Introduction:
Langerhan’s cell histiocytosis is a malignancy caused by uncontrolled proliferation of the Langheran’s cell line with associated granulomatous deposition. The disease exists in a wide spectrum of clinical presentations ranging from localized skin manifestation to diffuse disease with neurological involvement. Typically, diabetes insipidus occurs when the hypothalamic-pituitary axis is affected by histiocytic infiltration. Langheran’s cell histiocytosis can be confirmed by performing immunohistochemical testing for CD1a. Although Langheran’s cell histiocytosis can affect the central nervous system directly, there have been reported cases of delayed neurodegeneration that occur years after primary diagnosis and treatment of LCH.
History and Presentation:
Our patient is a 11 year old girl with a PMHx of Langerhan’s cell histiocytosis presenting to an outside hospital with a 2 week history of worsening headache, ataxia, and double vision. A scan(MRI??) was performed which showed concerning signs of …show more content…
The following day, she became nonverbal on examination. Two days after admission, she underwent a sub-occipital craniotomy for stealth biopsy and was started on cytarabine and IVIG. Follow up imaging on the next day showed worsening cerebellar edema and increased compression of the brainstem. Due to her progressive deterioration, a midline sub-occipital craniectomy with C1 laminectomy and partial thickness dural scoring was performed for posterior fossa decompression. She experienced an upgaze palsy after this procedure. Her neurological deficits were stable and persisted for 5 days at which time she gradually recovered her extraoccular movements and speech. Approximately 12 days after admission, she had returned to near baseline with only mild dysarthria and was transferred to the hematology and oncology service for further
Langerhan’s cell histiocytosis is a malignancy caused by uncontrolled proliferation of the Langheran’s cell line with associated granulomatous deposition. The disease exists in a wide spectrum of clinical presentations ranging from localized skin manifestation to diffuse disease with neurological involvement. Typically, diabetes insipidus occurs when the hypothalamic-pituitary axis is affected by histiocytic infiltration. Langheran’s cell histiocytosis can be confirmed by performing immunohistochemical testing for CD1a. Although Langheran’s cell histiocytosis can affect the central nervous system directly, there have been reported cases of delayed neurodegeneration that occur years after primary diagnosis and treatment of LCH.
History and Presentation:
Our patient is a 11 year old girl with a PMHx of Langerhan’s cell histiocytosis presenting to an outside hospital with a 2 week history of worsening headache, ataxia, and double vision. A scan(MRI??) was performed which showed concerning signs of …show more content…
The following day, she became nonverbal on examination. Two days after admission, she underwent a sub-occipital craniotomy for stealth biopsy and was started on cytarabine and IVIG. Follow up imaging on the next day showed worsening cerebellar edema and increased compression of the brainstem. Due to her progressive deterioration, a midline sub-occipital craniectomy with C1 laminectomy and partial thickness dural scoring was performed for posterior fossa decompression. She experienced an upgaze palsy after this procedure. Her neurological deficits were stable and persisted for 5 days at which time she gradually recovered her extraoccular movements and speech. Approximately 12 days after admission, she had returned to near baseline with only mild dysarthria and was transferred to the hematology and oncology service for further