Coexisting Spinal Intramedullary and Intracranial Tuberculomas in an Immunocompetent Child.

Pediatric neurotuberculosis manifests commonly as tuberculous meningitis and intracranial tuberculomas. The ratio of occurrence of intracranial to intraspinal tuberculoma reported is 42:1. Intramedullary tuberculomas (IMTs) are rare, and the coexistence of intramedullary and intracranial tuberculoma is extremely rare. We report a case of coexisting intramedullary and intracranial tuberculoma in a 5-year-old boy who presented with fever for 12 days, progressive motor weakness in the lower limbs for 9 days, and retention of urine and constipation for 6 days. Neurological examination revealed signs of compressive myelopathy. Magnetic resonance imaging (MRI) of the spine detected IMT at D4-D5 level of the thoracic cord with perilesional edema. MRI of brain revealed a right frontal tuberculoma. Medical management with antituberculosis therapy and steroids resulted in complete neurological recovery. Copyright:

Introduction

Intracranial tuberculoma and tuberculous meningitis are frequent manifestations of pediatric neurotuberculosis. Intramedullary tuberculoma (IMT) of the spinal cord is rare, constituting 0.2%–5% of central nervous system (CNS) tuberculomas.[12] Coexistence of IMT and intracranial tuberculoma is rare, only few adult and scarcely pediatric cases are reported in the literature.[34567] Clinically, IMT presents with insidious symptoms of progressive myelopathy indistinguishable from other benign or malignant spinal space occupying lesions (SOLs). Magnetic resonance imaging (MRI) of the spine is a sensitive tool for differentiating tuberculoma from other SOLs and has significantly reduced the need for invasive procedures such as biopsy or open surgery for diagnosis. IMT is benign and curable, however delayed diagnosis and treatment can lead to significant morbidity.[8] We report a five-year-old boy with coexisting spinal IMT with intracranial tuberculoma who was treated conservatively with antituberculosis therapy (ATT) and steroids.

Case Report

A 5-year-old boy presented with fever for 12 days, progressive motor weakness in lower limbs for 9 days, and retention of urine and constipation for 6 days. The patient was treated elsewhere for fever for three days. However, on the fourth day of the illness, the child developed progressive symmetrical lower limb weakness causing difficulty in standing, sitting, and turning from side to side. Over the next three days, he developed retention of urine and constipation, hence was referred to our hospital for further management.

There was no history of headache, vomiting, altered sensorium, cranial nerve or sensory deficit, recent immunization, and head or spine trauma. The patient had no tuberculosis (TB) in the past nor TB contact.

At admission, his pulse rate was 88/min, respiratory rate was 32/min, and blood pressure was 90/60 mm of Hg. Bacillus Calmette Guerine vaccine scar was seen. Spine palpation revealed no tenderness or deformity. His weight (16kg) and height (106cm) both were between the 10th and 25th centiles for his age and sex. His higher mental functions, cranial nerves, and sensory examination were normal, and papilledema was absent. Tone, power, and reflexes of both upper limbs were normal. Both lower limbs had hypotonia, power grade 2/5, and exaggerated (3+) deep tendon reflexes. Abdominal and cremasteric reflexes were absent and plantar reflexes were extensor bilaterally. Meningeal signs were absent. The patient had mild firm hepatomegaly (2cm) and a palpable distended urinary bladder. Respiratory and cardiovascular system examination was normal.

His initial investigations showed iron deficiency anemia and elevated Erythrocyte sedimentation rate (25 mm at 1h), a negative Mantoux test, normal chest radiograph, and gastric aspirate negative for acid-fast bacilli. Enzyme-linked immunosorbent assay was negative for human immunodeficiency virus infection. MRI of dorsal spine (P + C) showed a well-defined oval T2 hypointense ring-enhancing lesion (4.5 × 2.5 mm) in the thoracic cord at D4-D5 level, with mild perilesional edema from D3 to D5 level suggestive of IMT [Figure 1]. MRI of brain revealed few tiny T2/fluid attenuation inversion recovery (FLAIR) hyperintense enhancing foci in left frontal white matter and subependymal region of the left ventricle. Minimal focal subcortical white matter edema was observed in right frontal lobe suggestive of tuberculoma [Figure 2].

Figure 1

A well-defined oval T2 hypointense ring-enhancing lesion is seen in the thoracic cord at D4-D5 level, with mild perilesional edema from D3 to D5 level suggestive of intramedullary tuberculoma

Figure 2

MRI of brain showing few tiny T2/FLAIR hyperintense enhancing foci in left frontal white matter and subependymal region of the left ventricle. Minimal focal subcortical white matter edema is seen in right frontal lobe suggestive of tuberculoma

The patient was started on ATT regime containing two months of Isoniazid (H), Rifampicin (R), pyrazinamide (Z) and Ethambutol (E) followed by 7 months of Isoniazid (H), Rifampicin (R) and Ethambutol (E) therapy (2HRZE +7HRE) with steroids for four weeks. Parenteral dexamethasone was given for two days followed by oral prednisolone for a total of four weeks. He showed clinical improvement in three days and had complete clinical recovery by nine days. Meanwhile, family screening for TB detected sputum positive for pulmonary TB in father. His father was enrolled and started on standard ATT, the younger sibling was started on isoniazid prophylaxis as per Revised National Tuberculosis Control Program guidelines. On follow-up, the patient was found to be compliant with ATT and clinically well without adverse effects.

Discussion

Primary neurotuberculosis is seen predominantly in young population of developing countries. CNS involvement is seen in 10% of cases with systemic TB secondary to hematogenous spread from primary focus, commonly the lungs.[4] IMT is a rare occurrence even in TB-endemic regions.[8] Our patient had primary TB of CNS, affecting spinal cord, which is similar to the case reported by Thacker and Puri.[4] Cases by Krishnan et al.,[3] Chitre et al.,[5] Bansal et al.,[6] and Kulkarni et al.[7] showed evidence of extra-neural TB or were under treatment for tuberculous meningitis. The literature suggests that 50% of cases with CNS involvement are below 10 years of age with a slight male preponderance for IMT (M:F = 1.5:1).[89] Clinical symptomatology and presentation of IMT are indistinguishable from other spinal cord tumors. The symptoms of IMT are of myelopathy with motor and sensory symptoms depending on the level of lesion.[4567] Associated intracranial tuberculoma can be an incidental finding or asymptomatic manifestation.[2] However, in upto one third cases of neurotuberculosis, evidence of extra-neural TB may not be found. Therefore, the absence of an extra-neural source, as in our case, should not rule out the possibility of tuberculous etiology.[345678]

Our patient is a 5-year-old immunized male from TB-endemic region presented with features of compressive myelopathy alone. A definitive diagnosis of coexisting intramedullary spinal tuberculoma and intracranial tuberculoma was established with MRI. He had complete neurological recovery with ATT and steroids. No evidence of extra-neural TB or immunosuppression was reported, indicating it as a primary neurotuberculosis in an immunocompetent patient. Eleven adult[1011121314151617181920] and five pediatric[34567] cases (≤12 years) of coexisting intracranial and intraspinal tuberculoma were reported in the literature [Table 1]. The age and clinical presentation of compressive myelopathy alone in our patient was similar to the cases reported by Thacker and Puri[4] and by Bansal et al.[6] The cases presented by Krishnan et al.[3] and Chitre et al.[5] had features of compressive myelopathy and intracranial symptoms. However, Kulkarni et al.[7] reported a case who presented with only intracranial symptoms.

Table 1

Characteristic features of reported cases of coexisting intracranial and intraspinal tuberculomas

Year	Author	Age/sex of patient	Spinal lesion site	Intracranial lesion site	Symptoms		Surgery	Outcome
					Spinal	Cranial
1993	Shen et al.	30 year/M	Cervical thoracic	Brain stem cerebral cerebellar	Yes	No	No	Partially recovered
1999	Huang et al.	38 year/M	Cervical thoracic	Basal subarachnoid space, optic chiasma, internal auditory canal, right Sylvian fissure	Yes	Yes	Yes	Partially recovered
2000	Kim et al.	40 year/F	Thoracic	Right temporal, left basilar cistern	Yes	Yes	Yes	Partially recovered
2002	Bansal et al.*	11 year/M	Cervical	Cerebellum	Yes	No	No	Recovered
2003	Yen et al.	67 year/M	Thoracicolumbar	Brain stem, cerebrum, cerebellum	Yes	No	Yes	Partially recovered
2004	Thacker and Puri*	6 year/F	Thoracic	Right frontal right cerebellar	Yes	No	No	Recovered
2004	Shenoy and Raja	38 year/M	Cervical	Cerebral	Yes	Yes	Yes	Not Recovered
2006	Muthukumar et al.	14 year/F	Thoracic	Frontal thalamus	Yes	Yes	Yes	Partially recovered
2007	Muthukumar et al.	21 year/M	Thoracic	Multiple intracranial	Yes	No	Yes	Recovered
2008	Park and Song	66 year/F	Cervical thoracic	Subcortical	Yes	Yes	Yes	Partially recovered
2009	Chitre et al.*	6 year/F	Thoracic	Left temporal	Yes	Yes	Yes	Partially recovered
2010	Kulkarni et al.*	4 year/M	Cervical	Cerebrum, cerebellum, brain stem	No	Yes	No	Recovered
2013	Lim et al.	66 year/F	Thoracicolumbar	Frontoparietal, thalamus, cerebellum	Yes	No	No	Recovered
2014	Krishnan et al.*	12 year/M	Multiple site	Multi-lobar cerebral, cerebellar, brain stem	Yes	Yes	No	Recovered
2015	Dong-Yoon et al.	65 year/F	Thoracic	Frontoparietal	Yes	No	Yes	Partially recovered
2017	Jaiswal et al.	16 year/M	Lumbar	Multiple supra- and infratentorial	Yes	Yes	No	Recovered
2019	Present case report*	5 year/M	Thoracic	Right frontal	Yes	No	No	Recovered

F = female, M = male

* pediatric cases (≤12 years)

IMTs are usually solitary lesions located commonly in the thoracic cord, the higher incidence is explained based on the fact that thoracic cord receives approximately 45% of the entire supply to the cord.[45] The MRI is a sensitive, noninvasive tool for accurate localization and diagnosis of tuberculoma, thus avoiding the need for an invasive procedure such as biopsy or open surgery.[5202122] The radiological differential diagnosis of intramedullary spinal tuberculoma or lesion apart from tuberculoma would be neurocysticercosis, glioma, ependymoma, fungal infections, toxoplasmosis, and lymphoma. However, these lesions are common in adult population.[23]

Controversy prevails regarding the management options of IMT and no standard treatment protocol is available due to rarity of the disease. Conservative management with ATT and steroids as anti-edema agents is the mainstay of treatment.[6]

Considering the management and outcome, four[34567] out of the five pediatric cases reported had recovered completely with medical management, only one case reported by Chitre et al.[5] required surgical intervention and had partial recovery.

Conservative medical therapy is an effective, inexpensive, safe, and feasible option, especially in developing countries and resource-poor settings.[4] The role of steroids is largely unproven yet remains helpful in patients with perilesional edema. Surgery is indicated in patients with large lesions causing significant compression, lack of clinical or radiological response, deterioration during conservative treatment, or in case of uncertain diagnosis.[5] Patients with profound neurological deficits require early surgical decompression as a delay can cause irreversible damage to the spinal cord, leaving permanent neurological sequelae.[20]

In TB-endemic countries, tuberculoma should be considered as a differential diagnosis for intramedullary SOLs, irrespective of age or evidence of systemic TB.

The presence of an IMT is an indication for MRI of brain to detect coexisting intracranial tuberculoma.

The conservative treatment with ATT and steroids is effective and results in complete clinical recovery in most cases.

Conclusion

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Conflicts of interest

There are no conflicts of interest.