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Langerhans cell histiocytosis in an adult with involvement of the calvarium, cerebral cortex and brainstem: discussion of pathophysiology and rationale for the use of intravenous immune globulin.

Dardis C, Aung T, Shapiro W, Fortune J, Coons S - Case Rep Neurol (2015)

Bottom Line: Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition.These lesions improved after treatment with cladribine.They remained stable while on treatment with intravenous immune globulin.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Barrow Neurological Institute, Phoenix, Ariz., USA.

ABSTRACT
We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin.

No MeSH data available.


Related in: MedlinePlus

Right temporal lobe biopsy. HE staining. Magnification is ×40. Mildly hypercellular and gliotic gray matter with focal reactive changes. No neoplasia is seen.
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Figure 3: Right temporal lobe biopsy. HE staining. Magnification is ×40. Mildly hypercellular and gliotic gray matter with focal reactive changes. No neoplasia is seen.

Mentions: Initially, we were unsure of the cause of his right temporal lesion. Given the rarity of LCH with onset in the central nervous system (CNS) in adults, the possibility of another tumor was raised, and so a second biopsy was performed. In retrospect, it would have been better to avoid performing this, as the diagnosis was already clear and his other lesions were attributable to LCH. Furthermore, the procedure precipitated status epilepticus and led to preventable complications which ultimately contributed to his death. The biopsy is shown in figure 3. This illustrates mild edema, hypercellularity and reactive changes, all of which are consistent with the edge of an inflammatory process. No Langerhans or neoplastic cells are seen.


Langerhans cell histiocytosis in an adult with involvement of the calvarium, cerebral cortex and brainstem: discussion of pathophysiology and rationale for the use of intravenous immune globulin.

Dardis C, Aung T, Shapiro W, Fortune J, Coons S - Case Rep Neurol (2015)

Right temporal lobe biopsy. HE staining. Magnification is ×40. Mildly hypercellular and gliotic gray matter with focal reactive changes. No neoplasia is seen.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4386111&req=5

Figure 3: Right temporal lobe biopsy. HE staining. Magnification is ×40. Mildly hypercellular and gliotic gray matter with focal reactive changes. No neoplasia is seen.
Mentions: Initially, we were unsure of the cause of his right temporal lesion. Given the rarity of LCH with onset in the central nervous system (CNS) in adults, the possibility of another tumor was raised, and so a second biopsy was performed. In retrospect, it would have been better to avoid performing this, as the diagnosis was already clear and his other lesions were attributable to LCH. Furthermore, the procedure precipitated status epilepticus and led to preventable complications which ultimately contributed to his death. The biopsy is shown in figure 3. This illustrates mild edema, hypercellularity and reactive changes, all of which are consistent with the edge of an inflammatory process. No Langerhans or neoplastic cells are seen.

Bottom Line: Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition.These lesions improved after treatment with cladribine.They remained stable while on treatment with intravenous immune globulin.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Barrow Neurological Institute, Phoenix, Ariz., USA.

ABSTRACT
We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin.

No MeSH data available.


Related in: MedlinePlus