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Unilateral Poliosis And Vitiligo: A Case Report

机译:单侧性脊柱侧弯和白癜风:一例报告

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We report a 34-year old patient presenting with unilateral poliosis, vitiligo, epilepsy, and pseudoseizures without other characteristic signs of Vog-Koyanagi-Harada syndrome or Alezzandrini syndrome. Similar combinations have not been reported to medical literature. Introduction In clinical practice, patients presenting with decolouration of the skin, eyebrow, eyelashes, alopecia, chronic uveitis, and meningoencephalitis are diagnosed as Vogt-Koyanagi-Harada (VKHS) syndrome.1 In 1959, Arturo Alberto Alezzandrini, (Argentine ophthalmologist) reported a patient presenting with vitiligo, poliosis, unilateral pigmentary retinitis and hearing disturbances2. Another two patients were studied two years after their first report,3 and five years later another 3 patients with unilateral vitiligo, poliosis and ipsilateral retinal degeneration plus hypoacusia were described.4 Later, several patients presenting similar clinical features have been described. 5 6 Since then this combination of clinical features is known as Alezzandrini Syndrome (AS) and a small number of patients have been reported.7 8 Most of the patients diagnosed as VKHS reported in the medical literature showed bilateral poliosis, chronic uveitis, vitiligo and neurological manifestations, however unilateral manifestations of skin and hair decolouration plus retinal disease are named as AS preferably. We report a patient with unilateral poliosis and alopecia without ophthalmological or hearing problems, refractory epileptic seizures and pseudo seizures. Case Report A 34-year-old lady was referred to Neurology Clinic of the Nelson Mandela Academic Hospital, Umtata, South Africa because her uncontrolled seizures. She began to present fits in 1984 and those events were described as palpitations followed by blurred vision and jerking movements of the right arm for a few seconds followed by generalized tonic movements all over the body and loss of consciousness as well as urinary incontinence lasting 10 minutes approximately. It occurred at least once a day and after the seizure she experienced a generalized headache. She was on Carbamazepine 200 mg orally three times a day with very good respond in spite of defaulting treatment several times due to different reasons including unavailability of medications at the rural clinic. Typical pseudoseizures (PS) characterized by pedalling movements of the lower limb, stereotype position of the upper limbs, with normal pupils superficial reflexes, theses PS never happened when the patient remained alone and associated injuries such as biting of the tongue or post-ictal manifestations were not found. Three week prior to these seizures she noticed whitish discolouration of eyebrows, eyelashes and regimentation of the skin in her face. A traditional healer treated her unsuccessfully near her villages. On general examination, apart from whitening of the eyebrow and eyelashes on the right side, patchy loss of colour on the skin in the right frontal region and some areas of hyper pigmentation in the same region identified as veiling were seen (Figure 1 and 2)
机译:我们报道了一名34岁的病人,表现为单侧脊髓灰质炎,白癜风,癫痫和假性癫痫发作,没有其他Vog-Koyanagi-Harada综合征或Alezzandrini综合征的特征性体征。类似的组合尚未被医学文献报道。简介在临床实践中,表现出皮肤,眉毛,睫毛,脱发,慢性葡萄膜炎和脑膜脑炎脱色的患者被诊断为Vogt-Koyanagi-Harada(VKHS)综合征。11959年,Arturo Alberto Alezzandrini(阿根廷眼科医生)报告说患有白癜风,脊髓灰质炎,单侧色素性视网膜炎和听力障碍的患者2。在首次报告后两年对另外两名患者进行了研究[3],五年后又对另外3例单侧白癜风,脊髓灰质炎和同侧视网膜变性伴低视力低下的患者进行了研究。4随后,对几例具有相似临床特征的患者进行了描述。 5 6从那时起,这种临床特征的组合被称为Alezzandrini综合征(AS),并且已经报道了少数患者。78医学文献中报告的大多数诊断为VKHS的患者显示双侧脊髓灰质炎,慢性葡萄膜炎,白癜风和神经学表现,但是皮肤和头发脱色再加上视网膜疾病的单方面表现最好被命名为AS。我们报告了单侧小儿麻痹和脱发的患者,没有眼科或听力问题,难治性癫痫发作和假性癫痫发作。病例报告一名34岁的女士因无法控制的癫痫发作被转诊至南非Umtata纳尔逊·曼德拉学术医院的神经病学诊所。她于1984年开始出现健康状况,这些事件被描述为心followed,视力模糊和右臂猛烈抽动几秒钟,随后全身全身性强直运动,失去知觉以及尿失禁持续10分钟约。它每天至少发生一次,发作后她会全身性头痛。尽管由于多种原因(包括在农村诊所无法获得药物)而默认几次治疗,但她仍每天口服200毫克卡马西平200毫克,反应良好。典型的假性癫痫发作(PS),其特征是下肢的脚蹬运动,上肢的定型姿势,正常的学生的浅表反射,当患者独自一人并伴有诸如咬伤舌头或发作后表现等受伤时,这些PS从未发生找不到。癫痫发作前三周,她发现眉毛,睫毛和脸部皮肤发白发白。一位传统的治疗师在她的村庄附近给她治疗失败。在一般检查中,除了右侧的眉毛和睫毛变白之外,还可以看到右侧额叶区域的皮肤出现色斑的消失以及同一区域的一些色素沉着过度的区域(图1和2)。

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