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  1. Article ; Online: Disseminated Intravascular Coagulation-Related Microangiopathy.

    Sharma, Rohan / Cobb, Sarah / Ramakrishnaiah, Raghu

    Neurocritical care

    2023  Volume 39, Issue 2, Page(s) 533–538

    MeSH term(s) Humans ; Disseminated Intravascular Coagulation/diagnosis ; Disseminated Intravascular Coagulation/etiology
    Language English
    Publishing date 2023-05-06
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 2381896-7
    ISSN 1556-0961 ; 1541-6933
    ISSN (online) 1556-0961
    ISSN 1541-6933
    DOI 10.1007/s12028-023-01733-1
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    Zs.A 6538: Show issues Location:
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  2. Article ; Online: Clinical and radiological evaluation of caudal regression syndrome.

    Krishnan, Venkatram / Jaganathan, Sriram / Jayappa, Sateesh / Glasier, Charles / Choudhary, Arabinda / Ramakrishnaiah, Raghu / Murphy, Janice

    Pediatric radiology

    2024  

    Abstract: Caudal regression syndrome is a form of segmental spinal dysgenesis involving the caudal spinal column, ranging from segmental coccygeal agenesis to extensive thoracolumbar agenesis with varying degrees of spinal cord dysgenesis. A majority of caudal ... ...

    Abstract Caudal regression syndrome is a form of segmental spinal dysgenesis involving the caudal spinal column, ranging from segmental coccygeal agenesis to extensive thoracolumbar agenesis with varying degrees of spinal cord dysgenesis. A majority of caudal regression cases are sporadic but maternal pre-gestational diabetes mellitus is an important risk factor. Imaging is an integral part of management of caudal regression syndrome. Antenatal diagnosis on obstetric ultrasound and evaluation with fetal MRI is ideal. Early postnatal diagnosis and/or detailed evaluation with MRI is essential for early management to improve outcomes. Pang classification categorizes caudal regression syndrome into two categories based on the position of the conus while Renshaw classification is based on the degree of vertebral column agenesis. Caudal regression syndrome may be associated with several additional anomalies, both spinal and extraspinal. A number of genitourinary and gastrointestinal anomalies have been described in association with caudal regression syndrome. The field of view of MRI of the lumbosacral spine in caudal regression syndrome needs to be extended to visualize the retroperitoneal structures without the use of a saturation band. Syndromic associations may be suspected, and additional imaging performed, based on findings of extended field of view MRI of the spine. Associated sacral masses and filar abnormalities need to be identified and may also require surgical treatment. The multisystem nature of this disease necessitates a multimodality approach to the evaluation and management of caudal regression syndrome with close cooperation between pediatric neuroradiologists and body radiologists as well as multiple clinical teams. Appropriate early management with surgical correction as necessary can significantly improve prognosis and survival in caudal regression syndrome.
    Language English
    Publishing date 2024-05-16
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 124459-0
    ISSN 1432-1998 ; 0301-0449
    ISSN (online) 1432-1998
    ISSN 0301-0449
    DOI 10.1007/s00247-024-05945-1
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    Zs.A 927: Show issues Location:
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  3. Article ; Online: Complex Heterozygous Polymerase Gamma Mutation and Cerebral Folate Deficiency in a Child with Refractory Partial Status.

    Samanta, Debopam / Ramakrishnaiah, Raghu / Frye, Richard E

    Neurology India

    2022  Volume 70, Issue 5, Page(s) 2265–2267

    MeSH term(s) Child ; Humans ; Folate Receptor 1 ; Neuroaxonal Dystrophies/genetics ; Mutation/genetics ; Folic Acid/therapeutic use
    Chemical Substances Folate Receptor 1 ; Folic Acid (935E97BOY8)
    Language English
    Publishing date 2022-11-09
    Publishing country India
    Document type Letter
    ZDB-ID 415522-1
    ISSN 1998-4022 ; 0028-3886
    ISSN (online) 1998-4022
    ISSN 0028-3886
    DOI 10.4103/0028-3886.359254
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    Zs.A 698: Show issues Location:
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  4. Article ; Online: Intravenous Immunoglobulin in the Treatment of Alternating Hemiplegia of Childhood.

    Samanta, Debopam / Ramakrishnaiah, Raghu

    Clinical neuropharmacology

    2020  Volume 44, Issue 1, Page(s) 23–26

    Abstract: Abstract: Alternating hemiplegia of childhood (AHC) is a neurological disorder with early-onset alternating hemiplegia and other paroxysmal events such as epilepsy and dystonia due to de novo pathogenic mutations in the ATP1A3. Physicians and scientists ...

    Abstract Abstract: Alternating hemiplegia of childhood (AHC) is a neurological disorder with early-onset alternating hemiplegia and other paroxysmal events such as epilepsy and dystonia due to de novo pathogenic mutations in the ATP1A3. Physicians and scientists investigated several agents in the treatment without strong evidence of definitive long-term benefit. Knowledge regarding utility of anti-inflammatory agents in the treatment is scarce except the anecdotal report of corticosteroid use. We described 2 patients with AHC who were exposed to intravenous immunoglobulin (IVIG) treatment because of an alternative diagnosis. An 8-year-old girl received 4 years of periodic IVIG infusion and was free of paroxysmal events during the first 16 months of therapy. A 2-year-old boy received IVIG infusion for 10 months and remained seizure-free for 2 years since the beginning of the treatment, but without a definite change in hemiplegic episodes. Our report is the first description of IVIG use in patients with AHC. Although these patients did not achieve complete remission, partial responsiveness was noted. Although the definite role of IVIG in the treatment of AHC cannot be extrapolated from our study, further prospective controlled studies should be done because of the recent discovery of the connection between an anti-inflammatory hormone (Oubain) and Na+/K+ ATPase.
    MeSH term(s) Child ; Child, Preschool ; Female ; Hemiplegia/diagnostic imaging ; Hemiplegia/drug therapy ; Hemiplegia/genetics ; Humans ; Immunoglobulins, Intravenous/administration & dosage ; Immunologic Factors/administration & dosage ; Infant ; Male ; Treatment Outcome
    Chemical Substances Immunoglobulins, Intravenous ; Immunologic Factors
    Language English
    Publishing date 2020-11-11
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 199293-4
    ISSN 1537-162X ; 0362-5664
    ISSN (online) 1537-162X
    ISSN 0362-5664
    DOI 10.1097/WNF.0000000000000420
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    Se 437: Show issues Location:
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  5. Article: Early-Onset Parkinsonism and Halo Sign: Beta-propeller Proteinassociated Neurodegeneration.

    Samanta, Debopam / Ramakrishnaiah, Raghu

    Journal of pediatric neurosciences

    2020  Volume 15, Issue 3, Page(s) 325–327

    Abstract: A 13-year-old girl with infantile-onset self-resolving epilepsy and developmental delay had an unremarkable workup, including normal brain magnetic resonance imaging (MRI) and chromosomal microarray. During adolescence, she presented with features of ... ...

    Abstract A 13-year-old girl with infantile-onset self-resolving epilepsy and developmental delay had an unremarkable workup, including normal brain magnetic resonance imaging (MRI) and chromosomal microarray. During adolescence, she presented with features of early-onset parkinsonism: gait dyspraxia, freezing during walking, cogwheel rigidity in both upper extremities, and left arm dystonia. Repeat brain MRI showed iron deposition on the substantia nigra (SN) and basal ganglia, with hyperintense halo sign around a central linear hypointensity within the SN on the T1 imaging sequence. Whole-exome sequencing with trio revealed
    Language English
    Publishing date 2020-11-06
    Publishing country India
    Document type Case Reports
    ISSN 1817-1745
    ISSN 1817-1745
    DOI 10.4103/jpn.JPN_62_20
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  6. Article ; Online: Arachnoid cyst in the pediatric patient: What the radiologist needs to know.

    Krishnan, Venkatram / Jaganathan, Sriram / Baker, Andrew / Jayappa, Sateesh / Murphy, Janice / Glasier, Charles / Choudhary, Arabinda / Albert, Gregory / Ramakrishnaiah, Raghu

    The neuroradiology journal

    2024  , Page(s) 19714009241248746

    Abstract: Arachnoid cysts are the most common incidentally discovered intracranial lesions on imaging and the most common cystic intracranial lesions. They may be developmental or secondary. A relative lack of recent literature and any comprehensive radiological ... ...

    Abstract Arachnoid cysts are the most common incidentally discovered intracranial lesions on imaging and the most common cystic intracranial lesions. They may be developmental or secondary. A relative lack of recent literature and any comprehensive radiological review on arachnoid cysts has led to a general lack of awareness among radiologists of symptomatic or complicated arachnoid cysts. This is particularly concerning in pediatric patients. While arachnoid cysts are asymptomatic in most cases, they can cause clinical symptoms in a minority of cases, especially when they occur in unusual sites. These include intraventricular locations where they may cause hydrocephalus, the basal cisterns where they may compress cranial nerves, the cerebellopontine angle where they have to be differentiated from a number of cystic lesions, the cavum septum pellucidum or cavum velum interpositum, the choroid fissure where they can entrap the temporal horn and compress the hippocampus, the posterior fossa where they need to be differentiated from other posterior fossa cystic lesions, and within the spinal canal where there is a concern for cord or nerve root compression. Larger cysts are more prone to complications such as mass effect, hemorrhage, and rupture. Hemorrhage and rupture often present with acute symptoms. Ruptured cysts lose their characteristic imaging appearance and can mimic several ominous pathologies. It therefore becomes vital to accurately diagnose these cases as complications of pre-existing arachnoid cysts for appropriate management. A detailed review of all diagnostic imaging aspects of arachnoid cysts will help fill in the existing information void on this important entity.
    Language English
    Publishing date 2024-04-22
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2257770-1
    ISSN 2385-1996 ; 1971-4009 ; 1120-9976
    ISSN (online) 2385-1996
    ISSN 1971-4009 ; 1120-9976
    DOI 10.1177/19714009241248746
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    Zs.A 2823: Show issues Location:
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  7. Article ; Online: Clinical and Radiologic Findings in Patients With Morning Glory Disc Anomaly and Associated Optic Pathway Enlargement.

    Elhusseiny, Abdelrahman M / Kwok, Alexander / Kanfi, Alisa / Ramakrishnaiah, Raghu H / Phillips, Paul H

    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society

    2023  

    Language English
    Publishing date 2023-07-07
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1189901-3
    ISSN 1536-5166 ; 1070-8022
    ISSN (online) 1536-5166
    ISSN 1070-8022
    DOI 10.1097/WNO.0000000000001930
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    Zs.A 1670: Show issues Location:
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  8. Article ; Online: Recurrent Oral Ulcers in a Child With Seizures.

    Samanta, Debopam / Ramakrishnaiah, Raghu

    Pediatric neurology

    2019  Volume 103, Page(s) 89–90

    MeSH term(s) Behcet Syndrome/complications ; Behcet Syndrome/diagnosis ; Behcet Syndrome/genetics ; Child ; Female ; HLA-B51 Antigen/genetics ; Humans ; Magnetic Resonance Angiography ; Magnetic Resonance Imaging ; Oral Ulcer/etiology ; Recurrence ; Seizures/etiology
    Chemical Substances HLA-B51 Antigen
    Language English
    Publishing date 2019-04-25
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 639164-3
    ISSN 1873-5150 ; 0887-8994
    ISSN (online) 1873-5150
    ISSN 0887-8994
    DOI 10.1016/j.pediatrneurol.2019.04.008
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    Zs.A 2260: Show issues Location:
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  9. Article ; Online: Neuroimaging Findings in a Child With SensorineuralHearing Loss.

    Samanta, Debopam / Ramakrishnaiah, Raghu

    Pediatric neurology

    2018  Volume 88, Page(s) 75–76

    MeSH term(s) Agenesis of Corpus Callosum/complications ; Agenesis of Corpus Callosum/diagnostic imaging ; Cerebellum/diagnostic imaging ; Hearing Loss, Sensorineural/diagnostic imaging ; Hearing Loss, Sensorineural/etiology ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Polymicrogyria/complications ; Polymicrogyria/diagnostic imaging
    Language English
    Publishing date 2018-08-16
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 639164-3
    ISSN 1873-5150 ; 0887-8994
    ISSN (online) 1873-5150
    ISSN 0887-8994
    DOI 10.1016/j.pediatrneurol.2018.08.012
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    Zs.A 2260: Show issues Location:
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  10. Article ; Online: Diagnosis of Charcot-Marie-Tooth Disease in a Patient With Decreased Vision From Optic Atrophy and No Other Neurological Symptoms.

    Chacko, Joseph A / Phillips, Paul H / Ramakrishnaiah, Raghu H / Schaefer, G Bradley / Uwaydat, Sami H

    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society

    2022  Volume 43, Issue 4, Page(s) e146–e148

    MeSH term(s) Humans ; Charcot-Marie-Tooth Disease/complications ; Charcot-Marie-Tooth Disease/diagnosis ; Optic Atrophy/diagnosis ; Optic Atrophy/etiology
    Language English
    Publishing date 2022-03-24
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1189901-3
    ISSN 1536-5166 ; 1070-8022
    ISSN (online) 1536-5166
    ISSN 1070-8022
    DOI 10.1097/WNO.0000000000001520
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