Disseminated Hormographiella aspergillata Infection with Lung and Brain Involvement after Allogenic Hematopoietic Stem-Cell Transplantation in a 54-Year-Old Man.

Hormographiella is a rare fungal pathogen in humans; however, case reports have described disseminated infection in immunocompromised hosts. This pathogen has been described to yield poor prognosis in patients who harbor it. Herein, we present a case report of autopsy-proven disseminated Hormographiella aspergillata infection, confirmed by DNA sequencing, in a patient experiencing a relapse of leukemia. This 54-year-old Caucasian man with chronic myelogenous leukemia (CML) that had been diagnosed in 1989, after having received a hematopoietic cell allotransplant from a compatible sibling donor, had B-cell lymphoid-blast phase of CML in April of 2013, with multiple relapses. His most recent relapse was in September of 2016, when bone marrow biopsy showed 90% blasts. The results of bronchoalveolar lavage (BAL) cultures were positive for filamentous fungus infection. The patient developed encephalopathy and worsening respiratory statusand tachycardia with flutter and hypotension, which resulted in his death. At autopsy, bilateral pleural effusions, multiple right pleural nodules, and subarachnoid hemorrhage were noted. Angioinvasive hyphal fungi were found in the right frontal lobe of the brain and the right upper lobe of the lung. Morphologically, the fungi had multiseptate, branching hyphae. The bronchoalveolar lavage specimen grew a fungus for which the colony morphologic characteristics and microscopic features were compatible with a Hormographiella species. H. aspergillata from the bronchoalveolar lavage was further identified by sequencing the D2 hypervariable region of the large-subunit (LSU) ribosomal DNA gene and the full internal transcribed spacer (ITS) regions.

Neonatal Respiratory Failure Caused by Congenital Diffuse Intrinsic Pontine Glioma.

The authors present a case of diffuse intrinsic pontine glioma presenting in a newborn with stridor and respiratory distress that progressed to respiratory failure. Magnetic resonance imaging (MRI) of the brain revealed findings compatible with the diagnosis of diffuse intrinsic pontine glioma. The family pursued palliative care and postmortem examination confirmed WHO grade III astrocytoma.

Recurrent anaplastic medulloblastoma in cerebrospinal fluid after autologous hematopoietic stem cell transplant.

Cerebrospinal fluid (CSF) dissemination can be seen relatively frequently in medulloblastomas and its presence at diagnosis is important for determining both treatment and prognosis. The anaplastic variant is an aggressive variant of medulloblastoma with characteristic histopathologic features and unfavorable prognosis that was included in the latest WHO classification. Herein, we report the CSF cytologic features of a case of recurrent anaplastic medulloblastoma in a 17-year-old male patient who had undergone autologous hematopoietic stem cell transplant as a part of the treatment protocol. The malignant cells were large, had high nucleocytoplasmic ratios, and highly pleomorphic, frequently polylobated nuclei with coarse chromatin and 1-3 visible nucleoli. These CSF cytologic features differed significantly from those of classic medulloblastoma, which usually shows small cells with rounded, rather uniform nuclei with fine ("blastic") chromatin. The differential diagnosis of anaplastic medulloblastoma is broader than that of classic medulloblastoma, as it includes metastatic carcinomas and large cell lymphoma, a differential diagnosis especially pertinent in this patient with a history of autologous hematopoietic stem cell transplant. Awareness of these unusual but distinctive cytologic features is important for the accurate diagnosis of anaplastic medulloblastomas in CSF specimens and to avoid possible diagnostic pitfalls.

Overexpression of CREB reduces CRE-mediated transcription: behavioral and cellular analyses in transgenic mice.

The CREB transcription factor mediates neuronal plasticity in many systems, but the relationship between CREB levels and CRE-mediated transcription in individual neurons in vivo is unclear. In FVB/N nontransgenic mice, we observed that Purkinje cells showed low basal levels of Ser(133)-phosphorylated CREB protein yet displayed strong CRE-directed transcription. Transgenic mice overexpressing CREB in Purkinje cells and dentate gyrus granule cells showed a decreased CRE-lacZ signal in the same cells, indicating repression of ATF/CREB family function. Dentate region long-term potentiation was not altered by these changes in CREB expression. CREB transgenic mice demonstrated an inability to perform the rotarod task, without signs of overt ataxia. Our results demonstrate that the level of phosphorylated CREB protein is not a reliable indicator of CRE-mediated function. Furthermore, we conclude that CRE-mediated transcription may be linked to only a subset of cerebellum-mediated motor behaviors and may not be universally required for long-lasting synaptic potentiation.