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2015| October-December | Volume 10 | Issue 4
Online since
January 20, 2016
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REVIEW ARTICLE
Autism and sleep disorders
Preeti A Devnani, Anaita U Hegde
October-December 2015, 10(4):304-307
DOI
:10.4103/1817-1745.174438
PMID
:26962332
“Autism Spectrum Disorders” (ASDs) are neurodevelopment disorders and are characterized by persistent impairments in reciprocal social interaction and communication. Sleep problems in ASD, are a prominent feature that have an impact on social interaction, day to day life, academic achievement, and have been correlated with increased maternal stress and parental sleep disruption. Polysomnography studies of ASD children showed most of their abnormalities related to rapid eye movement (REM) sleep which included decreased quantity, increased undifferentiated sleep, immature organization of eye movements into discrete bursts, decreased time in bed, total sleep time, REM sleep latency, and increased proportion of stage 1 sleep. Implementation of nonpharmacotherapeutic measures such as bedtime routines and sleep-wise approach is the mainstay of behavioral management. Treatment strategies along with limited regulated pharmacotherapy can help improve the quality of life in ASD children and have a beneficial impact on the family. PubMed search was performed for English language articles from January 1995 to January 2015. Following key words: Autism spectrum disorder, sleep disorders and autism, REM sleep and autism, cognitive behavioral therapy, sleep-wise approach, melatonin and ASD were used. Only articles reporting primary data relevant to the above questions were included.
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ORIGINAL ARTICLES
Extended endoscopic endonasal transsphenoidal approach for retrochiasmatic craniopharyngioma: Surgical technique and results
Suresh K Sankhla, Narayan Jayashankar, Ghulam M Khan
October-December 2015, 10(4):308-316
DOI
:10.4103/1817-1745.174457
PMID
:26962333
Objective:
Surgical treatment of retrochiasmatic craniopharyngioma still remains a challenge. While complete removal of the tumor with preservation of the vital neurovascular structures is often the goal of the treatment, there is no optimal surgical approach available to achieve this goal. Transcranial and transsphenoidal microsurgical approaches, commonly used in the past, have considerable technical limitations. The extended endonasal endoscopic surgical route, obtained by removal of tuberculum sellae and planum sphenoidale, offers direct midline access to the retrochiasmatic space and provides excellent visualization of the undersurface of the optic chiasm. In this report, we describe the technical details of the extended endoscopic approach, and review our results using this approach in the surgical management of retrochiasmatic craniopharyngiomas.
Methods:
Fifteen children, including 9 girls and 6 boys, aged 8 to 15 years underwent surgery using extended endoscopic transsphenoidal approach between 2008 and 2014. Nine patients had a surgical procedure done previously and presented with recurrence of symptoms and regrowth of their residual tumors.
Results:
A gross total or near total excision was achieved in 10 (66.7%) patients, subtotal resection in 4 (26.7%), and partial removal in 1 (6.7%) patient. Postoperatively, headache improved in 93.3%, vision recovered in 77.3%, and the hormonal levels stabilised in 66.6%. Three patients (20%) developed postoperative CSF leaks which were managed conservatively. Three (20%) patients with diabetes insipidus and 2 (13.3%) with panhypopituitarism required long-term hormonal replacement therapy.
Conclusions:
Our early experience suggests that the extended endonasal endoscopic approach is a reasonable option for removal of the retrochiasmal craniopharyngiomas. Compared to other surgical approaches, it provides better opportunities for greater tumor removal and visual improvement without any increase in risks.
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CASE REPORTS
Nonketotic hyperglycinemia case series
Mehtab Iqbal, Manish Prasad, Santosh R Mordekar
October-December 2015, 10(4):355-358
DOI
:10.4103/1817-1745.174445
PMID
:26962342
To present three cases who presented with neonatal hiccups and who were later diagnosed with nonketotic hyperglycinemia (NKH). Case series. We present three babies who presented in neonatal life with hiccups who later were diagnosed with NKH. Two babies presented on the 2
nd
day of life with hypotonia, poor feeding, and abnormal movements including jitteriness, hiccups, and twitching. The third baby only had transient hiccups lasting for a couple of days in the 1
st
week of life but later presented at 3 months of age with poor feeding, drowsiness, and jerky movements. All three cases needed extensive investigations before reaching the diagnosis including metabolic screen, lumbar puncture, electroencephalography, and computed tomography/magnetic resonance imaging. The first two babies needed intubation on their 2
nd
day of life because of apneas in whom later, the care was withdrawn after reaching the diagnosis of NKH because of poor prognosis. The third baby was discharged home on oral dextromethorphan and ketogenic diet. We discuss the importance of early recognition of symptoms (frequent hiccups) and investigation needed to reach the diagnosis early as it helps in making decision to either carry on treatment or withdraw care because of poor prognosis. It also helps in genetic counseling and prenatal diagnosis can be offered at the subsequent pregnancy.
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Primary intraventricular central nervous system lymphoma in an immunocompetent patient
Vinit Suri, Venkatesh Mittapalli, Manish Kulshrestha, Kaushal Premlani, SK Sogani, Kunal Suri
October-December 2015, 10(4):393-395
DOI
:10.4103/1817-1745.174433
PMID
:26962354
We report a young 15-year-old boy with 6 months history of headache, vomiting, and seizure. He underwent septostomy followed by right ventriculoperitoneal shunt for obstructive hydrocephalus and was managed with empirical antituberculosis treatment. Magnetic resonance imaging (MRI) revealed solid, nodular, enhancing masses in bilateral lateral ventricles and 4
th
ventricle. Surgical biopsy from 4
th
ventricular lesion confirmed a B-cell lymphoma. Staging evaluation with MRI positron emission tomography and bone marrow biopsy were normal suggesting an intraventricular primary central nervous system lymphoma.
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ORIGINAL ARTICLES
Brain tumors in infants
Seyyed Mohammad Ghodsi, Zohreh Habibi, Sara Hanaei, Ehsan Moradi, Farideh Nejat
October-December 2015, 10(4):335-340
DOI
:10.4103/1817-1745.174454
PMID
:26962338
Background:
Brain tumors in infants have different clinical presentations, anatomical distribution, histopathological diagnosis, and clinical prognosis compared with older children.
Materials and Methods:
A retrospective analysis was done in patients <12 months old who were operated on for primary brain tumor in Children's Hospital Medical Center since 2008 to 2014.
Results:
Thirty-one infants, 20 males and 11 females, with the mean age of 7.13 months (0.5–12) were enrolled. There were 16 supratentorial and 15 infratentorial tumors. The presenting symptoms included increased head circumference (16); bulge fontanel (15); vomiting (15); developmental regression (11); sunset eye (7); seizure (4); loss of consciousness (4); irritability (3); nystagmus (2); visual loss (2); hemiparesis (2); torticollis (2); VI palsy (3); VII, IX, X nerve palsy (each 2); and ptosis (1). Gross total and subtotal resection were performed in 19 and 11 cases, respectively. Fourteen patients needed external ventricular drainage in the perioperative period, from whom four infants required a ventriculoperitoneal shunt. One patient underwent ventriculoperitoneal shunting without tumor resection. The most common histological diagnoses were primitive neuroectodermal tumor (7), followed by anaplastic ependymoma (6) and grade II ependymoma. The rate of 30-day mortality was 19.3%. Eighteen patients are now well-controlled with or without adjuvant therapy (overall survival; 58%), from whom 13 cases are tumor free (disease free survival; 41.9%), 3 cases have residual masses with fixed or decreased size (progression-free survival; 9.6%), and 2 cases are still on chemotherapy.
Conclusion:
Brain tumors in infants should be treated with surgical resection, followed by chemotherapy when necessary.
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Pediatric encephaloceles: A series of 20 cases over a period of 3 years
Shashank Ravindra Ramdurg, M Sukanya, Jayabrata Maitra
October-December 2015, 10(4):317-320
DOI
:10.4103/1817-1745.174462
PMID
:26962334
Background:
Encephalocele is the protrusion of the cranial contents beyond the normal confines of the skull through a defect in the calvarium and is far less common than spinal dysraphism. The exact worldwide frequency is not known.
Aims and Objectives:
To determine the epidemiological features, patterns of encephalocele, and its postsurgical results.
Materials and Methods:
The study was carried from year July 2012 to June 2015. Patients with encephalocele were evaluated for epidemiological characteristics, clinical features, imaging characteristics, and surgical results.
Results:
20 encephaloceles patients were treated during the study period. Out of these 12 (60%) were male and 8 (40%) female. Age range was 1 day to 6 years. The most common type of encephalocele was occipital 12 (60%), occipito-cervical 4 (20%), parietal 2 (10%), fronto-nasal 1 (5%), and fronto-naso-ethmoidal 1 (5%). One patient had a double encephalocele (one atretic and other was occipital) with dermal sinus tract and limited dermal myeloschisis. Other associations: Chiari 3 malformation (2), meningomyeloceles (4), and syrinx (4). Three patients presented with rupture two of whom succumbed to meningitis and shock. Seventeen patients treated surgically did well with no immediate surgical mortality (except a case of Chiari 3 malformation who succumbed 6 months postsurgery to unrelated causes). Shunt was performed in 4 cases.
Conclusion:
The most common type of encephalocele is occipital in our set up. Early surgical management of encephalocele is not only for cosmetic reasons but also to prevent tethering, rupture, and future neurological deficits.
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Diphtheritic polyneuropathy in the wake of resurgence of diphtheria
D Manikyamba, A Satyavani, P Deepa
October-December 2015, 10(4):331-334
DOI
:10.4103/1817-1745.174441
PMID
:26962337
Objective:
To study the clinical profile and outcome in children with diphtheritic polyneuropathy (DP).
Methodology:
13 children with polyneuropathy were included in this study. Their demographic profile, age, sex and immunization status were recorded. Detailed clinical and neurological examination was done. Investigations like CSF analysis, NCV studies, MRI brain were done. The results were tabulated and analyzed.
Results:
All the children presented with bulbar palsy and had h/o membranous tonsillitis. Isolated palatal palsy was seen in 7 children (53%). 6 (46.1%) children developed quadriparesis. 1 child expired and recovery is complete in rest of the 12 children. Children with isolated bulbar palsy recovered within 2 to 4 weeks while children with quadriparesis recovered within 5-6 wks.
Conclusions:
Any child diagnosed with diphtheria should be followed for 3-6 months in anticipation of neurological complications. DP carries good prognosis hence timely diagnosis and differentiation from other neuropathies is a prerequisite for rational management.
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CASE REPORTS
Atypical teratoid rhabdoid tumor of the lateral ventricle
Mehdi Darmoul, Atef Ben Nsir, Imene Chabchoub, Mohamed Nejib Hattab
October-December 2015, 10(4):382-385
DOI
:10.4103/1817-1745.174455
PMID
:26962351
Atypical teratoid/rhabdoid tumors (AT/RTs) are rare and highly malignant embryonal central nervous system neoplasms, usually seen in very young children with rapid fatal outcome despite aggressive treatment. They are most commonly located in the posterior fossa. Intraventricular location is extremely rare. To the best of our knowledge, only 4 cases of lateral ventricle location were reported in the literature. We report the fifth case of lateral ventricle AT/RT in a 2-month-old male who presented with rapid increase of his head circumference. Brain computed tomography scan and magnetic resonance imaging showed heterogeneous huge mass within the left lateral ventricle extending to the parieto-occipital parenchyma and markedly enhancing by contrast. The baby underwent left transparietal approach with complete removal of the tumor. Histological examination confirmed the diagnosis of AT/RT. An aggressive chemotherapy was administrated postoperatively. The outcome is good without neurological deficit or recurrence after 3 years and half of follow-up.
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Vesical calculi formation on the slit valves of a migrated distal end of ventriculoperitoneal shunt
Rahul Gupta, Rajan Dagla, Lila Dhar Agrawal, Pramila Sharma
October-December 2015, 10(4):368-370
DOI
:10.4103/1817-1745.174444
PMID
:26962346
Various complications of distal end of the ventriculoperitoneal (VP) shunt have been described in the literature. We present, here, an extremely rare and potentially severe complication of vesical calculi formation on the slit valves of distal end of VP shunt which erosively migrated into the urinary bladder. Suprapubic cystolithotomy performed, peritoneal end of the tube found to be eroding and entering into the bladder with two calculi firmly stuck to slit valves in the distal end of the tubing were removed. Shunt was functional, therefore, it was pulled out and repositioned on the superior aspect of the liver; the urinary bladder was repaired. Patient did well postoperatively. This complication was revealed 1.5 years after the shunt was implanted. Although there were symptoms of dysuria and dribbling of urine of short duration, the patient did not show obvious peritoneal signs; suggesting that, penetration of a VP shunt into the urinary bladder can remain asymptomatic for a long period of time, disclosed late and can lead to considerable morbidity. Careful follow-up is important and management should be individualized.
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A pediatric chronic subdural hematoma: More than meets the eye
Dan Wright, Erlick A. C Pereira, Shailendra A Magdum
October-December 2015, 10(4):376-378
DOI
:10.4103/1817-1745.174451
PMID
:26962349
We present an unusual case of a 13-year-old child who following minor head injury presented with what appeared to be a thin chronic subdural hematoma on plain computed tomography imaging. The child was found to have an underlying arachnoid cyst. Intra- and extra-cystic bleeding had occurred simultaneously causing an isodense cyst with an accompanying subdural collection. This radiographically occult pathology should be excluded using magnetic resonance imaging in any child presenting with a subdural collection.
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A case report of pontine glioblastoma presenting as subcutaneous metastasis in nape of neck in a child
Vikas Kumar, Daljit Singh, Hukum Singh, Ravindra Kumar Saran
October-December 2015, 10(4):386-388
DOI
:10.4103/1817-1745.174458
PMID
:26962352
Glioblastoma (GBM) is the most common malignant tumor in adults. Extracranial metastasis of GBM is very rare. The incidence of brainstem glioblastoma is not known due to low biopsy and resection rates. In this case report, we experienced an 11-year-old male who was diagnosed as a case of pontine GBM after biopsy of lesion and underwent radiotherapy with adjuvant chemotherapy. He presented with a subcutaneous swelling in the nape of neck 1 year after the first procedure. Swelling was excised. Pathological examination and immunohistochemical staining confirmed it as GBM. This case shows us that GBM can at times present as a swelling in soft tissue.
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Single staged complete length excision of the holocord ependymoma: Team work
Kamlesh S Bhaisora, Pradeep Sharma, Arun Kumar Srivastava, Anant Mehrotra, Kuntal Kanti Das, Jayesh Sardhara, Sanjay Behari, AK Jaiswal, RN Sahu
October-December 2015, 10(4):396-398
DOI
:10.4103/1817-1745.174450
PMID
:26962355
The authors present a case of a 15-year-old male patient who presented with gradually progressive quadriparesis for 3 years. Magnetic resonance imaging of the spine was suggestive of heterogeneously enhancing mass lesion extending from cervicomedullary junction to conus. This holocord spinal tumor was excised in a single stage with standard microsurgical technique. In immediate postoperative period, the patient had deterioration in power in both lower limbs which improved in follow-up at 6 months. Histopathology of the tumor was suggestive of ependymoma. Holocord ependymoma is a rare entity; until now, only six cases have been described in the literature. To the author's best knowledge, this is only the second case of holocord ependymoma excised in a single stage.
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ORIGINAL ARTICLES
A clinical study to identify the possible etiology of complex partial seizures using magnetic resonance imaging brain findings and its implications on treatment
V Nancy Jeniffer, S Udayakumar, K Pushpalatha
October-December 2015, 10(4):350-354
DOI
:10.4103/1817-1745.174435
PMID
:26962341
Context:
Epilepsy is one of the common neurological disorders worldwide. Fundamentally, there are two types of epilepsy—primary generalized epilepsy and localization-related epilepsy. Partial seizures account for about 40% of childhood seizures in some series and can be classified as simple or complex.
[1]
Partial seizures, more so the complex partial seizures (CPSs), are presumed to have a structural etiology.
Aims:
(1) To study the magnetic resonance imaging (MRI) brain findings in CPSs in children aged 1–18 years. (2) To identify treatable causes of CPSs based on MRI findings and institute appropriate treatment.
Statistical Analysis:
Statistical analysis was performed using percentages and proportions.
Methods:
Hospital based prospective study in which MRI brain was done on all newly diagnosed children with complex partial seizures, aged 1 to 18 yrs, during the study period. Final diagnosis was made correlating clinical features, radiological features and other supportive evidences, and appropriate treatment instituted. Follow up of cases was done until the completion of treatment (maximum 6 months).
Results:
Among the 64 children who were clinically diagnosed to have CPSs and subjected to MRI study of the brain, 40(62.5%) children were detected to have structural lesions, of which neurocysticercosis (NCC) was noted in 17 (42.5%), tuberculoma in 12 (30%), hippocampal sclerosis (HS) in 6 (15%), gliosis in 4 (10%), and tumor in 1 (2.5%) patient. Sixty-two (96.8%) children were treated medically, and 2 (3.2%) children underwent surgery.
Conclusions:
Etiology of CPS based on MRI findings showed a substantial number of medically- and surgically-treatable pathologies. This study done on South Indian children showed neuro infections to be the most common cause of CPS, followed by HS, with NCC being the most common lesion noted. MRI not only identifies specific epileptogenic substrates, but also determines the specific treatment and predicts prognosis and should be the imaging modality of choice in the evaluation of CPS.
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3,509
137
The relationship between obesity and symptomatic Chiari I malformation in the pediatric population
Sandi Lam, Brenda Auffinger, Matthew Tormenti, Christopher Bonfield, Stephanie Greene
October-December 2015, 10(4):321-325
DOI
:10.4103/1817-1745.174443
PMID
:26962335
Background:
Concomitant with the rise in childhood obesity in the United States is an increase in the diagnosis of Chiari I malformation (CM1).
Objective:
To discern a correlation between obesity and CM1, defined as >5 mm of cerebellar tonsillar descent on sagittal magnetic resonance imaging.
Methods:
Charts of CM1 patients aged 2–20 years were retrospectively reviewed. Chiari size, age, body mass index (BMI), and CM1 signs/symptoms were recorded. Patients were stratified by age: 2–9, 10–14, and 15–20 years. Mixed-effect linear models and linear regression analysis were applied to investigate the relationship between BMI-for-age percentiles and CM1 signs/symptoms.
Results:
One hundred sixty-seven patients were included (mean age 14.5 ± 2.97 years, BMI 22.98 ± 6.5, and Chiari size 12.27 mm ± 5.91). When adjusted for age, 42% were overweight or obese–higher than normative BMI for children in the studied area (29.6%). When stratified by age, patients between 2 and 9 years were most commonly obese and presented the highest mean BMI (25.66), largest Chiari size (13.58), and highest incidence of headache (75%) and syringomyelia (66.67%). Patients between 15 and 20 years were most commonly overweight and presented the smallest Chiari size (11.76 mm), but the highest incidence of cerebellar (50%) and brainstem (8.55%) compression symptoms. A significant positive correlation existed between BMI and headache in the first two age groups: (
R
2
: 0.36,
P
= 0.03;
R
2
: 0.39,
P
= 0.01, respectively). Obese patients had higher incidence of headache in the 10–14 group (
R
2
: 0.37,
P
= 0.02) and the largest Chiari size in the 15–20 group (
R
2
: 0.40,
P
= 0.03).
Conclusions:
The pediatric CM1 population is more likely to be overweight or obese. Younger obese patients presented the highest incidence of Chiari-related headache symptoms, and older obese patients, the highest incidence of findings other than headache. Thus, body weight and age should be considered when evaluating children with CM1.
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CASE REPORTS
Pediatric gliomatosis cerebri mimicking tubercular encephalitis
Namit Singhal, Vinay Agarwal
October-December 2015, 10(4):379-381
DOI
:10.4103/1817-1745.174430
PMID
:26962350
Gliomatosis cerebri (GC) is a diffuse infiltrating glial neoplasm of astrocytic origin. GC in children is rare and difficult to diagnose, often presenting with a variety of signs and symptoms that may mimic myriad conditions. We discuss here the presentation and diagnosis of GC in a child who was initially treated on lines of tubercular encephalitis, with transient clinical relief and disappearance of enhancing component of the disease on magnetic resonance imaging. In this report, we highlight the limitations of clinical presentation and neuroimaging as well as the essential role of histological evaluation for the diagnosis of GC in children. Also is highlighted a more benign and protracted clinical course following radiotherapy in a subset of patients, with Ki index <10%, thereby stressing earliest possible diagnosis. A new prognostic classification can also be proposed for pediatric GC based on various parameters. Since these are rare cases, a combined effort is required for this.
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Cerebellopontine angle arachnoid cyst associated with mirror movements
Esra Gurkas, Buket Yucel Altan, Kivilcim Gucuyener, Ebru Kolsal
October-December 2015, 10(4):371-373
DOI
:10.4103/1817-1745.174440
PMID
:26962347
Arachnoid cysts are benign developmental collections of cerebrospinal fluid (CSF). They constitute approximately 1% of intracranial masses. The cerebellopontine angle (CPA) arachnoid cysts are rare and often asymptomatic. The onset of symptoms and signs is usually due to the compression of the brain, cranial nerves and obstruction of CSF circulation. The major clinical symptoms for CPA arachnoid cysts were reported as headache, ataxia and 8
th
cranial nerve palsy. We report a patient with a CPA arachnoid cyst. He presented with cranial nerve palsies and mirror movements found in upper extremities. We postulated that CPA arachnoid cyst compressing the brain stem and the pyramidal decussation may lead to mirror movements. We conclude that mirror movements can be associated with CPA arachnoid cyst.
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Spondylocostal dysostosis associated with right-sided polythelia, right-sided rib deformity and meningocoele: Case report with review of literature
Narendra Rai, Neha Thakur, Naheed Zia Khan, Akhilesh Dutt Dwivedi
October-December 2015, 10(4):362-364
DOI
:10.4103/1817-1745.174456
PMID
:26962344
Spondylocostal dysostosis (SCD) is a rare anomaly of axial skeleton due to flawed embryological development. A newborn baby boy with right-sided polythelia, meningocoele, fan-like configuration of third and fourth rib, fused fifth and sixth rib and third thoracic hemivertebra was delivered to a second gravid mother by elective caesarean section. Baby was active, cried immediately after birth, had normal muscle tone and reflexes. He passed urine and meconium within 24 h of birth. Based on clinical examination and radiological picture, a diagnosis of SCD with meningocele with right-sided polythelia was made. Our case is the first SCD with polythelia and meningocele in newborn. SCD with right-sided polythelia and rib deformity with neural tube defect (NTD) may be due to a genetic defect with the culprit genes missing. Further genetic and embryological studies might find a link between right-sided rib defects, polythelia and NTD.
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79
Primary parietal myeloid sarcoma
Laxminadh Sivaraju, Dilip Mohan, Nandita Ghosal, Bevinahalli N Nandeesh, Alangar S Hegde
October-December 2015, 10(4):389-392
DOI
:10.4103/1817-1745.174431
PMID
:26962353
Intracranial occurrence of myeloid sarcoma without any evidence of systemic hematological disorder is uncommon. We report the case of a 17-year-old girl who presented with features of raised intracranial pressure and paraparesis of short duration. Magnetic resonance imaging showed a 6 cm bilateral middle 1/3
rd
para sagittal contrast enhancing extra-axial mass with mass effect. The tumor was subtotally excised. Histology and immunohistochemistry proved to be a myelosarcoma. Further evaluation done with peripheral blood smear and bone marrow biopsy ruled out the possibility of leukemia or myeloproliferative disorder. She was referred for chemotherapy and clinically showed improvement after 6 months of follow-up. Authors report a case of intracranial myelosarcoma which closely resembled meningioma both radiologically and in intraoperative morphological appearance. Authors discuss in detail the radiological and histological features of myelosarcoma along with differential diagnoses and treatment options.
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Guillain-Barre syndrome masquerading as acute respiratory failure in an infant
Praveen Kishore, Pradeep Kumar Sharma, Bhaskar Saikia, Praveen Khilnani
October-December 2015, 10(4):399-400
DOI
:10.4103/1817-1745.174461
PMID
:26962356
Guillain-Barré syndrome (GBS) is a rare entity in infants. We report a case of GBS in a 5-month-old girl. The child presented with cough, loose stools, breathing difficulty, and listlessness. The child was treated as pneumonia with respiratory failure. Due to difficulty in weaning from ventilation with areflexia, marked hypotonia, and reduced power in all four limbs; possibilities of spinal muscular atrophy, poliomyelitis, and myopathies were kept. Nerve conduction velocity study was suggestive of mixed sensory-motor, severe axonal, and demyelinating polyradiculoneuropathy. Cerebrospinal fluid study revealed albuminocytological dissociation. Child was diagnosed as GBS and treated with intravenous immunoglobulin. Child recovered completely on follow-up. GBS should be considered as a differential diagnosis in acute onset respiratory failure with neuromuscular weakness in infants.
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3,501
102
Epstein-Barr virus encephalitis with substantia nigra involvement
Tamer Celik, Umit Celik, Orkun Tolunay, Mustafa Komur, Huseyin Baspinar, Cengiz Yilmaz, Gulen Mert, Dincer Yildizdas
October-December 2015, 10(4):401-403
DOI
:10.4103/1817-1745.174436
PMID
:26962357
Infectious mononucleosis due to Epstein–Barr virus (EBV) is a usually benign systemic viral illness common in children. Many studies described nervous system manifestations of infectious mononucleosis with a wide spectrum of neurologic deficits. Neurologic complications of EBV are seen in both acute and reactivate infection. Herein, we describe a patient diagnosed by acute EBV encephalitis with substantia nigra involvement and excellent clinical recovery.
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Paraneoplastic encephalitis as a first evidence of recurrent neuroblastoma: A rare case entity
Suhas Vilasrao Aagre, Apurva Patel, Mukesh Choudhary, Pritam Kataria, Krunal Baldaniya
October-December 2015, 10(4):404-407
DOI
:10.4103/1817-1745.174463
PMID
:26962358
Prevalence of paraneoplastic encephalitis in association with recurrent neuroblastoma (NB) is rare. Here, we report a case of recurrent NB presented as paraneoplastic encephalitis, treated successfully with high dose steroids leading to complete neurological recovery. This case highlights the importance of early recognition, diagnosis, and treatment of paraneoplastic encephalitis in a pediatric patient with the new development of neurological symptoms in the background of NB. Paraneoplastic encephalitis also served as an early sign of disease relapse.
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3,153
112
LETTERS TO THE EDITOR
Treatment-resistant mania in Dandy–Walker malformation with seizure disorder: A case report
Saurabh Baliyan, Amit Arya, Sujita Kumar Kar
October-December 2015, 10(4):413-414
DOI
:10.4103/1817-1745.174447
PMID
:26962361
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ORIGINAL ARTICLES
Survival with concurrent temozolomide and radiotherapy in pediatric brainstem glioma with relation to the tumor volume
Shachi Jain Taran, Rakesh Taran, Manika Batra, Deah Deepak Ladia, Virendra Bhandari
October-December 2015, 10(4):341-345
DOI
:10.4103/1817-1745.174453
PMID
:26962339
Background:
Brainstem gliomas account for approximately 25% of all posterior fossa tumors. In pediatric age group, it constitutes about 10% of all brain tumors. Brainstem glioma is an aggressive and lethal type of malignancy with poor outcome despite all treatments.
Aim:
We studied the incidence and treatment outcome in pediatric patients with brainstem glioma depending on their tumor volume presenting in our institution in last 5 years. Brain tumors comprised 2.95% of all cancers and brainstem gliomas were 8% of all brain tumors.
Materials and Methods:
Nine pediatric patients were included in this analysis, who were treated with localized external radiotherapy 54–59.4 Gy along with temozolomide 75 mg/m
2
during the whole course of radiotherapy.
Results:
The median survival in all these patients was 20 months and the overall 2 years survival is 44.4% (4/9). The median survival of patients with primary disease volume <40cc is 26 months whereas when the volume is more than 40cc the median survival is 13.5 months as calculated by Chi-square test.
Conclusion:
As this study includes a small number of patients with unknown histology and treated on the basis of magnetic resonance imaging findings, no definite opinion can be given as some patients may have a low-grade tumor. More studies are required to establish the relation of size of the tumor with survival.
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122
Merosin-negative congenital muscular dystrophy: Report of five cases
Faruk Incecik, Ozlem M Herguner, Serdar Ceylaner, Sakir Altunbasak
October-December 2015, 10(4):346-349
DOI
:10.4103/1817-1745.174432
PMID
:26962340
Context:
Congenital muscular dystrophy type 1A (MDC1A) is caused by mutations in the laminin α-2 gene encoding laminin-a2.
Aims:
The purpose of this study is to determine clinical and genetic results in five Turkish patients with MDC1A.
Setting and Designs:
Five children with MDC1A were retrospectively analyzed.
Results:
Three (60%) were boys, and 2 (40%) were girls. Parental consanguinity was found in all the families. In all the patients, hypotonia, weakness, delayed motor milestones, markedly elevated creatine phosphokinase (CPK) concentration, and brain white matter abnormalities on magnetic resonance imaging were detected. Mutation analysis was performed in all the patients, and 3 different mutations were detected. However, a mutation in patient 1 and 2 has not been previously described in the literature.
Conclusions:
When a patient presents with severe congenital hypotonia, muscle weakness, high serum CPK levels, and white matter abnormalities, should be suspected as MDC1A.
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CASE REPORTS
A rare case of abdominal compartment syndrome following repair of large myelomeningocele
Rajendra Singh Chouhan, Ashish Bindra, Nitasha Mishra, Neha Hasija, Sumit Sinha
October-December 2015, 10(4):365-367
DOI
:10.4103/1817-1745.174459
PMID
:26962345
Abdominal compartment syndrome is a rare entity that can be life-threatening if not diagnosed and correctly managed in time. We report a case of abdominal compartment syndrome following an apparently uneventful excision and repair of myelomeningocele (MMC). Though MMC is the most complex congenital spinal cord malformation compatible with life and early surgery is recommended to decrease the risk of meningitis and sepsis but generally surgery is safe without major perioperative turbulence. The majority of the skin defects following excision of MMC are repaired by primary skin closure, but large defects may require secondary closure by skin grafts or rotational flaps. We report a case of an infant with large MMC, who developed abdominal compartment syndrome following excision and repair of the swelling. Intraoperatively, it manifested as bradycardia, hypotension, and ventilatory difficulty which were managed successfully. Subsequently, in the postoperative period, the presence of tight abdomen and fall in urine output raised strong suspicion regarding development of abdominal compartment syndrome. Condition of infant improved following the release of flap sutures. A high index of suspicion along with early management is imperative for a successful outcome in such cases.
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4,041
98
Delayed diagnosis of Gorlin syndrome: Learning from mistakes!
Subramaniyan Ramanathan, Devendra Kumar, Mahmoud Al Heidous, Yegu Palaniappan
October-December 2015, 10(4):359-361
DOI
:10.4103/1817-1745.174437
PMID
:26962343
Gorlin syndrome (GS) is a rare inherited multisystem disorder with predisposition to basal cell carcinomas and various other neoplasms. Characteristic features include falx calcification, multiple odontogenic keratocysts (OKCs), early onset medulloblastoma, craniofacial and skeletal malformations, cardiac and ovarian fibroma. We present a case of GS in a 9-year-old girl with recurrent dental infections which was overlooked for 8 years. Diagnosis was finally suggested by the incidental detection of multiple OKCs and ovarian fibromas on follow-up magnetic resonance imaging performed for surveillance of previous operated brain tumor.
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3,487
87
Occipital lobe seizures: Rare hyperglycemic sequelae of type 1 diabetes mellitus
Muhammed Jasim Abdul Jalal, Murali Krishna Menon, K Arun Kumar, Ramesh Gomez
October-December 2015, 10(4):374-375
DOI
:10.4103/1817-1745.174449
PMID
:26962348
A 15-year-old boy presented with osmotic symptoms and photopsia. He had short-term memory impairment, visual hallucinations, and headache. His random blood sugar was 474 mg/dl, HbA1c −9.4%, and glutamic acid decarboxylase −65 >2000 IU/ml. Magnetic resonance imaging brain and cerebrospinal fluid study were normal. Digital electroencephalography was suggestive of bilateral hemispheric occipital lobe seizures. He responded well to insulin and antiepileptic medications.
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FROM THE EDITORS DESK
From the Editor's desk
VP Singh
October-December 2015, 10(4):303-303
DOI
:10.4103/1817-1745.174464
PMID
:26962331
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2,942
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LETTERS TO THE EDITOR
Hypergonadotrophic hypogonadism and cerebellar ataxia in an Indian adolescent: A rare report
Rohan R Mahale, Anish Mehta, Madhusudhan B Kempegowda, Suryanarayana Sharma, Mahendra Javali, Srinivasa Rangasetty
October-December 2015, 10(4):414-415
DOI
:10.4103/1817-1745.174442
PMID
:26962362
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2,271
71
A rare cause of occipital headache
Lokesh Saini, Biswaroop Chakrabarty, Sheffali Gulati, Atin Kumar
October-December 2015, 10(4):416-417
DOI
:10.4103/1817-1745.174448
PMID
:26962363
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2,310
73
Febrile seizures in 1–5 aged infants in tropical practice: Frequency, etiology and outcome of hospitalization
Mahmood Dhahir Al-Mendalawi
October-December 2015, 10(4):417-418
DOI
:10.4103/1817-1745.174446
PMID
:26962364
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94
Concurrent acute disseminated encephalomyelitis and Guillain–Barré syndrome in a child
Mahmood Dhahir Al-Mendalawi
October-December 2015, 10(4):418-419
DOI
:10.4103/1817-1745.174460
PMID
:26962365
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80
Diffuse leptomeningeal spread of supratentorial recurrent pilocytic astrocytoma in a child
Amol Raheja, Pankaj Kumar Singh, Aruna Nambirajan, Mehar Chand Sharma, Bhawani Shankar Sharma
October-December 2015, 10(4):408-411
DOI
:10.4103/1817-1745.174452
PMID
:26962359
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2,746
76
Faun tail associated with bony tail like projecting dysplastic sacral vertebral segments in natal cleft: Unique twin tails
Dutta Guru Satyarthee, AK Mahapatra
October-December 2015, 10(4):411-412
DOI
:10.4103/1817-1745.174439
PMID
:26962360
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2,636
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ORIGINAL ARTICLES
Does diffusion restriction changes in magnetic resonance imaging predict neurological outcome in neonatal seizures?
Manipriya Ravindran, Prakash Amborium, B Umamaheswari, Gokul Ramani, Binu Ninan
October-December 2015, 10(4):326-330
DOI
:10.4103/1817-1745.174434
PMID
:26962336
Background:
Neonatal seizures are a common manifestation of brain dysfunction. Neonatal magnetic resonance imaging (MRI) has rapidly become the study of choice for the evaluation of central nervous systems disorders in newborns. According to a study conducted in Wilhelmina Children's Hospital, University Medical Center Utrecht, diffusion Restriction (DR) changes in the MRI is a good indicator of cell dysfunction (reversible or irreversible) within one week of insult.
Objectives:
The main aim of this study was to find the association of DR changes in MRI of brain for neonatal seizures with long term neurodevelopment outcome.
Methods:
This is a retrospective observational study conducted in Sri Ramachandra University. Retrospective data was collected for the time period of January 2010 to December 2011 from medical records department (MRD) for patient data, neonatal intensive care unit and reports from PACS for MRI images and the Karthikeyan child development unit for their developmental follow up reports.
Results:
Comparison of composite score for various domains with DR changes was done with a t-test and comparison of babies with developmental delay and DR changes with Chi-square test. MRI DR changes with developmental outcome in different domains namely cognition, language-receptive/expressive, fine and gross motor was studied. There is no statistical significance among those who have DR changes and with those who do not have DR changes.
Conclusion:
Though diffusion restriction changes in MRI may not predict adverse long term neuro developmental outcome, they can be of use with regards to individual etiological profile as in stroke. Larger group study and long term follow up is required to substantiate these findings.
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© 2005 - Journal of Pediatric Neurosciences | Published by Wolters Kluwer -
Medknow
Online since 10
th
November, 2005