Τρίτη 23 Φεβρουαρίου 2021

Fatal hyperleukocytic T lymphoblastic lymphoma/leukemia complicated with multi-gene fusion and mutation: clinical revelation and perception

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Am J Blood Res. 2020 Dec 15;10(6):440-446. eCollection 2020.

ABSTRACT

T lymphoblastic lymphoma/leukemia (T-LBL/ALL) is a highly malignant hematological tumor common in young males. Most T-LBL/ALL patients usually initially seek medical treatment for clinical manifestations of non-hematological diseases. Presently, T-ALL chemotherapy is often used for the treatment of T-LBL/ALL internationally. With the application of high-intensity standard chemotherapy, the efficacy and prognosis of T-LBL/ALL are still not optimistic. The authors present a young male patient with facial and neck edema as the initial symptoms. This young patient of T-LBL/ALL was found to have a mediastinal mass after CT examination and he was finally diagnosed as highly malignant T-LBL/ALL. Unfortunately, after undergoing three standard courses of high-intensity chemotherapy, the young male patient eventually died of white blood cell stasis and severe infection caused by hyperleukocytosis. To this end, we find that the prognosis of T-LBL/ALL with multiple gene mutations or fusions and hyperleukocytosis, is extremely poor, and probably becomes a medical problem worthy of continuing resolution in the field of hematology and oncology.

PMID:33489453 | PMC:PMC7811898

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Effects of Ibrutinib on biophysical parameters of platelet in patients with chronic lymphocytic leukaemia

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Am J Blood Res. 2020 Dec 15;10(6):311-319. eCollection 2020.

ABSTRACT

Patients with chronic lymphocytic leukemia (CLL) treated with Ibrutinib often present hemorrhagic complications. Platelets dysfunction is well documented by aggregometry and flow cytometry, but the mechanisms by which Ibrutinib treatment influences the platelets status is yet to be evaluated. The aim of this study is to identify platelet membrane parameters in chronic lymphocytic leukemia (CLL) that could be altered by Ibrutinib administration. In this paper we propose a set of fluorescence measurements of the following parameters: membrane fluidity, resting membrane potential, and reactive oxygen species production of platelets suspensions obtained from CLL patients treated or not with Ibrutinib as markers for platelets status in this pathological situation. Platelets from CLL patients treated with Ibrutinib have higher membrane fluidity, lower resting membrane pote ntial and higher level of reactive oxygen species production compared to the untreated CLL patients. These patients are also presenting higher membrane fluidity and lower resting membrane potential compared to healthy volunteers.

PMID:33489439 | PMC:PMC7811905

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Reflux clinic: proof-of-concept of a Multidisciplinary European Clinic

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Eur Arch Otorhinolaryngol. 2021 Feb 23. doi: 10.1007/s00405-021-06705-9. Online ahead of print.

NO ABSTRACT

PMID:33620580 | DOI:10.1007/s00405-021-06705-9

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Cranial aseptic osteomyelitis associated with pyoderma gangrenosum

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Neurocirugia (Astur). 2021 Feb 2:S1130-1473(21)00001-4. doi: 10.1016/j.neucir.2020.11.007. Online ahead of print.

ABSTRACT

Neutrophilic dermatoses include a heterogeneous group of entities. Uncommonly, they can accumulate aseptic neutrophilic abscesses in other tissues in addition to the skin. A 34-year-old female complained of a headache which was unresponsive to usual drugs. A TAC revealed an osteolytic lesion in the right parietal bone. The biopsy showed osteomyelitis. One year later, pyoderma gangrenosum appeared in the anterior aspect of both legs. The headache and the cutaneous lesions disappeared after treatment with oral prednisone. The bone involvement in the background of neutrophilic dermatoses is exceptional. Usually, it involves children in the context of chronic recurrent multiple osteomyelitis (CRMO). Only two cases have been described in adults. One of them was a 26-year-old woman who had had CRMO since childhood, and the othe r one in contiguity with the cutaneous lesions of pyoderma gangrenosum.

PMID:33546980 | DOI:10.1016/j.neucir.2020.11.007

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3-Step didactic white matter dissection of human cerebellum: Micro-neuroanatomical training

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Neurocirugia (Astur). 2021 Feb 3:S1130-1473(20)30137-8. doi: 10.1016/j.neucir.2020.11.006. Online ahead of print.

ABSTRACT

OBJECTIVES: A thorough understanding of cerebellum anatomy is essential in 4th ventricle approaches (more frequent in pediatric neurosurgery), avoiding relevant complications such as cerebellar mutism. The aim of the present work is to show the feasibility of a didactic dissection of human cerebellum focusing on cerebellar peduncles and dentate nucleus (DN), which are structures at high risk during these surgical procedures.

MATERIAL AND METHODS: The cerebellum was dissected according to the Klingler method for white matter, using standard and specific microsurgery tools. Surgical microscope magnification (x6-x40) provided by a D.F. Vasconcellos M900 was required. A Canon EOS T7 18-55 mm digital camera was used and Adobe Lightroom Classic CC and Keynote were selected as photo enhancing software. Special m ethods such as LED light endoscopic transillumination were used for photographical reasons.

RESULTS: DN dissection was successfully achieved and the relations between these nucleus and the cerebellar peduncles, inferior vermis and medullary velums were described. Through this three steps dissection guide (1. tentorial surface; 2. suboccipital surface; 3. 4th ventricle structures), the most relevant anatomical structures were shown and its implications in different 4th ventricle approaches were characterised.

CONCLUSION: 3 D perspective provided by real specimen anatomical dissection is critical for learning neuroanatomy. LED transillumination was shown as a useful technique for the 4th ventricle structures photographic documentation which improves spatial recognition. This benefit can be applied for the study of the relations between the medullary velums and the rhomboid fossa foramina, which are permeable to light. The proposed three-ste ps dissection guide helps to a better understanding of human cerebellum and to gain self-confidence, allowing safer practice for neurosurgeons in all stages of their career.

PMID:33549481 | DOI:10.1016/j.neucir.2020.11.006

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A rare central nervous system tumor of childhood with spongiform appearance on brain magnetic resonance imaging; primary diffuse leptomeningeal oligodendrogliomatosis

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Neurocirugia (Astur). 2021 Feb 4:S1130-1473(21)00006-3. doi: 10.1016/j.neucir.2020.12.004. Online ahead of print.

ABSTRACT

Primary diffuse leptomeningeal oligodendrogliomatosis is a rare fatal tumor of childhood. Symptoms usually occur when the tumor causes hydrocephalus. Brain magnetic resonance imaging (MRI) may be nearly normal in the early stages of the disease, while hydrocephalus and multiple leptomeningeal cysts with spongiform appearance may appear later on. One may consider the diagnosis when radiologic findings become apparent with multiple leptomeningeal cysts. However, failure to recognize the imaging findings due to the rarity of the disease may delay the diagnosis. Here, we report a 3.5-year-old girl who presented with ataxia and vomiting and had a diagnosis of primary diffuse leptomeningeal glioneuronal tumor with remarkable brain MRI findings as diffuse multiple tiny cystic lesions on the brain and spinal cord. She benefited f rom radiotherapy and temozolomide treatment with remission of brain MRI findings. Increasing the number of reported cases will enable the elucidation of the disease's pathogenesis and the development of treatment protocols.

PMID:33549480 | DOI:10.1016/j.neucir.2020.12.004

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Neuromodulation of the anterior thalamic nucleus as a therapeutic option for difficult-to-control epilepsy

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Neurocirugia (Astur). 2021 Feb 4:S1130-1473(21)00002-6. doi: 10.1016/j.neucir.2020.12.001. Online ahead of print.

ABSTRACT

Deep brain stimulation (DBS) consists of the electrical stimulation of the subcortical structures by implanting electrodes connected to a pulse generator. The thalamus, being a structure that has multiple connections with various parts of the central nervous system, is a suitable target for DBS. The anterior thalamic nucleus (ANT) serves as an important relay site for the limbic system by receiving input from the hippocampus and mammillary bodies, and sending input to the cingulate gyrus; thus forming the Papez circuit. Due to these connections, the ANT constitutes an ideal route for the propagation of epileptogenic activity. ANT-DBS has excellent results in the control of complex partial seizures. The vast majority of patients with ANT-DBS have shown a significant reduction in the frequency of their seizures of more than 50%.

PMID:33551281 | DOI:10.1016/j.neucir.2020.12.001

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Spinal cord stimulation and cauda equina syndrome: Could it be a valid option? A report of two cases

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Neurocirugia (Astur). 2021 Feb 5:S1130-1473(21)00003-8. doi: 10.1016/j.neucir.2020.12.002. Online ahead of print.

ABSTRACT

Spinal cord stimulation (SCS) consists of the application of electrical stimuli to the dorsal columns of the spinal cord or to the posterior nerve roots in order to modulate the pain signals carried by the ascending pain pathways to the brain. Two cases of SCS in patients with cauda equina syndrome after lumbar surgery are presented. They were treated for persistent neuropathic pain but also experienced improvement in their motor and urinary symptoms after this treatment. Although the primary indication for SCS is neuropathic pain control, its application can also lead to improvement of motor deficits, sensory disorders, and urinary incontinence, as shown in these two cases. SCS will likely play a fundamental role in rehabilitative therapies in different neurological diseases. Further investigation in the field is needed.

PMID:33558146 | DOI:10.1016/j.neucir.2020.12.002

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Two-step treatment of a giant skull vault hemangioma: A rare case report and literature review

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Neurocirugia (Astur). 2021 Feb 8:S1130-1473(21)00007-5. doi: 10.1016/j.neucir.2020.12.005. Online ahead of print.

ABSTRACT

Skull vault hemangiomas are benign vascular tumours of the calvaria that are usually asymptomatic or present as firm, painless lumps. We present a case of a 59-year-old female with a giant intraosseous calvarial hemangioma that was admitted in our department with a palpable mass over the left frontoparietal region, personality changes and impaired emotional and cognitive functions. The patient was treated with a two-step approach involving endovascular and surgical treatment, and suffered two rare, but recognized complications, a contrecoup intracerebral haemorrhage and valproate-induced stupor and parkinsonism. At the 6-month follow-up, the patient had complete recovery with a good neurological outcome.

PMID:33573868 | DOI:10.1016/j.neucir.2020.12.005

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Primary spinal melanoma: A case report

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Neurocirugia (Astur). 2021 Feb 10:S1130-1473(21)00004-X. doi: 10.1016/j.neucir.2020.12.003. Online ahead of print.

ABSTRACT

Primary spinal cord melanoma is a rare disease that accounts for only 1% of all melanocytomas. Here we report a case of primary melanoma of the cervical spinal cord. In our case, 26-year-old female who were admitted to the hospital for left arm pain. Spinal magnetic resonance image (MRI) revealed a spinal cord tumor at the level of C2-3. The MRI images showed that the tumor compressed the spinal cord. At surgery, the spinal cord was under pressure and covered with shaped blackish brown neoplastic tissue. There were not any metastatic lesions. The patient is still alive six months after surgery.

PMID:33581992 | DOI:10.1016/j.neucir.2020.12.003

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A rare case of a secondary pituitary abscess arising in a craniopharyngioma with atypical presentation and clinical course

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Neurocirugia (Astur). 2021 Feb 10:S1130-1473(21)00005-1. doi: 10.1016/j.neucir.2020.11.008. Online ahead of print.

ABSTRACT

Pituitary abscesses are very uncommon. They are divided into primary, arising within a healthy gland, and secondary, observed with an underlying pre-existing lesion. Here we present the eighth case reported of a secondary abscess within a craniopharyngioma. A 59-year-old-woman presented with a 3-week history of headache, and fever. Physical examination was unremarkable. An Magnetic Resonance Imaging (MRI) showed a pituitary lesion suggestive of a chronic inflammatory process. She was diagnosed with lymphocytic meningitis with hypophysitis and she was treated with corticosteroids. Two months later she presented with headache and fever again. Control MRI showed enlargement of the pituitary lesion. Therefore, a transsphenoidal biopsy was performed. During the procedure, purulent material was released. Histological study dem onstrated a craniopharyngioma and meningeal inflammation. Empiric antibiotics were started. Three months post-operatively, a follow-up MRI showed a suspect minimal residual mass. Secondary pituitary abscesses are rare. The key to successful management is a high index of suspicion. Transsphenoidal surgical evacuation plus antibiotics is the mainstay of treatment. Although most symptoms resolve, endocrinopathies improve only rarely.

PMID:33581993 | DOI:10.1016/j.neucir.2020.11.008

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