The Spectrum of Intracranial Arteriopathies and Ischemic Strokes in Pediatric Tubercular Meningitis: A Tricentric Study From Eastern India.

INTRODUCTION: Addressing the need to uniformly classify arteriopathies among patients with arterial ischemic stroke (AIS) due to tubercular meningitis (TBM), we used the Childhood AIS Standardised Classification and Diagnostic Evaluation (CASCADE) criteria. METHODS: This tri-centric prospective study included children aged 0.5-12 years with TBM and AIS. Magnetic resonance angiographies (MRAs) were done during admission and repeated 3 and 12 months after discharge. Arteriopathies were classified according to the primary CASCADE criteria. We used the modified Pediatric Alberta Stroke Programme Early Computed Tomography Score as an ordinal measure of infarct volume. The severity of arteriopathies was graded using the focal cerebral arteriopathy severity score (FCASS). The final outcomes were measured at the 12-month follow-up visit using the Pediatric Stroke Outcome Measure (PSOM). RESULTS: Out of 55 patients, 64% had MRA-evidenced arteriopathies and 84% had multiple infarcts. The middle cerebral (46%) and internal carotid arteries (22%) were most commonly affected. The basal ganglia (70%) and the cerebral cortex (61%) were most commonly infarcted. CASCADE categories included 3b (40%), 1d (38%), 2b (16%), 2c (5%), progressive (32%), and stable (44%) arteriopathies. Younger age, hypertrophic pachymeningitis, cortical infarcts, recurrent strokes, progressive arteriopathies, EEG abnormalities, and mortality were significantly higher among patients with MRA-proven arteriopathies. Patients with progressive arteriopathies had a significantly higher prevalence of hypertrophic pachymeningitis, cortical infarcts, and recurrent strokes. FCASS correlated positively with outcomes measured by the Pediatric Stroke Outcome Measure and modified Pediatric Alberta Stroke Programme Early Computed Tomography Score. CONCLUSION: The CASCADE classification clarified the arteriopathy patterns, enabling us to correlate them with the characteristics of the infarcts. FCASS is useful to grade the arteriopathy severity and progression in TBM.

The Spectrum of Pediatric Infection-Associated Intracranial Arteriopathies and Acute Ischemic Stroke at 2 Eastern Indian Tertiary Care Centres.

INTRODUCTION: Major and minor pediatric infections may cause intracranial arteriopathies, the long-term outcome of which we investigated and identified the factors influencing the progression/resolution of arteriopathies. METHODS: We collected the clinical and radiological data of children aged 1 month-15 years who had ischemic stroke with definite arteriopathy following a recent febrile infection. Repeated neuroimaging was done over the next year to ascertain recurrent strokes and the progression and resolution of arteriopathies. RESULTS: The anterior circulation was more frequently affected (83.33%), predominantly involving the middle cerebral artery (41.67%), resolving in 20.84% of cases and progressing in 33.33% of cases. Lesions were commonly unilateral (54.17%) and stenotic (75%), resulting predominantly in cortical infarcts (45.83%), with hemiparesis being the most common neurodeficiency. Apart from tubercular meningitis patients, others had a good functional outcome. CONCLUSION: Lower age, minor infections, and unilateral arteriopathies had a significantly higher chance of resolution. Postviral arteriopathies had a significantly lower chance of progression compared with those following bacterial infections. Progressive and bilateral arteriopathies were significantly associated with worse outcomes and recurrent strokes.

Atypical presentations of hydatid cyst in children and their management.

Hydatid cyst (HC) is uncommon in children and usually involves a single organ, the lung being the most common site followed by the liver. A series of 18 children who presented with HC at different organs of the body managed at our institute over a period of 5 years is presented here. The clinico-radiological evaluation was done in all patients followed by pharmacotherapy/surgery and was followed up for 2 years. Demographic data, organs of involvement, clinical presentations and investigations, response to oral albendazole therapy, surgical procedures, operative findings, perioperative clinical courses, and surgical outcomes were recorded. The mean age of presentation was 7.7 years with a male:female ratio of 11:7. All patients were symptomatic at presentation and four (22.22%) had atypical symptoms (obstructive jaundice, bladder outlet obstruction, and acute abdominal pain). Liver HC was more common than lung HC. Three patients (16.6%) had synchronous involvement of the lung and liver/spleen. All the patients underwent surgical excision of the cyst as none of them responded to preoperative pharmacotherapy. Open surgery was done in 15 patients and laparoscopic excision was carried out in three (16.6%). Two patients had abnormal communications (cysto-biliary/cysto-bronchial), which were managed successfully. Neither any major perioperative morbidity nor mortality was nor any recurrence was seen in a 2-year clinical follow-up, no significant perioperative morbidity or mortality occurred, and no recurrence was noted. In conclusion, single organ HC is more common in children, with more prevalence of hepatic than pulmonary HC. Early surgical excision of the cyst should be considered (preferably laparoscopic whenever possible) instead of pharmacotherapy.

Generalized epimerase deficiency galactosemia.

Galactosemia is caused by inherited deficiencies in one of three enzymes involved in the metabolism of galactose: galactose-1-phosphate uridyltransferase (GALT), galactokinase (GALK), and uridine diphosphate galactose-4-epimerase (GALE). The rarest and most poorly understood form of galactosemia is due to epimerase deficiency. We are reporting such a rarest form of galactosemia presenting with progressively increasing cholestatic jaundice and failure to thrive at one month of age. After confirmation of decreased epimerase level in RBC hemolysate, the patient was put on galactose restricted diet and vitamins supplementation, which reversed the clinical signs as well as altered liver function. Patient is on regular follow-up and now at 15 months of age he has no marked developmental delay.

Basal ganglia involvement in a child with herpes simplex encephalitis.

Herpes simplex encephalitis (HSE) is a leading cause of sporadic, nonepidemic viral encephalitis in children and adults. We report a very rare case of HSE with involvement of bilateral thalamus, putamen, upper pons and midbrain, with development of extrapyramidal symptoms which responded to corticosteroid therapy. A 15-mth-old female baby admitted with complaint of fever for 5 days and generalised tonic clonic seizure 10 hours before admission. On clinical examination patient was drowsy, temperature was 39.4 degrees C and vitals were stable with signs of increased intracranial tension. There were no signs of meningeal irritation. Patient gradually become unconscious in the next few hours and pupils were constricted bilaterally with development of atonia in all four limbs and neck muscles. Doll's eye phenomenon was absent.