The BIS value may decrease by cerebral hypoperfusion. We report a case in which the BIS value suddenly decreased during cervical spine surgery, which led us to find cervical screws compressing the vertebral arteries. In a 79-year-old man undergoing cervical spine surgery, the BIS suddenly decreased from about 40 to 10-20, about 4 h after the start of surgery. Intraoperative 3-dementional computed tomography indicated that both the two tips of cervical screws inserted in the 6th cervical vertebra were within bilateral transverse foramens. These cervical screws were removed, and the BIS increased immediately. The cervical screws were re-inserted again thorough the same vertebra into the bilateral transverse foramens, and the BIS decreased immediately. Postoperatively, cerebral hypoperfusion due to compression of bilateral vertebral arteries by two cervical screws was identified. The BIS may be a useful to detect cerebral hypoperfusion due to compression of the vertebral artery by a cervical screw.

Ascending aortic dissection is typically characterized by severe chest or back pain. However, its presentation can be atypical, leading to diagnostic challenges, especially in settings where classic symptomatology may not be evident. In this report, we described the case of a 74-year-old woman who presented to the emergency room of a rural community hospital with chief complaints of vertigo, nausea, and vomiting, without the classic symptoms of chest or back pain associated with aortic dissection. Despite initial treatment for autonomic dysregulation, the patient\'s symptoms persisted. Subsequent comprehensive assessments, including computed tomography angiography, revealed an ascending aortic dissection extending to the bilateral common carotid arteries. This atypical presentation, characterized by cerebral hypoperfusion and systemic hypotension without tachycardia, emphasizes the need to maintain a high suspicion index, even in the absence of hallmark symptoms. This case underscores the importance of considering the possibility of ascending aortic dissection in patients with nontraditional symptoms. Recognizing these atypical presentations is crucial for timely intervention, especially in rural settings with limited advanced diagnostic tools. This case also highlights potential sex disparities in symptom presentation, emphasizing the need for clinicians to recognize nontraditional symptoms in women. Rapid identification, evaluation, and management are imperative to prevent severe outcomes, and a multidisciplinary approach has proven to be the most effective in such cases.

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BACKGROUND: Bradycardia frequently occurs in heart-transplanted patients, mainly as a temporally restricted manifestation early after transplantation and often without symptoms. A high-degree atrioventricular block is mostly symptomatic through cerebral hypoxia induced through cerebral hypoperfusion. Only a few published cases show this specific electroencephalography result in this context. The purpose of this case is to bring attention to atypical manifestations of typical cardiac complications after heart transplantation and the importance of perseverance in the diagnostic.
METHODS: A Central European man in his 50s with history of heart transplantation 31 years previously was admitted to the internal medicine ward for short-lived recurrent episodes of generalized weakness with multiple falls but without loss of consciousness. During routine electroencephalography, the patient perceived this recurrent sensation. This episode coincided with a transient third-degree atrioventricular block followed 8-10 seconds later by a generalized slowing of the electroencephalography, reflecting cerebral hypoxia due to cerebral hypoperfusion. Holter monitoring confirmed the diagnosis. A pacemaker was implanted, consequently resolving the episodes.
CONCLUSIONS: This case report illustrates the pathophysiological central hypoxemic origin of episodes of generalized weakness caused by a high-degree atrioventricular block in a patient surviving 29 years after heart transplant. It highlights the benefit of electroencephalography as a diagnostic tool in well-selected patients.

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly-progressive dementing illness, the challenge of diagnosis during life. We presented a 78-year-old woman reported stupor, right facial palsy, and fluctuations in consciousness. 99mTc-ECD brain SPECT/CT with eZIS analysis revealed significant decreased regional cerebral blood flow mainly in specific regions of Alzheimer\'s disease as the published article reported with involving frontal region. Brain DWI MRI increased signal intensities corresponding to similar location of 99mTc-ECD brain SPECT/CT. In this case, we reported the pattern of decreased rCBF may correlate to rapidly progressive dementia and associated neurodegenerative symptoms of the highly suspected sCJD patient.

UNASSIGNED: Aorto-carotid bypass is a rare procedure. It is reported to be performed for management of cerebral malperfusion in the setting of supra-aortic branch vessel disease. Malperfusion requiring a bypass is largely secondary to dissection or vasculitis. Atherosclerotic disease of the supra-aortic branch vessels is commonly managed via an endovascular approach. We report a rare and atypical presentation of cerebral malperfusion in the setting of atherosclerotic disease of the innominate and carotid arteries managed with an aorto-carotid bypass graft.
METHODS: A case report of an 80-year-old female presenting with orthostatic mediated hypoperfusion transient ischaemic attacks with episodes of limb shaking and unilateral weakness with postural changes. The malperfusion was in the setting of severe atherosclerotic disease of the innominate and carotid arteries.
UNASSIGNED: Our patient was not amendable to endovascular intervention or a less invasive open approach. The patient underwent an aorto-carotid bypass graft with complete resolution of symptoms. This case highlights a rare manifestation of orthostatic mediated cerebral malperfusion and a successful novel treatment method.

BACKGROUND: Primary familial brain calcification (PFBC) is a rare inherited disease characterized by multiple calcified foci in the brain parenchyma. MYORG is the first gene found to be associated with autosomal recessive PFBC. The precise pathogenic mechanism of neurodegeneration in PFBC remains unclear. The clinical phenotypes of PFBC are variable, and there is no clear correlation between clinical manifestations and radiological and pathological features of calcification.
METHODS: Two sisters in a Taiwanese family presented with young-onset Parkinsonism and multifocal dystonia. Their brain CTs showed multiple intracerebral calcifications. The genetic study detected two heterozygous novel variants, c.104 T > A (p.Met35Lys) and c.850 T > C (p.Cys284Arg) in the MYORG gene. In both patients, MR susceptibility weighted images revealed calcification of the deep medullary veins. Tc99m ECD SPECT demonstrated a significant decrease of tracer uptake in the brain cortex and subcortical gray matter. Tc99m TRODAT-1 SPECT revealed decreased tracer uptake in the bilateral striatum.
CONCLUSIONS: Two novel MYORG variants were identified in Taiwanese family members presenting with PFBC. Abnormalities in the brain perfusion and dopamine transporter SPECTs suggest that cerebral ischemia due to extensive calcified vasculopathy, disruption of the basal ganglia-thalamo-cortical circuit, and nigrostriatal dopaminergic dysfunction are plausible pathogenic mechanisms of neurodegeneration in PFBC patients. Further investigation into the correlations between the pathogenicity-implicated imaging findings and the clinical phenotype are recommended.

Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke. We report the case of a patient with acute CVST, showing perfusion abnormalities on computed tomography perfusion with perfusion defect in the relevant hemisphere. The defect was found in a region adjacent to the occluded sinus and was not corresponding to an arterial territory. To the best of our knowledge this is the first ever report on CT perfusion abnormalities few hours after acute symptom onset in CVST.

Cerebral atrophy is a common finding in elderly patients; however, cerebrovascular disease causing progressive focal cerebral atrophy and dysfunction is unusual. In this report, we present 3 cases of hemicerebral atrophy due to ipsilateral internal carotid artery (ICA) stenosis or occlusion mimicking neurodegenerative conditions. Patient 1 had a frontal dysexecutive syndrome potentially consistent with a diagnosis of behavioral variant frontotemporal dementia; however, neuroimaging revealed a chronically occluded left ICA and a pattern of atrophy restricted to the left middle cerebral artery territory, suggestive of a vascular etiology. Patient 2 presented with progressively worsening seizures and right-sided weakness consistent with left hemispheric dysfunction, with radiographic evidence of left hemicerebral atrophy. Angiography revealed a chronic dissection of the left ICA leading to left cerebral hypoperfusion. Patient 3 had asymmetric parkinsonism, alien limb, and cognitive impairment consistent with a diagnosis of corticobasal syndrome. His imaging, however, revealed atrophy and encephalomalacia within the anterior circulation watershed territories with chronic, severe stenosis of the left ICA suggestive of a chronic hypoperfused state. In this case series, we report 3 examples of hemicerebral atrophy secondary to chronic ipsilateral ICA vascular disease with diverse progressive clinical symptoms mimicking primary neurodegenerative conditions. This case series highlights the importance of considering chronic hypoperfusion and large-vessel severe stenosis or occlusion in patients with cognitive impairment and evidence of asymmetric brain atrophy. In addition to symptomatic treatment, the management of vascular risk factors including treatment with antiplatelet agents, statins, and revascularization procedures can be considered.

An aberrant course of the internal carotid artery, such as a carotid loop or kinking, is a rare type of malformation. These malformations are typically asymptomatic, their occurrence is unpredictable, and they can exacerbate clinical disorders such as heart failure. We report the case of a seven-year-old child who died consecutively to cardiac arrest during otologic surgery for a cholesteatoma. After rapid and effective cardiorespiratory resuscitation, the child was transferred to a paediatric intensive care unit. In the following days, pronounced cerebral hypoperfusion led to the death of the child. The autopsy revealed a right carotid loop and a left carotid kink that presumably led to the cerebral hypoperfusion.