MRI Patterns in Pediatric CNS Hemophagocytic Lymphohistiocytosis

Malik, Prateek; Antonini, L.; Mannam, Pavithra; Aboobacker, Fouzia N.; Merve, Ashirwad; Gilmour, Kimberly C.; Rao, Kanchan; Kumar, ST; Mani, Sunithi; Eleftheriou, Despina; Rao, Anupama; Hemingway, Cheryl; Sudhakar, Sniya; Bartram, Jack; Mankad, Kshitij

doi:10.3174/ajnr.a7292

Cited by 14 publications

(12 citation statements)

References 31 publications

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“…Imaging findings regarding HLH in the central nervous system range from normal findings, to nonspecific findings such as isolated cortical atrophy, to extensive parenchymal disease with multifocal white matter lesions with or without leptomeningeal and/or perivascular enhancement, with frequent cerebellar involvement (7,8,10,11). Tumefactive lesions, optic neuritis and spinal cord lesions have also been described (12).…”

Section: Discussionmentioning

confidence: 99%

“…Tumefactive lesions, optic neuritis and spinal cord lesions have also been described (12). Malik et al grouped imaging patterns into two main groups: 1) parenchymal disease, and 2) normal or nonspecific findings, and subdivided the first group into: 1.1) multifocal cerebral/cerebellar lesions, 1.2) brainstem predominant pattern (CLIPPERS-like pattern), and 1.3) diffuse cerebellar involvement/cerebellitis (11). Our case mainly showed features of group 1.3 at presentation, with some features of 1.2 (a CLIPPERS-like enhancement pattern, although with relative sparing of the pons), and 1.1 (multifocal white matter lesions).…”

Section: Discussionmentioning

confidence: 99%

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Child Neurology: Familial Hemophagocytic Lymphohistiocytosis Underlying Isolated CNS Inflammation

et al. 2022

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Encephalitis and encephalopathy in children represent a diagnostic challenge. We describe a patient with relapsing encephalitis in whom the differential diagnosis included acute disseminated encephalomyelitis (ADEM), human herpesvirus 6 (HHV-6) encephalitis, and hemophagocytic lymphohistiocytosis (HLH). Because of its rarity, HLH is often overlooked as a differential diagnosis in encephalitis, especially in the isolated central nervous system (CNS) forms. As this case illustrates, inborn errors of immunity (IEIs) can underlie isolated encephalitis and should be included in the differential diagnosis of these presentations.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Child Neurology: Familial Hemophagocytic Lymphohistiocytosis Underlying Isolated CNS Inflammation

et al. 2022

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“…Pattern I (significant parenchymal disease) subdivided into multifocal white matter, brainstem-predominant, and cerebellitis group. Pattern II shows non-specific imaging findings [ 89 ]. Three histopathological stages of CNS-HLH are described with perivascular enhancement in a few stages, similar to small vessel CNS vasculitis [ 88 ].…”

Section: Secondary To Systemic Inflammatory Diseasementioning

confidence: 99%

“…Arterial narrowing, subarachnoid hemorrhage, tiny scattered infarcts, and PRES-like appearance are the most frequent imaging findings [ 7 , 15 , 54 ]. Reversibility of angiographic findings within 12 weeks and the absence or subtle enhancement of the vessel wall help to differentiate it from cPACNS [ 18 , 46 , 89 ]. The multifocal nature is against the diagnosis of focal cerebral arteriopathy.…”

Section: Mimicsmentioning

confidence: 99%

Childhood Cerebral Vasculitis

et al. 2022

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Cerebral vasculitis is increasingly recognized as a common cause of pediatric arterial stroke. It can present with focal neurological deficits, psychiatric manifestations, seizures, and encephalopathy. The etiopathogenesis of childhood cerebral vasculitis (CCV) is multifactorial, making an inclusive classification challenging. In this review, we describe the common and uncommon CCV with a comprehensive discussion of etiopathogenesis, the role of various imaging modalities, and advanced techniques in diagnosing CCV. We also highlight the implications of relevant clinical, laboratory, and genetic findings to reach the final diagnosis. Based on the clinicoradiological findings, a stepwise diagnostic approach is proposed to facilitate CCV diagnosis and rule out potential mimics. Identification of key clinical manifestations, pertinent blood and cerebrospinal fluid results, and evaluation of central nervous system vessels for common and disease-specific findings will be emphasized. We discuss the role of magnetic resonance imaging, MR angiography, and vessel wall imaging as the imaging investigation of choice, and reservation of catheter angiography as a problem-solving tool. We emphasize the utility of brain and leptomeningeal biopsy for diagnosis and exclusion of imitators and masqueraders.

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“…Hemaphagocytic lymphohistiocytosis (HLH) is a heterogeneous disorder characterized by toxic uncontrolled immune activation. 31 This immune activation is often driven by genetic mutations occurring along the perforin-dependent granule exocytosis pathway and is referred to as primary HLH. 32 In secondary HLH, there is no underlying genetic mutation but systemic inflammation or immunotherapy may cause macrophage activation.…”

Section: Cns Involvement In Hematologic Disordersmentioning

confidence: 99%

Imaging of Central Nervous System Involvement in Pediatric Hematologic Disorders

Kalın¹,

Koral²

2022

Turk Arch Pediatrics

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Hematologic disorders and malignancies are commonly encountered in children. The central nervous system is affected by many benign and malignant hematological diseases. Neurologic findings may occur due to central involvement of hematological disease or neurotoxic effects of treatment during the disease. At all these stages, radiological imaging and evaluation have a critical role. In this review article, we aim to describe the imaging findings of central nervous system involvement in pediatric hematologic diseases. This review was prepared based on the latest literature available in the PubMed database in the English language from inception to March 2022. The radiological images of the patients in our archive were obtained from the PACS (Picture Archiving and Communication Systems) to set an example for the diseases described in the text.

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MRI Patterns in Pediatric CNS Hemophagocytic Lymphohistiocytosis

Cited by 14 publications

References 31 publications

Child Neurology: Familial Hemophagocytic Lymphohistiocytosis Underlying Isolated CNS Inflammation

Child Neurology: Familial Hemophagocytic Lymphohistiocytosis Underlying Isolated CNS Inflammation

Childhood Cerebral Vasculitis

Imaging of Central Nervous System Involvement in Pediatric Hematologic Disorders

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