Primary familial brain calcification (PFBC) is an idiopathic pathology characterized by the development of calcific deposits in the supratentorial region such as telencephalon and diencephalon but also, in more extensive forms, in the cerebellum. Meningiomas are among the most common central nervous system (CNS) tumors generally related to a good prognosis. The simultaneous presence of intracerebral or extra-axial tumors and PFBC represents an exceedingly rare occurrence. A 72-year-old female patient was admitted to our department because of anoculogyric crisis followed by generalized seizures. She performed a brain CT scan which showed widespread hyperdensities in the paraventricular supratentorial region, basal ganglia and at the level of bilateral cerebellar dentate nuclei, characteristics of PFBC. Concomitant left frontal and smaller right temporal extra-axial lesions were identified and then confirmed in a brain MRI. The patient underwent a microsurgical resection of the lesion and subsequent histological examination reported a meningothelial meningioma (WHO Grade I). According to our literature review, this is the first paper that reports the coexistence of both intracranial meningiomas and PFBC. To date, it is not possible to provide an exact correlation between pathogenesis and genetic mechanism underlying this association.

Microphysiological system (MPS), a new technology for in vitro testing platforms, have been acknowledged as a strong tool for drug development. In the central nervous system (CNS), the blood‒brain barrier (BBB) limits the permeation of circulating substances from the blood vessels to the brain, thereby protecting the CNS from circulating xenobiotic compounds. At the same time, the BBB hinders drug development by introducing challenges at various stages, such as pharmacokinetics/pharmacodynamics (PK/PD), safety assessment, and efficacy assessment. To solve these problems, efforts are being made to develop a BBB MPS, particularly of a humanized type. In this study, we suggested minimal essential benchmark items to establish the BBB-likeness of a BBB MPS; these criteria support end users in determining the appropriate range of applications for a candidate BBB MPS. Furthermore, we examined these benchmark items in a two-dimensional (2D) humanized tricellular static transwell BBB MPS, the most conventional design of BBB MPS with human cell lines. Among the benchmark items, the efflux ratios of P-gp and BCRP showed high reproducibility in two independent facilities, while the directional transports meditated through Glut1 or TfR were not confirmed. We have organized the protocols of the experiments described above as standard operating procedures (SOPs). We here provide the SOPs with the flow chart including entire procedure and how to apply each SOP. Our study is important developmental step of BBB MPS towards the social acceptance, which enable end users to check and compare the performance the BBB MPSs.

Anti-myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG) associated disorder (MOGAD) is an immune-mediated central nervous system (CNS) inflammatory demyelinating disorder that has been widely recognized in recent years. It is distinct from multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD), which are separate disease spectrums. Here we report the case of a 5-year-old boy who was admitted for 3 days with fever, headache, and vomiting. Magnetic resonance imaging revealed abnormal hyperintensity in the left thalamus and positive serum IgM for M. pneumoniae. After treatment with azithromycin, the headache gradually disappeared, but paralysis and urinary retention occurred on the 6th day after admission. MRI re-examination showed that the original abnormal signal in the left thalamus was significantly weakened, but new abnormal signals appeared in the brain and cerebrospinal cord, and the serum MOG-IgG was positive. After treatment, the child has fully recovered and is still receiving follow-up care. We believe that this is a case of MOGAD in a child with a biphasic ADEM phenotype secondary to M. pneumoniae infection, which has potential value in elucidating the pathophysiology of MOGAD.

Although well described in the current literature, Neurocysticercosis [NCC] remains an enigma when confronted by practitioners. This is in part due to the haphazard nature of the parasitic infection on the central nervous system [CNS]. These include single or multiple anatomic sites of infection, stage of parasitosis, and the resultant inflammatory response. As a result, NCC can present with a complex constellation of symptomatic presentations, making therapeutic regiments highly individualized. Despite intervention, other impediments may arise post-therapy due to the nature of the infection. We present a case of rapidly progressive symptomatic NCC that initially was successfully treated, however would eventually succumb to complications of ventriculitis.

UNASSIGNED: Alectinib, a highly potent, highly selective, brain-penetrant anaplastic lymphoma kinase (ALK) inhibitor is now the first line therapy for patients with metastatic ALK-positive non small cell lung cancer (NSCLC).
UNASSIGNED: We report a rare case of pneumoperitoneum following alectinib initiation for metastatic non small cell lung cancer in a 74-year-old African American female. Patient developed abdominal pain approximately 2 weeks after starting alectinib. She was hemodynamically stable, and imaging revealed pneumoperitoneum. Patient was successfully managed non-operatively.
UNASSIGNED: Gastrointestinal perforation presenting as pneumoperitoneum is a very rare complication of alectinib. To our knowledge our patient is only the second case to be reported in the literature since its approval. The complication is likely attributable to the rapid tumor regression in the gastrointestinal tract. Non-operative management should be attempted if possible.
UNASSIGNED: Oncologists should be aware of the risk of gastrointestinal perforation when initiating cytotoxic chemotherapy on patients with metastatic NSCLC. A multidisciplinary approach is critical in appropriately individualizing care in this patient population.

\"Pediatric acute-onset neuropsychiatric syndrome\", or PANS, is a rare syndrome characterized by an acute onset of obsessive-compulsive disorder (OCD), and/or severely restricted food intake accompanied by a variety of neuropsychiatric symptoms. To our knowledge, this is the first case report of twin adolescents with COVID-19-associated PANS. Dizygotic twin sisters in late teens, with abrupt and acute onset of severely restrictive food intake, weight loss, OCD, anxiety with intermittent auditory and visual hallucinations, depression, attention deficit, and sleep disturbances, simultaneously accompanied by milder neurologic symptoms such as hand tremor, tinnitus, dizziness, headache, and weakness of proximal muscles, were applied to child and adolescent psychiatry clinic. The only relevant agent underlying those neuropsychiatric and somatic complaints was COVID-19, and it was validated with laboratory testing, such as positive IgG titers of SARS-CoV-2 and negative biomarkers for other possible bacterial or viral agents. Generalized epileptic anomaly and a vermian/folial atrophy in the cerebellum were detected in further evaluations. Treatment options consisted of psychotropic agents, antibiotics, antiepileptic, and intravenous immunoglobulin transfusion finely treated the neuropsychiatric symptoms. Clinicians should consider SARS-CoV-2 as a potential agent, when a child presents with abrupt onset, dramatic neuropsychiatric symptoms also consisting of PANS, even in asymptomatic patients or with mild respiratory symptoms.

UNASSIGNED: Craniopharyngiomas are benign tumours mainly confined to the cranial cavity in the suprasellar region.
UNASSIGNED: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented with a 4-month history of headache, visual disturbance, acute confusion and radicular leg pain. Previous history of breast carcinoma (ER ​+ ​PR ​+ ​HER2-) was noted. The importance of histological diagnosis prior to treatment of sellar or suprasellar lesions with atypical or aggressive features is explored.
UNASSIGNED: MRI demonstrated a partly solid and partly cystic pituitary mass lesion in the sellar and suprasellar region with chiasmal compression and hypothalamic involvement. The sella was mildly enlarged and there were no calcifications. Whole neuraxis MRI revealed intradural deposits involving the ventricular system, spinal cord and conus. Within a month, the lesion rapidly increased in size. The patient underwent a craniotomy and transventricular resection of the sellar and suprasellar mass. Cranial lesion histology favoured papillary craniopharyngioma, confirmed by BRAF V600 mutation. Lumbar puncture CSF cytology confirmed craniopharyngioma with BRAF mutation and no evidence of metastatic breast cancer.
UNASSIGNED: The patient remained confused postoperatively without focal neurological deficit and underwent palliative whole brain radiotherapy. She died 4 months later. A review of the literature identified 29 reports of ruptured craniopharyngioma.
UNASSIGNED: Ruptured craniopharyngioma presents with a suprasellar mass and drop lesions in the spinal canal, characteristics radiologically indistinguishable from metastatic disease. The importance of histological diagnoses in directing the management of these cases is highlighted.

Every organ can be affected by Rosai-Dorfman disease, the most common being skin and soft tissue, bone and upper respiratory tract. Here we present a Rosai-Dorfman disease patient who manifested with multiple organ involvement. A 33-year-old male presented with multiple joint pain, diarrhea, a purulent nasal discharge and sinus pain. After having serials of investigations he was misdiagnosed as having tuberculosis and fungal sinusitis. Finally, cytology from lymph node aspiration suggested Rosai-Dorfman disease. The sino nasal presentation of Rosai-Dorfman disease can be misdiagnosed as fungal sinusitis and the gastrointestinal manifestation can mimic gastrointestinal tuberculosis. Diffuse joint involvement could also be an initial manifestation of Rosai-Dorfman disease.

Invasive fungal infections caused by Candida species are increasingly observed in immunosuppressed patients. Candida albicans is the more often identified species and neurocandidiasis is associated with high mortality rates. Diagnosis and treatment of these infections are frequently challenging. We report a case of central nervous system infection caused by Candida famata in an HIV-1 infected patient. To our best knowledge this is just the second published case of neural infection by this agent.

Central vertigo is a result of vestibular structure dysfunction in the central nervous system. Currently, misdiagnoses between peripheral and central lesions are frequent, and diagnostic testing costs are high. Identifying the characteristics of these 2 conditions is challenging. We can provide better treatment if we can establish a diagnosis earlier. Cerebral cavernous malformation (CCM) at the cerebellum is a cerebellar lesion that causes symptoms of central vertigo. We report a patient, 20th years old, female, with vertigo for 1 month before being admitted. Vertigo was getting worse, and when the patient arrived at our hospital, vertigo was accompanied by headache, right and left abducens nerve palsy, horizontal nystagmus bidirectional, vertical nystagmus, and weakness on the right side of the body. A brain magnetic resonance imaging (MRI) was performed before surgery and shows a lesion suggestive of CCM at the cerebellum with a hemorrhagic component inside and non-communicating hydrocephalus. There is no vascular malformation based on digital subtraction angiography result. MRI is the most sensitive and specific modality for detecting CCM, whereas cerebral angiography rarely detects this malformation. The patient got surgical treatment, with suboccipital decompression procedures and CCM excision. The histopathological results after surgical treatment revealed a cerebral cavernous malformation. Vertigo, headache, double vision, and weakness on the right side of the body were resolved after surgery.