = renal function test, TBM = tuberculous meningitis

drugs.[7] Epileptic spasms, as in our report, may be a marker for poorer developmental outcomes and possibly, for refractory epilepsy in children recovering from TBM.

Conclusion West syndrome can rarely be seen in children recovering from TBM. Its occurrence may be a marker for poorer

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developmental outcomes and possibly, for refractory epilepsy in these children. Acknowledgements

Author contribution: SD, JS, NS, and PDS were involved in patient management and preparation of the draft. SV interpreted radiological data and approved manuscript. PDS did the critical review and would act as the corresponding author/guarantor.

Journal of Pediatric Neurosciences ¦ Volume 13 ¦ Issue 2 ¦ April-June 2018


Figure 1: (A) Contrast-enhanced computed-tomography scan showing communicating hydrocephalous and basal meningeal enhancement (black arrows). (B and C) Gadolinium contrast-enhanced magnetic resonance imaging (6 months after starting therapy) showing gross hydrocephalous (right > left). The right cortical mantle in right frontoparietal region (white arrow) is grossly thinned out with ventricle nearly abutting the calvaria. Multiple ring-enhancing lesions are seen in the right sylvian fissure suggestive of tuberculoma (curved arrow)

Figure 2: (A) Computed-tomography scan showing mild hydrocephalous and exudates in right sylvian fissure and basal cisterns (arrow). These exudates (arrow) and hydrocephalous worsened after 1 month of therapy (B). (C) Gadolinium contrast-enhanced magnetic resonance imaging (MRI) showing multiple conglomerate ring-enhancing lesions in basal cisterns (arrow) and bilateral sylvian fissure (right > left). (D) MRI T2-weighted image showing hyperintensity in right lentiform nucleus (arrow) suggestive of chronic infarct. These areas were hypointense in corresponding T1-weighted images (not shown). These tuberculomas reduced significantly (E) and were calcified (E and F) after 3 years of therapy


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Financial support and sponsorship

Nil. Conflicts of interest

The authors have no conflicts of interest to disclose with regard to this article. Ethical approval

An informed consent form was signed by the parents of the patients to approve the use of patient information or material for scientific purposes. The patient’s identity has not been disclosed anywhere in the article, and the article does not contain any identifiable images.

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