小儿多系统萎缩症中的热十字包是一种不寻常的表现

Lokesh Naik Mude, O. Shukla, Rinki H. Shah, Rajesh Rambhai Barad
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摘要

一名 10 岁女性因主诉发烧、感冒和咳嗽 5 天,随后出现上肢无力,且无力程度超过下肢,伴有无法行走/站立,被送至急诊科。神经系统:神志不清,语调正常,力量:上肢3/3,下肢2/2,双侧膝关节反射亢进,双侧跖伸肌反射亢进;全身检查显示,患者有肢体功能减退、构音障碍、双侧运动迟缓、共济失调步态和锥体征。血液检查正常,通过神经影像学检查确诊:双侧中丘脑后部、双侧岛叶皮质、脑桥和双侧小脑中支对称性异常信号。异常信号延伸至脑桥,累及横桥小脑束和正中脑桥剑突核,出现 "热交叉包子征"(HCBS)。在治疗过程中,患儿出现自主神经紊乱。患儿接受了支持性药物治疗和甲基前列地龙治疗,随后口服类固醇。患儿对治疗反应良好,口服药物后无神经功能障碍,现已康复出院。我们的结论是,对于任何出现急性发作、持续时间较短的无力症状的患者,都必须对其自主神经功能障碍进行MSA评估,并采取适当的治疗措施。
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Hot cross bun in pediatric age multi-system atrophy an unusual presentation
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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