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  • 机译 脂肪栓塞和非惊厥性癫痫持续状态
    摘要:Fat embolism syndrome (FES) typically occurs following orthopedic trauma and may present with altered mental status and even coma. Nonconvulsive status epilepticus is an electroclinical state associated with an altered level of consciousness but lacking convulsive motor activity and has been reported in fat embolism. The diagnosis is clinical and is treated with supportive care, antiepileptic therapy, and sedation. A 56-year-old male presented with altered mental status following internal fixation for an acute right femur fracture due to a motor vehicle accident 24 hours earlier. Continued neuromonitoring revealed nonconvulsive status epilepticus. Magnetic resonance imaging of the brain showed multiple bilateral acute cerebral infarcts with a specific pattern favoring the diagnosis of fat embolism syndrome. He was found to have a significant right to left intracardiac shunt on a transesophageal echocardiogram. He improved substantially over time with supportive therapy, was successfully extubated on day 6, and discharged to inpatient rehabilitation on postoperative day 15. Fat embolisms can result in a wide range of neurologic manifestations. Nonrefractory nonconvulsive status epilepticus that responds to antiepileptic drugs, sedation, and supportive therapy can have a favorable outcome. A high index of suspicion and early recognition reduces the chances of unnecessary interventions and may improve survival.
  • 机译 神经内膜血管介入治疗后单侧上颈椎后路脊髓梗死:一例报告
    摘要:Context We describe a case of unilateral posterior upper cervical spinal cord infarction and propose a pathophysiologic mechanism causing this lesion after vertebral artery endovascular intervention.
  • 机译 急性横贯性脊髓炎患者颈椎管内残留玻璃碎片:一例报道并文献复习
    摘要:A 50-year-old male presented with a one-day history of right leg weakness, numbness, and urinary retention. Weakness was present for two weeks but worsened significantly during the last 24 hours. On the right there was sensory loss in the leg and below the Th8 dermatome. On the left there was sensory loss below the Th10 dermatome and distal loss of temperature sensation. Past medical history revealed a cervical trauma 30 years ago when a glass chip lodged into the left side of the neck. The patient did not seek medical attention after removing it himself. No neurological symptoms followed the incident. No cervical manipulation or other physical trauma occurred before current symptom onset. Magnetic resonance (MR) imaging showed features consistent with myelitis at the level of C4–Th3. At the level of C6–C7, a T1 and T2 hypointense lesion was noted. On computed tomography, this lesion was hyperdense and occupied the spinal canal and the left intervertebral foramen. It was deemed to be a glass fragment. Surgical removal was withheld because the fragment was clinically silent for 30 years, the risk of surgical removal would outweigh the benefits and the patient did not prefer surgical treatment. Acute demyelinating transverse myelitis was diagnosed and treated with methylprednisolone. 10 months later MR features of myelitis resolved and the patient's neurological condition improved. Our case shows that foreign bodies in the cervical spinal canal can remain asymptomatic for up to 30 years. In the case of a long asymptomatic retention period the need for surgical removal of a foreign body must be carefully evaluated, taking into account the probability that a foreign body is the cause of current symptoms, risk of a foreign body causing damage in the future, risk of damage to the spinal cord during removal, and probability of therapeutic success.
  • 机译 帕金森病与病理123I-FP-CIT SPECT(DaTSCAN)结果相关,是散发性Creutzfeldt-Jakob病的最初表现
    摘要:Sporadic Creutzfeldt-Jakob disease (sCJD) is a type of progressive, subacute encephalopathy associated with spongiform degeneration of the central nervous system. sCJD includes a broad and heterogeneous spectrum of clinical variants, but extrapyramidal symptoms and signs at disease onset were rarely reported. We describe a case of unilateral parkinsonism associated with pathological 123I-ioflupane SPECT (DaTSCAN) results as the initial manifestation of M129V subtype sCJD patient. To the best of our knowledge, only 2 cases of Creutzfeldt-Jakob disease demonstrating nigrostriatal dopaminergic deficits in vivo using DaTSCAN have been published in the literature.
  • 机译 CT灌注引导在蛛网膜下腔出血诱发的血管痉挛中放置有创脑灌注监测仪
    摘要:Background Vasospasm is a challenging component of the subarachnoid hemorrhage “syndrome” that is unpredictable and very difficult to monitor using noninvasive or invasive monitoring technologies in neurocritical units.
  • 机译 从芬戈莫德转用利妥昔单抗后广泛的多发性硬化症再激活
    摘要:During treatment with fingolimod, B cells are redistributed from blood to secondary lymphoid organs, where they are protected from the effect of anti-CD20 and other cell-depleting therapies. We describe a multiple sclerosis patient who had almost complete depletion of B cells in blood during and shortly after treatment with fingolimod. He developed severe disease activity resembling immune reconstitution syndrome after switching from fingolimod to rituximab, with first dose being six weeks after fingolimod cessation. Following recommendations from the Swedish MS Association, rituximab treatment was started as one single dose of 1000 mg. In patients treated with fingolimod, pathogenic B cells may still be sequestered in secondary lymph nodes if this dose is given early. To deplete such B cells as they egress from the lymph nodes, we propose that a second dose of rituximab a few weeks after the first dose should be considered.
  • 机译 原发性蝶窦淋巴瘤最初表现为单侧外展神经麻痹症状
    摘要:A 48-year-old man presented with 3 days of mild horizontal diplopia in the left direction, followed by the onset of headache 17 days later. A physical examination revealed isolated left abducens nerve palsy. Head computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed soft-tissue density neoplasms that occupied the sphenoidal sinus and further invaded to destroy the clivus. Immunohistochemical staining of neoplasms was performed from biopsies samples. The pathological diagnosis was extranodal natural killer (NK)/T-cell lymphoma (ENKL), nasal type, associated with Epstein-Barr virus (EBV). The patient subsequently exhibited secondary symptoms (fever, night sweats), enlarged lymph nodes, renal metastases, and hemophagocytic syndrome, with clinical diagnosis stage IV of ENKL. The patient has a poor prognosis. This report is unique in two aspects: the unilateral abducens nerve palsy as the initial and isolated symptom of ENKL, and the primary sphenoidal sinus ENKL.
  • 机译 溃疡性结肠炎耀斑患者的脑静脉窦血栓形成
    摘要:Inflammatory bowel disease is characterized by a chronic inflammatory state and is therefore associated with abnormalities in coagulation and a hypercoagulable state. Cerebral venous sinus thrombosis is a rare complication of inflammatory bowel disease yet contributes significant morbidity and mortality to those affected. Early diagnosis is critical, as a delay in diagnosis portends a worse prognosis. This paper seeks to highlight the increased risk of venous sinus thrombosis in patients with inflammatory bowel disease. We start by discussing the case of a seventeen-year-old female who presented with ulcerative colitis flare and developed new-onset seizures, found to be caused by a large venous sinus thrombosis.
  • 机译 一种新型的CACNA1A无意义变体[c.4054C> T(p.Arg1352⁎)],引起阵发性共济失调2型
    摘要:Episodic ataxia is a heterogenous group of uncommon neurological disorders characterised by recurrent episodes of vertigo, dysarthria, and ataxia for which a variety of different genetic variations have been implicated. Episodic ataxia type two (EA2) is the most common and also has the largest number of identified causative genetic variants. Treatment with acetazolamide is effective in improving symptoms, so accurate diagnosis is essential. However, a large proportion of patients with EA2 have negative genetic testing. We present a patient with a typical history of EA2 who had a novel variant in the CACNA1A gene not previously described. Report of such variations is important in learning more about the disease and improving diagnostic yield for the patient.
  • 机译 蝶窦粘液囊肿继发的类丛集性头痛
    摘要:Background The great majority of cases of cluster headache (CH) are primary, but there are several reported cases of CH secondary to underlying structural lesions. The identification of these lesions is crucial for the achievement of an effective treatment and favorable outcome, although the determination of a cause-effect relationship between the two entities may be challenging.
  • 机译 远程小脑出血:脊柱外科手术的潜在医源性并发症。
    摘要:We report the case of a 51-year-old man with no significant past medical history, who underwent elective revision spinal surgery and subsequently developed intracranial hypotension, remote cerebellar haemorrhage (RCH), and mild hydrocephalus on the fourth postoperative day. Remote cerebellar haemorrhage is a known complication of supratentorial surgery. This iatrogenic phenomenon may also occur following spinal surgery, due to dural tearing and rapid cerebral spinal fluid (CSF) leakage, resulting in intracranial hypotension and cerebellar haemorrhage. This complication may result in severe permanent neurologic sequelae; hence, it is of pertinence to diagnose and manage it rapidly in order to optimise patient outcome.
  • 机译 系统性红斑狼疮继发的糖皮质激素综合症与抗磷脂综合症
    摘要:We report the case of a 53-year-old woman diagnosed with corticobasal syndrome (CBS) due to antiphospholipid syndrome (APS) secondary to systemic lupus erythematosus. Brain MRI showed marked cortical atrophy, several small infarctions in the deep white matter, and mild white matter changes, all of which were probably due to thrombosis manifestations of APS and could also be related to the CBS. To the best of our knowledge, this is the fourth reported case of CBS due to APS. It is noteworthy that although the common underlying pathologies of the CBS are neurodegenerative diseases, either primary or secondary APS can manifest itself as the CBS.
  • 机译 迟发性运动障碍为唯一症状的三叉脑膜瘤
    摘要:Most meningiomas are benign, encapsulated tumors (95% of the cases), generally undergoing a limited number of genetic aberrations. We present the case of a 74-year-old patient with no significant pathological history, who is admitted to the neurology ward for orofacial dyskinesias accompanied by hypoesthesia in the left hemiface, a symptomatology that had started insidiously about two months before and worsened progressively over the past 3 weeks. A cerebral MRI was performed which revealed a small mass with discrete T2 hyperintensity and T1 iso-signal compared to the gray matter located in the left pontine cistern, with a large, well-defined base at the level of the cerebral tentorium. The diagnosis of trigeminal meningioma was established and the treatment was started, after hearing the opinion of a neurosurgeon.
  • 机译 继发于多种触发因素引起的高钾血症的急性上升弛缓性麻痹
    摘要:Background Acute flaccid paralysis is an uncommon, but potentially life threatening, sequel of severe hyperkalemia. Reported primary aetiologies include renal failure, Addison's disease, potassium sparing diuretics, potassium supplements, and dietary excess. Coconut water, when consumed in excess, has been reported to cause severe hyperkalemia. We report the case of acute ascending flaccid paralysis secondary to hyperkalemia induced by multiple trigger factors—king coconut water, renal failure, diabetes, metabolic acidosis, and potassium sparing diuretics.
  • 机译 幻觉性多形性星形细胞瘤伴幻觉。
    摘要:Musical hallucinations are a relatively rare form of auditory hallucination characterized by hearing of music in the absence of any external stimuli. This phenomenon has been linked to both psychiatric and structural lesions. We present the case of a previously healthy young male whose presentation with musical hallucinations led to the diagnosis of a rare tumour, anaplastic pleomorphic xanthoastrocytoma.
  • 机译 帕金森纯合子外显子缺失引起的帕金森氏病,无路易体病
    摘要:Parkinson's disease (PD) is a clinical diagnosis based on the presence of cardinal motor signs, good response to levodopa, and no other explanations of the syndrome. Earlier diagnostic criteria required autopsy for a definite diagnosis based on neuronal loss in the substantia nigra pars compacta (SNpc) and the presence of Lewy bodies and neurites. Here, we present a patient who developed parkinsonism around the age of 20, with an excellent response to levodopa who, at age 65, received bilateral STN deep brain stimulation (DBS). The patient died at age 79. The autopsy showed severe neuronal loss in the SN without any Lewy bodies in the brainstem or in the hemispheres. Genetic screening revealed a homozygous deletion of exon 3-4 in the Parkin gene. In this case report we discuss earlier described pathological findings in Parkin cases without Lewy body pathology, the current diagnostic criteria for PD, and their clinical relevance.
  • 机译 诊断领域的盲点:肿瘤性多发性硬化症的具有挑战性的诊断
    摘要:Tumefactive Multiple Sclerosis (TMS) is a rare variant with 1 per 1000 cases of MS and 3 per million cases per year. TMS can mimic clinical and radiological features of a neoplasm, infarction, or abscess and therefore can be diagnostically challenging for clinicians. We present a clinical scenario of a patient presenting with left homonymous hemianopia with atypical radiological features initially thought to be more consistent with neoplasm or infraction. Ultimately, biopsy was done which led to the diagnosis of tumefactive multiple sclerosis.
  • 机译 Moyamoya病患者的高同型半胱氨酸血症
    摘要:Moyamoya disease is a chronic progressive cerebrovascular disease characterized by bilateral occlusion or stenosis of arteries around circle of Willis. We report a case of 18-year-old female presented with recurrent episodes of headache and vertigo. On cerebral angiography, the patient was diagnosed to have moyamoya disease. On further evaluation, thrombophilia profile showed increased homocysteine level. The patient was treated conservatively with cobalamin and aspirin and advised for revascularization. According to the literature, there are few case reports of moyamoya disease with thrombotic disorders. Hence, we are reporting this interesting and rare case.
  • 机译 后路可逆性脑病综合征表现不典型:2例报告
    摘要:Background Posterior reversible encephalopathy syndrome (PRES) is characterized by a variable association of symptoms including headache, consciousness impairment, visual disturbances, seizures, and focal neurological signs. Treating the underlying cause usually leads to partial or complete resolution of symptoms within days or weeks. Brain MRI findings include hyperintensities on T2-weighted sequences and their reversibility on follow-up exams. We describe two patients, one with an atypical clinical presentation characterized by a severe and prolonged impairment of consciousness and the other with atypical neuroimaging findings.
  • 机译 潜在的脑震荡可能导致阿尔茨海默氏症痴呆
    摘要:Chronic traumatic encephalopathy (CTE) has been receiving increasing attention due to press coverage of professional football players. The devastating sequelae of CTE compel us to aim for early diagnosis and treatment. However, by current standards, CTE is challenging to diagnose. Clear clinical diagnostic criteria for CTE have not been established. Only recently, pathological diagnostic criteria have been recognized, but postmortem diagnosis is too late. Reliable biomarkers are not available. By imaging criteria, cavum septum pellucidum has been the only consistent identifiable MRI finding. Because of the imprecise nature of diagnosis based on clinical suspicion, physicians must become cognizant of the broad spectrum of presentations of CTE. With this awareness, appropriate workup can be initiated. CTE can present with early symptoms of emotional changes or late symptoms with memory decline and dementia. Here we present an unusual case of a patient with Alzheimer's disease secondary to suspected CTE that stems from subconcussive head impacts presenting with severe memory and MRI changes. Clinicians should be aware of this presentation and consider CTE in their differential diagnoses while undergoing workup of memory disorders.

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