Moueqqit Othman, Ayad Ghanam, Benhachem Madiha, Lahmar Abdelilah, Ramdani Hiba, Nadir Miry, Bensalah Mohammed, Bennani Amal, Kamaoui Imane, Seddik Rachid, Benajiba Noufissa
Department of General Medicine, Faculty of Medicine and Pharmacy of Oujda, Mohammed First University of Oujda, Oujda, MAR.
Pediatric Medicine, Centre Hospitalier Universitaire (CHU) Mohammed VI Oujda, Oujda, MAR.
Cureus. 2022 Sep 14;14(9):e29159. doi: 10.7759/cureus.29159. eCollection 2022 Sep.
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.
格里塞利综合征（GS）是一种罕见的遗传性疾病，包括三种不同的亚型（1型格里塞利综合征（GS1）、2型格里塞利综合征（GS2）和3型格里塞利综合征（GS3））。在GS1中通常可见无免疫系统影响的孤立性神经表现，而GS2中的神经受累应归因于在相关噬血细胞性淋巴组织细胞增生症（HLH）情况下巨噬细胞和淋巴细胞侵入中枢神经系统（CNS）。HLH的临床、生物学和血液学特征有助于解释GS2患者异常出现的神经缺陷。然而，在我们的病例报告中，我们试图强调GS2的一种不常见表现，即偏瘫，在此过程中我们没有HLH的任何临床或生物学特征。我们还收集并评估了类似的已发表病例，这些病例都存在解释GS2患者神经表现这一问题。
