Determinants of Central Nervous System Involvement in Herpes Zoster

BACKGROUND: Herpes zoster is a viral disease characterized by a painful skin rash with blisters in a limited area on one side of the body, often in a stripe. Central nervous system(CNS) involvements are uncommon complications of herpes zoster. The exact mechanism and risk factors are still unknown. METHODS: We retrospectively reviewed the clinical data of patients who was admitted at our hospital due to herpes zoster from 2003 to 2013. The patients under age 15, herpes zoster infection without skin lesions, and cases not confirmed by a dermatologist were excluded. CNS involvements are defined as meningitis, encephalitis, single or multiple cranial neuropathies and all cases were evaluated with brain magnetic resonance imaging, spinal tapping, serological tests and confirmed by a neurologist. We compared the herpes zoster patients with CNS involvement to those without CNS involvement. Age, sex, body mass index, associated chronic medical illnesses, site and extent of skin lesion and development of post herpetic neuralgia were compared between two groups. RESULTS: Total 1,131 subjects (male 460, female 671) were recruited. A group with CNS involvement was 91(8.04%). Sex, body mass index, associated chronic medical illnesses, extent of skin lesion were not different between two groups. A group with CNS involvement showed younger age(p<0.01), more facial and cervical skin lesions(p<0.01), lesser development of post herpetic neuralgia(p=0.048). CONCLUSIONS: CNS involvement is not a rare complication of herpes zoster and more frequent in patients with younger age and faciocervical zoster.

Leukemic Infiltration of the Optic Nerve Head as the Initial Manifestation of Leukemic Relapse

PURPOSE: To present a case of leukemic infiltration of the optic nerve head as the initial manifestation of leukemic relapse. CASE SUMMARY: A 65-year-old woman was diagnosed with acute myeloid leukemia. Complete remission was achieved after 4 complete courses of chemotherapy. She complained of a sudden decrease in visual acuity in her left eye. Fundus examination showed severe optic disc edema with peripapillary hemorrhage and serous retinal detachment. Visual acuity and fundus continued to aggravate and high-dose intravenous steroid therapy was instituted. Visual acuity and fundus deteriorated more after treatment. Brain magnetic resonance imaging and CSF study were normal but intrathecal chemotherapy and focal irradiation were performed on account of the suspected CNS involvement of leukemia. Morphologic improvement in the retinal structure was achieved, however, optic atrophy remained and her vision did not recover. CONCLUSIONS: The present case shows the involvement of the optic nerve head as the initial isolated manifestation for the relapse in a patient with complete remission. CNS involvement is rare in acute myeloid leukemia and in particular, the optic nerve is rarely reported as the initial isolated presentation for the relapse. Moreover, the disease progression relatively aggravated after treatment. In the atypical aspects of leukemic relapse, the present case was noticeable.

Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement / 소아과

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and might be an effective option in patients with EBV-HLH with progressive CNS disease. Moreover, the addition of rituximab to RIC appears to be safe and effective in suppressing EBV in the patients with EBV-HLH.

Primary Cerebellar Hodgkin's Lymphoma

Authors report a very rare case of primary Hodgkin's lymphoma limited to the cerebellum. A 64-year-old female patient presented with headache, nausea, and vomiting. Magnetic resonance imaging of the brain revealed a nodular enhancing mass in the left cerebellar hemisphere. Tumor was removed totally with retromastoid suboccipital approach. Diagnosis was Hodgkin's lymphoma of mixed cellularity type, consisting of a dense mixed inflammatory infiltrate containing scattered large atypical mononuclear Hodgkin cells and multinucleated Reed-Sternberg cells. Systemic work-up for the Hodgkin's lymphoma was followed with negative result. Postoperative radiation therapy was given and the patient is disease-free at 16 months after the operation.

Imaging findings of CNS lymphoma in Siriraj Hospital.

Objective: To describe the CT and MR features of CNS lymphoma (both PCNSL and SLCNS groups) and to determine whether there is a difference. Methods: A retrospective study of CNS lymphoma in 100 patients at the department of Medicine, Siriraj Hospital, Mahidol University, during January 1997 – September 2002, for neuroimaging analysis. We retrospectively analyzed all available CT and MR findings of these patients by a neuroradiologist who was blinded to the patients’ clinical histories. The imaging studies were evaluated for density in CT, intensity in MR, pattern of enhancement, leptomeningeal enhancement, number of lesions, location and degree of edema. For MRI of spines, we additionally classified 4 menifestations: osseous lymphoma, spinal epidural lymphoma, lymphomatous meningitis and intramedullary lymphoma. Results: Only 33 CTs of the brain, 12 MRs of the brain and 11 MRs of spines were available for retrospective evaluation in this study. For CTs of the brain, most of the lesions showed hyperdensity on non-contrast CT (45% in PCNSL and 59% in SLCNS) with homogenous enhancement on contrast CT (78% in PCNSL and 75% in SLCNS). Eleven percent of ring enhancement lesions in PCNSL and eight percent in SLCNS were found in all patients with HIV infection. For MRs of the brain, most lesions of PCNSL and SLCNS showed hypo-isointensity in both T1W and T2W and homogenous enhancement. Locations of PCNSL were found in cerebral white matter and corpus callosum (55.5%) and basal ganglia (33.3%). SLCNS were found in cerebral white matter (50%), cavernous sinus (37.5%), basal ganglia (6.25%) and brainstems (6.25%). Conclusion: CNS lymphoma has been increased in Thailand. Imaging findings in PCNSL and SLCNS groups were not difference in this study. The pattern of imaging findings of CNS lymphoma in Thailand was similar to CNS lymphoma in the world.

A Case of Subcutaneous Panniculitic T-cell Lymphoma Involved in Skin and Central Nervous System / 대한피부과학회지

Subcutaneous panniculitic T-cell lymphoma(SPTCL) is categorized as a rare subtype of peripheral T-cell lymphoma. It is characterized by primary involvement of the subcutaneous fat in a manner mimicking panniculitis with/without hemophagocytic syndrome. They share a generally aggressive course and are highly associated with Epstein-Barr virus infection. EBV infection plays an important role in the tumorigenesis and may be related to cutaneous lymphoma with hemophagocytic manifestations. We have seen a patient, a 67-year-old woman with tender erythematous nodules on both upper extremities and abdomen. With time, the skin lesion showed ulcerative change on her right thigh. She has also suffered from fever, weight loss, arthralgia, and general weakness without hepatosplenomegaly or lymphadenopathy for 4 months. During the admission, she complained of nausea, vomiting and dysarthria. On the MRI examination, we found a multi-focal solid lesion on her brain. The histopathological findings of the biopsy from her abdominal skin lesion showed a septal and lobular, histiocytic panniculitis with bean bag cells and atypical lymphoid cells identified as NK like T-cells and also dense diffuse infiltrates localized in the lower dermis and subcutaneous tissue, with minimal epidermal and upper dermal infiltrates without destructive change of blood vessels. The infiltrating atypical lymphoid cells expressed the phenotype of LCA, CD45RO, CD3, CD8, CD56 and also positive for EBV by in situ hybridization. Our case showed a clonal TCR gamma gene rearrangement by polymerase chain reaction. She was subjected to a course of treatment(cyclophosphamide, vincristine, prednisolone and radiation therapy) under the diagnosis of SPTCL. but died of sepsis due to urinary tract infections after 2 months.

A Case of Primary Bilateral Adrenal Lymphoma (PAL) with Central Nervous System (CNS) Involvement

Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in Korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.

A Case of Primary Bilateral Adrenal Lymphoma (PAL) with Central Nervous System (CNS) Involvement

Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in Korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.

The Clinical Characteristics and Gene Analysis of Korean Gaucher Disease with Central Nervous System Involvement

PURPOSE: We performed the study to determine the clinical characteristics and natural history of Gaucher disease with CNS involvement in Korea as well as to determine whether gene analysis would be helpful in the identification of neuronopathic Gaucher disease. METHODS: We described the initial symptom, clinical manifestations, age of onset, clinical neurophysiologic findings and brain MRI findings from the medical records of 13 patients with neuronopathic Gaucher disease identifed from the Korean Gaucher Disease Patient Registry. Mutation analysis was performed in 7 cases of neurono pathic Gaucher disease patients by PCR or PCR-single strand conformation polymorphism (SSCP). RESULTS: Among 46 cases of Korean Gaucher disease, type ll was found in 6 cases (13%) and type lll was found in 7 cases (15%). Type II Gaucher disease showed typical clinical features, and those patients expired in less than 2 years of age. All the type IIIa patients presented epileptic seizure as an initial symptom and most of the patients survived. All the type IIIa patients revealed epileptiform activity and photoparoxysmal response. Mutation analysis in neuronopathic Gaucher disease patients revealed L444P/L444P (2 cases), F213I/F213I (1 case), F213I/? (1 case) and N188S/? (1 case). However, mutation analysis in type I Gaucher disease revealed L444P/? (4 cases), F213I/? (2 cases) and L444P/F213I (1 case). CONCLUSION: The clinical characteristics and natural history of Korean Gaucher disease is similar to those of Jewish Gaucher disease. Brain MRI, clinical neurophysiologic study and gene analysis are not helpful in the identification of neuronopathic Gaucher disease. Regular neurologic examinations are the only method to determine CNS involvement in Gaucher disease.

A Case of Ectopic CD7(+) Congenital Monocytic Leukemia

Congenital acute leukemia is a rare disorder with approximately 200 cases reported. It is defined as a childhood leukemia occurring at birth or before 1 month of age at a rate of 1%. Acute leukemias are generally classified according to morphology, cytochemistry and cell surface marker expression. Most leukemias conform to an ordered lineage-specific pattern of gene expression, but a small subset of leukemias appears not to follow lineage restriction. Several reports revealed a subgroup of acute myelogenous leukemia(AML) that expresses CD7, a cell surface marker expressed early during T lineage differentiation, especially in less differentiated AML subtypes. We report a rare case of CD7(+) congenital monocytic leukemia(M5a) with detailed immunophenotypic and cytochemical characterization in an 8 week-old female. She had central nervous system (CNS) involvement at diagnosis. Chromosomal analysis revealed a mosaicism with 46,XX,-6,de1(7) (q21),t(19;21)(q13.3;q22)/46,XX, that has not been reported.

Malignant Fibrous Histiocytoma of the Pericranium with Intracranial Extension: A Case Report

A case of malignant fibrous histiocytoma(MFH) arising from the pericranium which extend to the calvaria and the dura mater in a 32-year-old man is reported. This tumor presented as scalp induration and headache without any other neurologic abnormalities. Radical excision presented a surgical challenge because of the extensiveness of the lesion. In this case radical excision of pericranial and calvarial lesions were possible, however it is impossible to remove the lesion in the dura mater radically because of its extensiveness. Thus the radiation therapy was necessary after operation. Histologically, pericranial, calvarial and dura mater tumor tissues were same as that of the typical malignant fibrous histiocytoma of the soft tissue. The authors review the literatures of the MFH involving central nerve systems and discuss the management and the radiologic findings of this rare tumor.