scholarly journals Sneddon’s Syndrome as rare and underdiagnosed cause of stroke: about two cases / A Síndrome de Sneddon como causa rara e subdiagnosticada de AVC: cerca de dois casos

2021 ◽  
Vol 4 (6) ◽  
pp. 28039-28042
Author(s):  
Ana Carolina Santiago Ribeiro ◽  
Maria Carolina Correia Amorim Casal Fartes ◽  
Débora de Paula Silva ◽  
Ana Carolina Perota Tavares ◽  
Luana Cordeiro Amorim da Silva ◽  
...  

Case 1. Female, 27 years old, with migraine with aura. Use of combined contraceptive therapy. She presented visual and cutaneous complaints for eight months, using prednisone 60mg/day due to the hypothesis of systemic vasculitis. On examination, right temporal hemianopsia, normal fundus of the eye, Raynaud ‘s phenomenon with necrosis stitches in fingers and livedo were noted. Brain MRI with diffusion restriction in the left occipital lobe, hypersignal foci in the parietal lobe, and head of the left caudate nucleus. Reticular Livedo (RL) compatible biopsy. Anticardiolipin IgM (13.3 U/mL), ESR (47mm/h in the 1st hour and 52mm/h in the 2nd hour), and VDRL 1:8 reagents. Normal CSF. Initially treated with warfarin, she has been using rivaroxaban for two years, remaining asymptomatic. Case 2. Female, 39 years old, with a report of sudden dysarthria and right hemiparesis, with a previous episode of vertigo. A lumbosacral spine MRI was performed, presenting no alterations. Normal CSF. Antiphospholipid antibodies: Beta-2-glycoprotein I IgM 21 UR/mL and IgG 9 UR/mL, lupus anticoagulant 0.98, anticardiolipin IgG 9.4 GPL and IgM 31 MPL. Cranial MRI showed old ischemic gaps and hyperintense areas on T2/FLAIR with diffusion restriction affecting the left paramedian portion of the pons, related to acute stroke. Presence of livedo in the distal region of the limbs. In current use of warfarin 7.5mg/day, presenting remission of symptoms. Discussion: Sneddon Syndrome (SS) is a non-inflammatory thrombotic vasculopathy characterized by the concomitant presence of livedo reticularis and stroke. Neurological manifestations are present in up to 77% of cases. It is more prevalent in women between 20 and 42 years old. Treatment is aimed at preventing potentially serious, disabling, and even fatal new vascular events.  Final Comments: SS is a rare syndrome that probably results from a series of acquired or congenital hemostatic abnormalities that preferentially involve the cutaneous and cerebral vascular beds. Although it has no specific biomarker, there are reports associating SS with antiphospholipid antibodies and VDRL reactor. Clinical presentation, laboratory findings and skin biopsy associated with an excellent response to anticoagulant treatment were fundamental in the diagnostic elucidation.

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Naomi Cret ◽  
Madalina Halalau ◽  
Shahab Rezvani ◽  
Alexandra Halalau

A common side effect of Fioricet (butalbital-acetaminophen-caffeine) is high blood pressure caused by the caffeine content. We report a case of a 54-year-old female who developed the worst headache of her life after taking 2 tablets of butalbital-acetaminophen-caffeine every six hours for three days before presenting to the emergency department, where her blood pressure was 178/87 mmHg. A brain MRI showed edema in the subcortical white matter of the right occipital lobe, right parietal lobe, and left occipital lobe. Posterior reversible encephalopathy syndrome (PRES) was diagnosed. The patient returned within the week with severe headaches, visual hallucinations, and a blood pressure of 150/80 mmHg. Repeat brain MRI showed slight improvement of edema. Her brain imaging studies completely normalized at 10 months after diagnosis. Although the patient received appropriate treatment, she was unable to make full recovery. Due to her inability to work and the constant pain, the patient continues to struggle with depression.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Shota Hirakata ◽  
Yusuke Sakiyama ◽  
Akiko Yoshimura ◽  
Mei Ikeda ◽  
Katsunori Takahata ◽  
...  

Abstract Background Granulomatous amoebic encephalitis (GAE) is an infrequent and fatal infectious disease worldwide. Antemortem diagnosis in this condition is very difficult because clinical manifestations and neuroimaging are nonspecific. Case presentation A 60-year-old Japanese woman was admitted with a chief complaint of left homonymous hemianopsia. Brain-MRI showed extensive necrotizing lesions enhanced by gadolinium, in the right frontal lobe, right occipital lobe, and left parietal lobe. Epithelioid granulomas of unknown etiology were found in the biopsied brain specimens. Shotgun metagenomic sequencing using a next-generation sequencer detected DNA fragments of Balamuthia mandrillaris in the tissue specimens. The diagnosis of granulomatous amoebic encephalitis was confirmed using an amoeba-specific polymerase chain reaction and immunostaining on the biopsied tissues. Conclusions Shotgun metagenomics is useful for the diagnosis of central nervous system infections such as GAE wherein the pathogens are difficult to identify.


2021 ◽  
pp. 43-48
Author(s):  
M R Shashikumar ◽  
Narasipur Lingaiah Rajendrakumar ◽  
Sanjay P ◽  
Nanjaraj Chakenalli Puttaraj ◽  
Shruti Shruti ◽  
...  

Introduction: HIE is often clinically suspected in the setting of a known perinatal stress event arising from a complicated or difcult delivery. The most sensitive and specic imaging technique for examining infants with suspected hypoxicischemic brain injury is MR imaging. It also gives information about the timing and specic patterns of injury and also suggest diagnoses other than HIE such as metabolic disorders and developmental disorders of the brain. MRI is also a useful tool in the determination of prognosis and also follow-up of HIE. Aim: This study was undertaken to evaluate the various MRI appearances of hypoxic ischemic encephalopathy in term and preterm neonates and to correlate the MRI appearances with clinical outcome. Materials and Methods: All neonates with history of birth asphyxia, referred for MRI examination to the Department of Radio-Diagnosis, K. R. Hospital attached to Mysore Medical College and Research Institute, during January 2018 to January 2019. All MRI scans were performed on GE optima MR360 1.5 Tesla. Sequences used were T1W axial, T1 FLAIR axial, T2W axial, T2 FLAIR axial, T1W sag, T2W coronal, T2W GRE axial, DWI axial and ADC maps. Results: 30 babies with clinically suspicion of HIE and positive ndings on MR imaging were evaluated in our study. Out of 30 babies, 18 were term and 12 babies were preterm. Periventricular luecomalacia is most common MRI pattern in preterm and central pattern in term neonates. 16 babies had abnormal developmental outcome at 6 months follow up study. 16 babies with diffusion restriction in corpus callosum, 12 had abnormal outcome. 7 out of 10 babies with loss of normal signal in internal capsule had abnormal outcome. Babies with diffusion restriction in basal ganglia had gross developmental delay. Conclusion: MRI is the modality of choice for evaluation of HIE because of excellent gray – white matter resolution, well-depiction of myelination pattern and multi-planar imaging capabilities. There is a strong relation between the MRI appearances of birth asphyxia and the clinical outcome. Therefore MRI has a strong role in prognosticating lesions.


2016 ◽  
Vol 32 (1) ◽  
pp. 47-51
Author(s):  
Imran Sarker ◽  
Maliha Hakim ◽  
Mahmudul Islam ◽  
Md Badrul Alam ◽  
Md Rafiqul Islam

Background and Objective:: Miller Fisher syndrome (MFS) is a variant of Guillan Barre syndrome characterized by ophthalmoplegia, ataxia and areflexia. Although self-limiting disease course is expected, disease modifying treatment options for MFS are no different than for GBS and include intravenous immune globulin (IVIG) and plasmapheresis. Here, we report a case of MFS presented with bilateral ptosis, ophthalmoplegia, ataxia with quadriparesis and normal NCS. Patient Methods: A 14- year-old young boy was admitted to our hospital with the complaints of double vision, vertigo, difficulty in walking, imbalance. He had no diarrhea or upper respiratory tract infection prior to this illness. On neurological examination, he had limited ability to move his eyes up and out, had bilateral ptosis, ataxia. The muscle strength was mildly impaired. The plantar reflexes were flexor and the deep tendon reflexes were absent. Results: The blood laboratory, CT and brain MRI were normal. In the first sample of CSF, there was no change. Subsequent sample after 14 days revealed high protein with albuminocytological dissociation. The NCS and EMG were normal. Anti GQ 1b antibody was negative. He showed marked improvement with conservative management. Conclusion: MFS is a rare disease that must be diagnosed with the clinical findings and in the following days the diagnosis can be supported by the laboratory findings. Bangladesh Journal of Neuroscience 2016; Vol. 32 (1): 47-51


2021 ◽  
Vol 11 ◽  
Author(s):  
Qian Shen ◽  
Jingjing Qu ◽  
Zhen Chen ◽  
Jianying Zhou

BackgroundAdvanced non-small cell lung cancer (NSCLC) harboring epidermal growth factor receptor (EGFR) mutations has been successfully treated with tyrosine kinase inhibitors (TKIs). However, resistance to osimertinib, a third-generation TKI, can be difficult to overcome in this small subset of patients and is attributed to secondary resistant mutations. Here, we report a case of acquired EGFR L858R/L718Q mutation with advanced NSCLC that resistant to osimertinib, which was successfully overcome using dacomitinib.Case PresentationA 64-year-old non-smoker woman was diagnosed with stage IV non-small cell lung adenocarcinoma with EGFR L858R mutation and brain metastasis in November 2018. Treatment with gefitinib and gamma knife radiosurgery was started as the first-line treatment. After 7 months, she experienced disease progression with increased primary lung lesions and switched to osimertinib based on an acquired EGFR T790M mutation. After another 4 months, the disease progressed, and she was switched to chemotherapy. During chemotherapy, brain MRI showed an increasing number of parietal lobe metastases. Hence, gamma knife radiosurgery was performed again. After 12 months, the disease progression resumed, and an EGFR L718Q mutation was found on biopsy. The patient was then challenged with dacomitinib, and the disease was partially responsive and under control for 6 months.ConclusionCurrently, there are no established guidelines for overcoming osimertinib resistance caused by the L718Q mutation. The acquired EGFR L718Q mutation in subsequent resistance to osimertinib could be overcome using dacomitinib, indicating a promising treatment option in the clinic.


2021 ◽  
Vol 36 (1) ◽  
Author(s):  
Venkatesan Sanjeevi ◽  
V. R. Roopesh Kumar

Abstract Background Achieving hemostasis during neurosurgical procedures within deep seated tumors is of paramount importance. Chemical methods like using ORC and gel foam are preferred to bipolar cautery as bipolar cautery causes thermal injury to the normal eloquent surrounding white matter fibers, thereby causing significant morbidities. In addition to the chemical methods, we advocate a new relatively simple mechanical method by using small size Foley catheter inflated with saline can achieve hemostasis in case of deep locating brain tumor surgery with diffuse oozing from the tumor bed and surrounding white matter tissues are of concern. The balloon tamponade effect of the inflated Foley catheter helps in achieving complete hemostasis without damaging the surrounding normal white matter parenchyma. Case presentation A 52-years-old female admitted with history of progressive drowsiness and altered sensorium. Brain MRI was done showing large right-sided trigonal meningioma. Right parieto-occipital craniotomy was done, through the superior parietal lobe, corticotomy was done, and tumor was reached. Gentle retraction was done using curved blades in between the tumor and normal brain parenchyma. During surgery, following tumor removal, there was a diffuse oozing from tumor bed and the surrounding stretched white matter fibers. Hemostasis was attempted with chemical methods like ORC and gel foam. We avoided bipolar cautery to prevent thermal injury to the normal stretched eloquent white matter, as bleeding was not settled over the period of 45 min using chemical methods. Then, we placed a 10 F size Foley catheter in the tumor cavity and inflated with 6 ml of saline over the period of 10 min. Prior to Foley placement, we coated ORC over the tumor bed and the surrounding white matter. This achieved hemostasis to a significant extent and the same was repeated for another 10 min, and finally, complete hemostasis was achieved. Postoperative period went uneventful. Patient was discharged with good neurological recovery. Conclusion Inflatable Foley catheter balloon is a simple, cost-effective technique for achieving hemostasis in deep white matter tumors in addition to the routinely available hemostatic techniques.


2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Silvia Chiesa ◽  
Mario Balducci ◽  
Luigi Azario ◽  
Simona Gaudino ◽  
Francesco Cellini ◽  
...  

Purpose. To correlate site and diameter of brain metastases with hippocampal sparing in patients treated by RapidArc (RA) technique on whole brain with simultaneously integrated boost (SIB).Methods and Materials. An RA plan was calculated for brain metastases of 1-2-3 cm of diameter. The whole brain dose was 32.25 Gy (15 fractions), and SIB doses to brain metastases were 63 Gy (2 and 3 cm) or 70.8 Gy (1 cm). Plans were optimized and evaluated for conformity, target coverage, prescription isodose to target volume, homogeneity index, and hippocampal sparing.Results. Fifteen brain lesions and RA plan were generated. Hippocampal volume was 4.09 cm3, and hippocampal avoidance volume was 17.50 cm3. Related to site of metastases, the mean hippocampal dose was 9.68 Gy2for occipital lobe, 10.56 Gy2for frontal lobe, 10.56 Gy2for parietal lobe, 10.94 Gy2for deep brain structures, and 40.44 Gy2for temporal lobe. The mean hippocampal dose was 9.45 Gy2, 10.15 Gy2, and 11.70 Gy2for diameter’s metastases of 1.2 and 3 cm, respectively, excluding results relative to temporal brain lesions.Conclusions. Location more than size of metastases can adversely influence the hippocampus sparing. Further investigation is necessary to meet definitive considerations.


2006 ◽  
Vol 13 (12) ◽  
pp. 1364-1369 ◽  
Author(s):  
D. Intiso ◽  
F. Di Rienzo ◽  
G. Rinaldi ◽  
M. M. Zarrelli ◽  
G. M. Giannatempo ◽  
...  

2020 ◽  
Vol 8 ◽  
Author(s):  
Xiao-Ping Liu ◽  
Yi-Shuang Huang ◽  
Han-Bing Xia ◽  
Yi Sun ◽  
Xin-Ling Lang ◽  
...  

Background: Kawasaki disease (KD) is a form of systemic vasculitis that occurs primarily in children under the age of 5 years old. No single laboratory data can currently distinguish KD from other febrile infection diseases. The purpose of this study was to establish a laboratory data model that can differentiate between KD and other febrile diseases caused by an infection in order to prevent coronary artery complications in KD.Methods: This study consisted of a total of 800 children (249 KD and 551 age- and gender-matched non-KD febrile infection illness) as a case-control study. Laboratory findings were analyzed using univariable, multivariable logistic regression, and nomogram models.Results: We selected 562 children at random as the model group and 238 as the validation group. The predictive nomogram included high eosinophil percentage (100 points), high C-reactive protein (93 points), high alanine transaminase (84 points), low albumin (79 points), and high white blood cell (64 points), which generated an area under the curve of 0.873 for the model group and 0.905 for the validation group. Eosinophilia showed the highest OR: 5.015 (95% CI:−3.068–8.197) during multiple logistic regression. The sensitivity and specificity in the validation group were 84.1 and 86%, respectively. The calibration curves of the validation group for the probability of KD showed near an agreement to the actual probability.Conclusion: Eosinophilia is a major factor in this nomogram model and had high precision for predicting KD. This report is the first among the existing literature to demonstrate the important role of eosinophil in KD by nomogram.


2012 ◽  
Vol 30 (4) ◽  
pp. 307-315 ◽  
Author(s):  
Yu Zheng ◽  
Shanshan Qu ◽  
Na Wang ◽  
Limin Liu ◽  
Guanzhong Zhang ◽  
...  

Objective The aim of the present work was to observe the activation/deactivation of cerebral functional regions after electroacupuncture (EA) at Yintang (EX-HN3) and GV20 by functional MRI (fMRI). Design A total of 12 healthy volunteers were stimulated by EA at Yintang and GV20 for 30 min. Resting-state fMRI scans were performed before EA, and at 5 and 15 min after needle removal. Statistical parametric mapping was used to preprocess initial data, and regional homogeneity (ReHo) and amplitude of low-frequency fluctuation (ALFF) were analysed. Results ReHo at 5 min post stimulation showed increases in the left temporal lobe and cerebellum and decreases in the left parietal lobe, occipital lobe and right precuneus. At 15 min post stimulation, ReHo showed increases in the left fusiform gyrus; lingual gyrus; middle temporal gyrus; postcentral gyrus; limbic lobe; cingulate gyrus; paracentral lobule; cerebellum, posterior lobe, declive; right cuneus and cerebellum, anterior lobe, culmen. It also showed decreases in the left frontal lobe, parietal lobe, right temporal lobe, frontal lobe, parietal lobe and right cingulate gyrus. ALFF at 5 min post stimulation showed increases in the right temporal lobe, but decreases in the right limbic lobe and posterior cingulate gyrus. At 15 min post stimulation ALFF showed increases in the left frontal lobe, parietal lobe, occipital lobe, right temporal lobe, parietal lobe, occipital lobe and cerebellum, but decreases in the left frontal lobe, anterior cingulate gyrus, right frontal lobe and posterior cingulate gyrus. Conclusions After EA stimulation at Yintang and GV20, which are associated with psychiatric disorder treatments, changes were localised in the frontal lobe, cingulate gyrus and cerebellum. Changes were higher in number and intensity at 15 min than at 5 min after needle removal, demonstrating lasting and strong after-effects of EA on cerebral functional regions.


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