Academic literature on the topic 'MSA; Multiple System Atrophy; Hot cross bun sign'

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Journal articles on the topic "MSA; Multiple System Atrophy; Hot cross bun sign"

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Sheetal, Sasikumar, and Reji Thomas. "Hot Cross Bun Sign- A Case Series." Nepalese Journal of Radiology 11, no. 1 (2021): 19–23. http://dx.doi.org/10.3126/njr.v11i1.37172.

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Multiple system atrophy (MSA) is an adult onset, sporadic, progressive neurodegenerative disorder. It is an alpha synucleinopathy and the presentations include parkinsonism, cerebellar ataxia and autonomic symptoms in varying combinations. The two clinical phenotypes recognized are parkinsonian variant (MSA-P) and the cerebellar variant (MSA-C). Since many of the clinical features overlap with Parkninson’s disease, it is often misdiagnosed as Parkinson’s disease in the early stages. Since MSA is a progressive disorder with poor prognosis, it is important for the treating physician to recognize this entity and provide prognostication. MRI brain may help in differentiating between these two and an important radiological sign in MSA-c is the ‘Hot cross bun’ sign. It refers to the cruciform T2 hyperintense signal on axial magnetic resonance imaging (MRI) of the pons. We hereby report two patients who presented with progressive slowness in waking, ataxia, pyramidal signs and orthostatic hypotension, whose MRI brain showed the characteristic hot cross bun sign.
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Shiraishi, Wataru, Ayano Matsuyoshi, Yusuke Nakazawa, Yukiko Inamori, Akira Yogi, and Tetsuya Hashimoto. "Bright middle cerebellar peduncle sign in multiple system atrophy with predominant cerebellar ataxia is more apparent in double-inversion recovery imaging than in conventional imaging." PLOS ONE 19, no. 11 (2024): e0313651. http://dx.doi.org/10.1371/journal.pone.0313651.

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Multiple system atrophy (MSA) is a neurodegenerative disorder that presents as parkinsonism, cerebellar ataxia, and autonomic dysfunction. Magnetic resonance imaging (MRI) findings of MSA are reported to be the atrophy of the putamen/pons/cerebellum, hot cross bun sign, and bright middle cerebellar peduncle (MCP) sign. Here, we assessed the sensitivity of detecting the bright MCP sign in patients with MSA cerebellar variant (MSA-C) using a double inversion recovery (DIR) procedure, comparing it to the sensitivity of detection by T2-weighted image (T2WI) and fluid-attenuated inversion recovery (FLAIR) sequences on conventional MRI. We evaluated 6 MSA-C patients and 6 control patients (multiple sclerosis, neuromyelitis optica, and spinocerebellar atrophy). Characteristics of all the patients were collected, and MRI was analyzed. Two neurologists independently evaluated the visualization of the bright MCP sign using a 4-point visual grade from Grade 0 to Grade 3. Differences in grade between DIR and T2WI or FLAIR were statistically analyzed. Also, as a quantitative analysis, the signal intensity of the MCP lesion was compared with that of the ipsilateral thalamus, and the MCP/thalamus ratio was evaluated. As a result, DIR more sensitively showed the bright MCP signs of MSA-C patients than T2WI or FLAIR. Also, the bright MCP sign deteriorated and expanded over time in the cases we followed with MRI. We also evaluated hot cross bun sign in the pons, but the hot cross bun sign in MSA-C patients was not significantly different between the DIR and conventional MRI sequences. The DIR procedure can be a more sensitive method for detecting the involvement of MCP lesions in MSA-C.
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de Mello, Ramon Andrade Bezerra, Diana Ferreira, José Manuel Dias da Costa, Maria José Rosas, and João Manuel Quinaz. "Multiple-System Atrophy with Cerebellar Predominance Presenting as Respiratory Insufficiency and Vocal Cords Paralysis." Case Reports in Medicine 2010 (2010): 1–4. http://dx.doi.org/10.1155/2010/351239.

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Background. MSA (Multiple System Atrophy) may be associated either with Parkinsonism or with cerebellar ataxia (MSA-c subtype). It is considered a rare disease, but many patients are misdiagnosed as suffering from idiopathic Parkinson's disease. In this paper, we report a case of a patient admitted with respiratory failure and vocal cords paralysis due to MSA-c.Case Report. A 79-year-old Caucasian woman was admitted in March 2010 with dyspnea, asthenia, stridor, and respiratory failure needing noninvasive ventilation. She had orthostatic blood pressure decline, constipation, insomnia, daytime sleepiness, and snoring. The neurologic examination revealed cerebellar ataxia. A laryngoscopy revealed vocal cord paralysis in midline position and tracheostomy was performed. The Brain Magnetic Resonance Imaging revealed atrophy of middle cerebellar peduncles and pons with the “hot cross bun sign.”Conclusion. Although Multiple-system atrophy is a rare disease, unexplained respiratory failure, bilateral vocal cord paralysis, or stridor should lead to consider MSA as diagnosis.
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Wang, Jiaqi, Atsuhiko Sugiyama, Hajime Yokota, et al. "Diagnostic efficacy of the magnetic resonance T1w/T2w ratio for the middle cerebellar peduncle in multiple system atrophy and spinocerebellar ataxia: A preliminary study." PLOS ONE 17, no. 4 (2022): e0267024. http://dx.doi.org/10.1371/journal.pone.0267024.

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Background The standardized T1-weighted/T2-weighted (sT1w/T2w) ratio for the middle cerebellar peduncle (MCP) has been reported to be sensitive for detecting degenerative changes in the cerebellar subtype of multiple system atrophy (MSA-C), even in the early stages. We aimed to investigate the diagnostic value of the MCP sT1w/T2w ratio for differentiating between MSA-C and spinocerebellar ataxia (SCA). Methods We included 32 MSA-C, 8 SCA type 3 (SCA3), 16 SCA type 6 (SCA6) patients, and 17 controls, and the MCP sT1w/T2w ratio was analyzed using a region-of-interest approach. The diagnostic performance of the MCP sT1w/T2w ratio in discriminating among MSA-C, SCA3, and SCA6 was assessed and compared with diagnosis based on visual interpretation of MCP hyperintensities and the “hot cross bun” (HCB) sign. Results MCP sT1w/T2w ratio values were markedly lower in patients with MSA-C than in those with SCA3, those with SCA6, and controls (p < 0.001). The MCP sT1w/T2w ratio showed high diagnostic accuracy for distinguishing MSA-C from SCA3 (area under curve = 0.934), SCA6 (area under curve = 0.965), and controls (area under curve = 0.980). The diagnostic accuracy of the MCP sT1w/T2w ratio for differentiating MSA-C from SCA3 or SCA6 (90.0% for MSA-C vs. SCA3, and 91.7% for MSA-C vs. SCA6) was comparable to or superior than that of visual interpretation of MCP hyperintensities (80.0–87.5% in MSA-C vs. SCA3 and 87.6–97.9% in MSA-C vs. SCA6) or the HCB sign (72.5–80.0% in MSA-C vs. SCA3 and 77.1–93.8% in MSA-C vs. SCA6). Conclusions The MCP sT1w/T2w ratio might be a sensitive imaging-based marker for detecting MSA-C-related changes and differentiating MSA-C from SCA3 or SCA6.
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Eleanor, d’Ersu, Anwar NurAizaan, Al-Mayhani Talal, and Sidhu Meneka. "THUR 101 SCA-1 with unusual early dysautonomia and delayed cerebellar signs." Journal of Neurology, Neurosurgery & Psychiatry 89, no. 10 (2018): A10.3—A10. http://dx.doi.org/10.1136/jnnp-2018-abn.38.

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A 59-year-old right-handed bus driver presented with gradually worsening erectile dysfunction, urinary frequency and nocturia over 18 months. He had a past medical history of hypertension with regular medications including amlodipine, aspirin and atorvastatin. Initial examination revealed a mild bilateral intention tremor.The patient had undergone brain imaging showing brainstem and cerebellar atrophy with a ‘hot cross bun sign’. The patient was then referred for autonomic function tests that demonstrated mild cardiovascular autonomic changes, and suggested sympathetic dysfunction. A working diagnosis of multiple system atrophy (MSA) was made.Over the next two years, his tremor worsened with the development of slurred speech and unsteady gait. On examination, he had prominent fasciculations and wasting in the right shoulder, severe bilateral intention tremor with past-pointing. In addition he had Parkinsonism and signs of a sensorimotor neuropathy.This atypical clinical evolution with prominent neuropathy and cerebellar signs led to investigations for spinocerebellar ataxia (SCA), particularly type 2 or 3 (given the autonomic dysfunction). However, the genetic testing showed a mutation in ATXN1, confirming a diagnosis of SCA type 1.To our knowledge, this is the first reported case of prominent early autonomic dysfunction associated with SCA type 1.
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Takao, Masaki, Taro Kadowaki, Yutaka Tomita, Yoji Yoshida, and Ban Mihara. "'Hot-cross Bun Sign' of Multiple System Atrophy." Internal Medicine 46, no. 22 (2007): 1883. http://dx.doi.org/10.2169/internalmedicine.46.0514.

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7

Jain, Rajendra Singh, Sunil Kumar, and Shankar Tejwani. "Medullary Hot-Cross Bun Sign in Multiple System Atrophy-Cerebellar." Journal of Medical Imaging and Radiation Sciences 47, no. 1 (2016): 113–15. http://dx.doi.org/10.1016/j.jmir.2015.10.001.

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8

Mude, Lokesh Naik, Omprakash Shital Shukla, Rinki H. Shah, and Rajesh Rambhai Barad. "Hot cross bun in pediatric age multi-system atrophy an unusual presentation." International Journal of Contemporary Pediatrics 11, no. 2 (2024): 245–47. http://dx.doi.org/10.18203/2349-3291.ijcp20240105.

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A 10-year-old female was brought to emergency department with complaint fever, cold, and cough for 5 days followed by weakness of upper limb weakness more than Lower limb associated with inability to walk/stand, brought to emergency with altered sensorium. Nervous system: Confused and altered, tone is normal, power: 3/3 in upper limbs 2/2 in lower limbs and reflexes in bilateral knee brisk, bilateral plantar-extensor; On general examination revealed hypomimia, dysarthria and bilateral bradykinesia along with ataxic gait and pyramidal signs. Blood investigation were normal and diagnosis made by neuroimaging s/o: Bilaterally symmetrical abnormal signal in both postero-medial thalami, bilateral insular cortices, pons and bilateral middle cerebellar peduncles. It is extending into pons involving transverse pontocerebellar tracts and median pontine raphe nuclei giving 'Hot cross bun sign' (HCBS). During course of treatment child had autonomic disturbances. Child was treated with supportive medication and methyl-presdnisolone followed by oral steroid. Child had responded to treatment given and child has been discharged with no neurological deficit on oral medications. We concluded autonomic dysfunction in any patient presenting with acute onset of weakness with short duration must evaluate for MSA and institute appropriate treatment.
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9

Srivastava, T., S. Singh, V. Goyal, G. Shukla, and M. Behari. ""Hot cross bun" sign in two patients with multiple system atrophy-cerebellar." Neurology 64, no. 1 (2005): 128. http://dx.doi.org/10.1212/01.wnl.0000141862.49535.e1.

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10

Recio Bermejo, M., S. Navarro Muñoz, B. Espejo Martinez, C. Mangas Aveleira, and R. García Ruiz. "“Hot-cross bun sign” in multiple system atrophy: A presentation of 3 cases." Neurología (English Edition) 27, no. 5 (2012): 314–15. http://dx.doi.org/10.1016/j.nrleng.2011.12.003.

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Conference papers on the topic "MSA; Multiple System Atrophy; Hot cross bun sign"

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Teixeira, Igor de Lima e., Stella de Angelis Trivellato, Igor Oliveira da Fonseca, Danielle Patrícia Borges Margato, Rodrigo Bazan, and Arthur Oscar Schelp. "An Eye of a Tiger cannot see all the true: a case series." In XIII Congresso Paulista de Neurologia. Zeppelini Editorial e Comunicação, 2021. http://dx.doi.org/10.5327/1516-3180.224.

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Background: Multiple System Atrophy is a neurodegenerative disease with parkinsonism, cerebelar ataxia and autonomic failure. Neurodegeneration with Brain Iron Accumulation diseases are neurodegenerative diseases, as the Pantothenate Kinase-Associated Neurodegeneration, with a very distinct pattern on neuroimaging, known as the “Eye of the Tiger Sign”, which is rare in MSA but many studies confirm the role of striatal regions iron accumulation in parkinsonisms. Objective: We describe MSA patients with iron accumulation in striatal regions in neuroimaging. Methods: We report clinical cases from São Paulo State University-Brazil. Results: 62-year-old with 5 years of bradykinesia and stiffness progressing to wheelchair, REM sleep behavioral disorder, no improvement with levodopa, disarthrophonia and choking with gastrostomy after 3 years, associated to syncope episodes. Neurological examination showed blood pressure of 105x80mmHg lying down and 80x60mmHg standing up, severe disarthrophonia, anterocapitis, severe parkinsonism, postural instability and ataxia. Neuroimaging showed the “Eye of the Tiger”, “putaminal rim” and the “hot cross bun” signs. 78-year-old with 1.5 years syncope episodes, slow walking, falls, difficulty moving hands and feet and constipation. No improvement with levodopa. Neurological examination showed blood pressure of 130x80 mmHg lying down and 90x60 mmHg standing up, severe bradykinesia and stiffness, drooling, ataxia and “square-wave jerks”. Neuroimaging showed “Eye of Tiger” and bilateral “putaminal rim” signs and cerebellar atrophy. In both cases were excluded all differential diagnosis. Conclusions: Both cases fulfilled criteria for MSA, with the radiological sign of the “Eye of the Tiger”. We emphasize the importance of knowing this variation of MSA to avoid diagnostic confusion.
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