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Multiple cranial neuritis associated with large granular lymphocytosis
1Department of Neurology and Gerontology, Kyoto Prefectural University of Medicine, Japan.
Insights
A rare case of large granular lymphocytosis (LGL) with cranial nerve involvement is presented. This unique CD4+ LGL condition highlights diagnostic challenges in neurology and hematology.
Area of Science:
- Hematology
- Neuroscience
- Immunology
Background:
- Large granular lymphocytosis (LGL) is a rare condition characterized by an overproduction of large granular lymphocytes.
- Cranial neuritis, affecting multiple cranial nerves, can manifest with symptoms like facial weakness, diplopia, and dysarthria.
Observation:
- A patient presented with symptoms of multiple cranial neuritis, including facial weakness, diplopia, and dysarthria.
- Elevated levels of large granular lymphocytes (LGLs) were detected in the patient's blood, cerebrospinal fluid (CSF), and bone marrow.
Findings:
- The LGLs exhibited a unique CD4+ phenotype (CD2+ CD3+ CD4+ CD8- CD16- CD56- CD57-).
- A notable abundance of LGLs was observed in the CSF, alongside their presence in blood and bone marrow.
Implications:
- This case underscores the importance of considering LGL lymphocytosis in the differential diagnosis of unexplained cranial neuritis.
- The distinct CD4+ phenotype and CSF LGL prominence offer insights into the pathophysiology of LGL-associated neurological disorders.
- Further research into LGL subtypes and their neurological manifestations is warranted.
Abstract:
A patient with a unique form of large granular lymphocytosis and multiple cranial neuritis is reported. The patient presented with facial weakness, diplopia and dysarthria. An increase in large granular lymphocytes (LGLs) was seen in blood (1.8 x 10(9)/l), CSF (237/microliters) and bone marrow (20% in a normocellular bone marrow). The phenotype of the LGLs in CSF, blood and bone marrow was CD2+ CD3+ CD4+ CD8- CD16- CD56- and CD57-. The unique features of this case include the CD4+ phenotype, the relative abundance of CSF LGL and the clinical presentation.