Does ensuring optimum vitamin D levels result in early resolution of neurocysticercosis?

BACKGROUND: Neurocysticercosis is a leading cause of acquired epilepsy. Calcified granulomas are known to cause seizure recurrence. Researchers have reported that vitamin D deficiency is associated with brain calcification and reduction in calcification occurs with vitamin D receptor agonist calcitriol through upregulation of SLC20A2. Based on these observations, a hypothesis was proposed that the occurrence of calcification could be reduced by optimizing vitamin D levels, resulting in early resolution of neurocysticercosis. METHODOLOGY: A case-control (retrospective and prospective) study on 60 children with solitary intraparenchymal neurocysticercosis, 20 new cases prior to starting cysticidal therapy and other 40 resolved cases was carried out. Among new cases, children deficient in vitamin D were given megadose of vitamin D and vitamin D levels were rechecked after 30 days. Children having normal vitamin D were taken as cases and the deficient ones were taken as controls. Standard treatment for neurocysticercosis was given. Three monthly MRI scans were done. Outcome was evaluated as resolution/persistence of neurocysticercosis at 3, 6, 9 and 12 months. STATISTICS AND RESULTS: Pearson chi square/Fisher's exact test was used along with Kaplan Meier and log rank test. Of 60 patients, at 6 months 3 cases and 4 controls (p value 0.43), at 9 months 2 cases and 6 controls (p value 0.037) and at 12 months 3 cases and 6 controls (p value 0.029) had complete resolution of NCC. CONCLUSION: The results do not show that adequate vitamin D levels result in early resolution of neurocysticercosis.

Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome.

Dystonia is a movement disorder characterized by involuntary sustained or intermittent muscle contraction causing repetitive twisting movements and abnormal postures. Status dystonicus (SD) is an enigmatic disease of cryptic etiology. We hereby report a child with West syndrome (WS) who went on to develop SD following intramuscular adrenocorticotropic hormone (ACTH) injection. An 11-month-old male child presented with complaints of flexor spasms for 2 months. The diagnosis of WS was confirmed by electroencephalography (EEG), which showed hypsarrhythmia. Intramuscular ACTH was added, and oral trihexyphenidyl was started for dystonia. On day 7 of ACTH, the child developed frequent opisthotonic posturing. Management protocol for grade IV SD was initiated. Administration of N-terminal of ACTH in rat locus coeruleus has been shown to produce human dystonia-like movement and abnormal posturing. How to cite this article: Singh J, Aulakh R. Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome. Indian J Crit Care Med 2022;26(8):961-962.

Arterial Ischemic Stroke in a Child with Internal Carotid Artery Hypoplasia and Protein S Deficiency.

Congenital heart diseases, hemolytic anemia, collagen vascular diseases, and neurometabolic disorders are known to be associated with the development of arterial ischemic stroke (AIS) in children. However, not all the children with the aforementioned conditions develop AIS. Hence, the possibility of interplay of various predisposing factors in children with inherent underlying medical conditions seems likely. Trauma, infections, and thrombophilic risk factors are well known to predispose to the development of AIS in children. Congenital hypoplasia of internal carotid artery (HICA) is a rare disorder in which patient may remain asymptomatic or may present with symptoms due to cerebrovascular insufficiency or compression by collateral vessels. We report a 7-year-old boy who presented with AIS and had a history of two transient ischemic attacks in the past 2 years. He was diagnosed to have congenital HICA. However, further evaluation revealed the presence of protein S deficiency as well. This case reemphasizes the need for investigation into multifactorial causation of pediatric AIS in every case.

Pediatric Tubercular Meningitis: A Review.

Childhood tuberculosis (TB) has a high incidence and prevalence in developing countries like India with tubercular meningitis (TBM) being the most common cause of death. Most cases of TBM are diagnosed late when despite adequate therapy; morbidity and mortality continue to remain high. This review aims to provide a pragmatic approach at dealing with cases of tubercular meningitis in children including clinical features, laboratory and radiological criteria, treatment options and prognostic implications. The objective of this review is to assist in early identification, proper investigation and timely treatment of TBM in children in order to reduce neurological morbidity and mortality associated with it.

Mortality in children with juvenile dermatomyositis: two decades of experience from a single tertiary care centre in North India.

Survival and outcomes have improved considerably among patients with juvenile dermatomyositis (JDM) in the west. However, mortality continues to be high in the developing world. There is paucity of literature on this aspect of JDM from developing countries. We reviewed case files of all patients with JDM registered in the Pediatric Rheumatology Clinic, Advanced Pediatrics Centre at the Post Graduate Institute of Medical Education and Research, Chandigarh, during the period 1993-2013. Seventy-six children were diagnosed to have inflammatory myopathy during this period. Of these, 63 had JDM, 3 had polymyositis while 10 had an overlap syndrome. We had reported 2 deaths out of 33 (8.3 %) patients with JDM in 2004, and over the last 9 years, we have encountered five more deaths in this group, thereby accounting for a mortality rate of 11.1 % (7/63) over two decades of follow-up. In these five children now being described, the mean duration between onset of symptoms and institution of appropriate therapy was 9.2 months. Four children (80 %) had severe muscle weakness needing nasogastric tubes at the onset, three (60 %) had cutaneous ulcers and three (60 %) had superadded infections. Two children (40 %) had gastrointestinal vasculitis and one of these developed an intestinal perforation. Three patients (60 %) had progressive pulmonary disease and air leak was identified in two of them. Although the prognosis for survival in JDM has steadily improved, in our experience the disease remains a serious illness and still carries significant mortality in the context of a developing country.

Kawasaki disease and the emerging coronary artery disease epidemic in India: is there a correlation?

Although Kawasaki disease (KD) is now being increasingly reported from India, the vast majority of children with KD are still not being diagnosed and treated. A recent study from Chandigarh has shown that the incidence of KD is at least 4.54/100,000 children below 15 y of age. Extrapolations of this figure suggest that a minimum of 17,417 new cases of KD would be occurring every year in our country. A significant proportion of these children may develop coronary artery abnormalities. These children would then be at risk of developing myocardial ischemia as young adults. It is authors' contention that (undiagnosed) KD in childhood may be contributing to the growing pool of coronary artery disease (CAD) in India. Similarly, a missed diagnosis of KD in childhood should be considered as a possibility while evaluating adults with CAD, especially when there are no overt risk factors and no family history of the disease.

Subacute sclerosing panencephalitis in a toddler: changing epidemiological trends.

Subacute sclerosing panencephalitis (SSPE) is a devastating "slow virus" brain disease resulting from persistent measles virus infection of neurons. The age at presentation is usually 8 to 11 years with onset usually occurring 2-10 years after measles infection. We report a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline. He was diagnosed as a case of SSPE based on clinical features, typical electroencephalographic finding, and elevated cerebrospinal fluid/serum measles antibody titers. He had measles 4 months prior to onset of symptoms. This case along with review of recently published reports suggests progressively decreasing latency period between measles infection and onset of symptoms observed in cases with SSPE. Clinical implication would mean investigating for SSPE even in infants or toddlers with compatible clinical features and recent history of measles infection.

Syndrome of hepatic cirrhosis, dystonia, polycythemia, and hypermanganesemia caused by mutations in SLC30A10, a manganese transporter in man.

Environmental manganese (Mn) toxicity causes an extrapyramidal, parkinsonian-type movement disorder with characteristic magnetic resonance images of Mn accumulation in the basal ganglia. We have recently reported a suspected autosomal recessively inherited syndrome of hepatic cirrhosis, dystonia, polycythemia, and hypermanganesemia in cases without environmental Mn exposure. Whole-genome mapping of two consanguineous families identified SLC30A10 as the affected gene in this inherited type of hypermanganesemia. This gene was subsequently sequenced in eight families, and homozygous sequence changes were identified in all affected individuals. The function of the wild-type protein and the effect of sequence changes were studied in the manganese-sensitive yeast strain Δpmr1. Expressing human wild-type SLC30A10 in the Δpmr1 yeast strain rescued growth in high Mn conditions, confirming its role in Mn transport. The presence of missense (c.266T>C [p.Leu89Pro]) and nonsense (c.585del [p.Thr196Profs(∗)17]) mutations in SLC30A10 failed to restore Mn resistance. Previously, SLC30A10 had been presumed to be a zinc transporter. However, this work has confirmed that SLC30A10 functions as a Mn transporter in humans that, when defective, causes Mn accumulation in liver and brain. This is an important step toward understanding Mn transport and its role in neurodegenerative processes.

Is Kawasaki disease incidence rising in Chandigarh, North India?

OBJECTIVE: To estimate incidence of Kawasaki disease (KD) over time among children in the city of Chandigarh, North India. PATIENTS AND METHODS: We analysed records of all children with KD below 15 years of age at the Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, from January 1994 to December 2008. Diagnosis and treatment of KD were based on American Heart Association criteria. Among these cases, children residing in Chandigarh were identified. Yearly incidence was calculated and compared from 1994 to 2008. RESULTS: During this period, 196 children were diagnosed as KD. Of these, 80 (40.8%; 48 boys, 32 girls) resided in Chandigarh. Four among these had coronary artery abnormalities (CAA) on echocardiography, while two had mitral regurgitation. Comparison of yearly data revealed increasing incidence of disease from 0.51 cases in 1994 to 4.54 cases per 100,000 children below 15 years of age in 2007. Majority (93.7%) of cases occurred in children ≤10 years of age with the highest incidence reported in the seventh year of life. Monthly distribution of disease showed two peaks with a maximum number of cases presenting in October followed by a second peak in May with a nadir in the month of February. CONCLUSION: Increasing incidence of KD in Chandigarh could be due to increasing clinical recognition as a result of greater awareness among paediatricians in the city, or may represent an actual increase in numbers. Striking differences from KD series reported from other countries include the older median age of our patients, low rate of CAA and a different bimodal seasonality, which may be epidemiologic clues to the nature of this vasculitis.

Kawasaki disease and Henoch Schonlein purpura: changing trends at a tertiary care hospital in north India (1993-2008).

The objective of the study was to evaluate the epidemiologic trends (1993-2008) in Kawasaki disease (KD) and Henoch Schonlein purpura (HSP) among children as seen in a tertiary care north Indian hospital. A hospital-based retrospective chart analysis of all the patients diagnosed with KD and HSP in our pediatric rheumatology clinic from January 1993 to December 2008 was performed. The diagnosis of KD and HSP was based on the American Heart Association and American College of Rheumatology criteria respectively. The collected data were analyzed for any yearly or seasonal trends in the incidence of the two diseases. KD was diagnosed in 196 patients (135 males, 61 females, ratio 2.21:1), while HSP was diagnosed in 209 patients (155 males, 54 females, ratio 2.87:1). The median age at the time of diagnosis was 5.1 +/- 3.04 years (range 4 months-14 years) for KD and 6.9 +/- 2.98 years (range 1-17 years) for HSP. Over this 16-year period, the number of patients diagnosed with KD progressively increased from 1993 to 2008 (p < 0.001, chi-square test) with a clear majority of these (162 cases, 82.6%) being diagnosed in the last 8 years. The number of children diagnosed with HSP progressively increased from 1993 to 1997, after which it has remained relatively uniform, and only 93 cases (44.5%) were diagnosed in the last 8-year period (p > 0.05, chi-square test). However, both the diseases exhibited a comparable seasonal trend in the distribution with a noticeable peak being discernible in the months of October and November. KD has shown a rising trend over the recent years in our hospital. Since 2004; the annual number of KD cases has outnumbered the HSP cases. Seasonal predilection is noticeable for both conditions. This is the first hospital-based report on epidemiological trends of KD and HSP from a developing country.

Spontaneous hematomyelia in a child with hemophilia A: a case report.

Hemorrhagic complications in patients with hemophilia have been occasionally reported in the spinal column and the spinal cord. Treatment is based on prompt replacement therapy as the occurrence and development of neurologic dysfunction are related to the length of time between the onset of symptoms and the factor replacement. We report case of a 7-year-old hemophilic boy who presented with flaccid paraparesis resulting from thoracic hematomyelia. The patient showed gradual improvement on medical management with cryoprecipitate infusions. This case calls attention to the need for prompt diagnosis of rarely reported spinal hematomyelia based on clinical manifestations and radiologic features and highlights its management options in patients with hemophilia.

Anomalous bilateral lateral rectus muscles and anterior polar cataract with dysmorphic features.

Congenital anomalies, such as absence, hypoplasia, bifurcation, or reduplication of various extraocular muscles, including lateral rectus muscles have been reported previously. We report a 5-year-old girl who had posteriorly malinserted lateral rectus at 15 mm from the limbus in the right eye with multiple associated ocular and systemic congenital malformations.

Red cell distribution width (RDW) in the diagnosis of iron deficiency with microcytic hypochromic anemia.

OBJECTIVE: To study the utility of red cell distribution width (RDW) in the diagnosis of iron deficiency among children with microcytic hypochromic anemia. METHODS: 151 children (6 months-12 years) with microcytic (MCV<75 fl) anemia were classified into iron deficient (IDA) and non-iron deficient anemia (non-IDA) on the basis of serum ferritin and total iron binding capacity (TIBC). RDW values were obtained on an automated hematology analyzer. Receiver operator curves (ROC) were constructed and the utility of RDW in diagnosis of iron deficiency was studied. RESULTS: The mean RDW value was 18.37+/-2.22% in IDA group (97 children) compared to 16.55+/-1.51 % in the non-IDA group (54 children) (p<0.0001, unpaired t test). In IDA group, the mean RDW value was 16.60+/-1.78%, 17.95+/-1.91% and 20.55+/-1.32% among mild, moderate and severely anemic children (p<0.0001, ANOVA test). The corresponding values in non-IDA group were 16.03+/-1.25%, 16.76+/-1.20% and 16.77+/-2.68% respectively (p=0.269, ANOVA test). At a cut-off value of 17.4%, as obtained from the ROC curve, the sensitivity and specificity of RDW in diagnosis of IDA were 81.0% and 53.4% and a positive and negative predictive value of 63.0% and 72.2% respectively. CONCLUSION: RDW has a limited specificity for diagnosis of IDA among children with microcytic hypochromic anemia.