Hemiparesis Revealing a Unique Neurological Hemophagocytic Lymphohistiocytosis in a Patient With Griscelli Syndrome Type 2

Griscelli syndrome (GS) is a rare genetic disorder that encompasses three different subtypes (GS type 1 (GS1), GS type 2 (GS2), and GS type 3 (GS3)), in which isolated neurological manifestations without immune system implications are typically seen in GS1, while neurological involvements in GS2 sho...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2022-09, Vol.14 (9)
Main Authors: Moueqqit, Othman, Ayad, Ghanam, Benhachem, Madiha, Lahmar, Abdelilah, Ramdani, Hiba, Nadir, Miry, Bensalah, Mohammed, Bennani, Amal, Kamaoui, Imane, Seddik, Rachid, Benajiba, Noufissa
Format: Article
Language:English
Subjects:
Age
Albinism
Case reports
Disease
Evolution
Fever
Genes
Genetics
Hematology
Hemoglobin
Immune system
Laboratories
Lymphatic diseases
Mutation
Paresis
Pediatrics
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Description
Summary:Griscelli syndrome (GS) is a rare genetic disorder that encompasses three different subtypes (GS type 1 (GS1), GS type 2 (GS2), and GS type 3 (GS3)), in which isolated neurological manifestations without immune system implications are typically seen in GS1, while neurological involvements in GS2 should be attributed to the macrophage and lymphocyte invasion of the central nervous system (CNS), under associated hemophagocytic lymphohistiocytosis (HLH). The presence of the clinical, biological, and hematologic features of HLH help explain the neurological defects that GS2 patients unusually present. In our case report, however, we attempt to highlight an uncommon presentation of GS2 involving a hemiparesis, along which we did not have any clinical or biological features of HLH. We also collect and evaluate similar published cases that feature this problem of explaining the neurological manifestations among GS2 patients.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.29159