Hashimoto's encephalopathy presenting with musical hallucinosis

Journal of Neurology -     

Hashimoto's encephalopathy presenting with bipolar affective disorder

OBJECTIVE: We report on a rare case of Hashimoto's encephalopathy associated with a bipolar affective disorder. CASE REPORT: A 32-year-old woman presented with a bipolar affective disorder and Hashimoto's thyroiditis. Neurological investigations (magnetic resonance imaging of the brain and cerebrospinal fluid) did not reveal any pathological findings except for a pathological electroencephalogram (EEG). Despite consequent antidepressant treatment, the patient remitted only in conjunction with normalization of the EEG after short-term treatment with high doses of prednisolone. CONCLUSION: In treatment resistant courses of disorders, the thorough clinical and laboratory investigation of our patient revealed a very efficient treatment strategy. Cases of Hashimoto's encephalopathy associated with the occurrence of a manic episode and hence with bipolar disorder are rare; this is the first reported case. However, clinicians should be alert to this possibility.     

Hashimoto's encephalopathy

We describe a 59-year-old female with Hashimoto's encephalopathy whose presentation was characterized by a subacute onset of confusion, depressed level of consciousness, tremor and pyramidal signs. She was euthyroid on presentation but antithyroid antibodies were elevated. Hashimoto's thyroiditis was confirmed by thyroid biopsy. There was a spontaneous remission of the neurological disorder. Subacute encephalopathy is often investigated with thyroid function tests only. In cases of unexplained encephalopathy, thyroid antibodies and/or thyroid biopsy should be performed to exclude this condition.     

Hashimoto's encephalopathy with bilateral pallidal lesions presenting memory disturbance and executive dysfunction]

A 76-year-old man began to mistake his family for another person, and fall asleep easily while watching TV. He was treated with donepezil but without any effect. He was referred to our hospital on June. On admission, his consciousness was alert. Cranial nerves and motor functions were normal, but pathological reflexes were positive bilaterally. Serological examinations revealed high titers of antibodies against thyroglobulin and TPO, and antibody against alpha-enolase was positive. Total protein level in CSF was 40 mg/dl and cell counts were normal. On MRI, localized symmetrical lesions were observed in bilateral pallidum to genu of internal capsule. SPECT revealed hypoperfusion areas in bilateral striate bodies and frontal lobes. Neuropsychological examinations indicated impairment of executive function and procedural memory. The diagnosis of Hashimoto's encephalopathy was made and we treated the patient with oral prednisolone 60 mg/day followed by gradual tapering. After the treatment, clinical symptom as well as neuropsychological function improved. Neuropsychological impairment in this case was probably due to the disconnection of the thalamo-frontal projection. This case provides interesting suggestions that Hashimoto's encephalopathy may present with vasculitic infarctions in bilateral MCA perforators, and that this disease should be included in one of the differential diagnoses of cerebral infarctions of unknown etiology.     

Encephalopathy as the presenting symptom of Hashimoto's thyroiditis

In recent years, encephalopathy has increasingly been recognized as a complication of Hashimoto's thyroiditis. It can begin abruptly as a stroke-like event, acute seizures, or confusion, or as an insidious decline in cognitive function. Most reported cases have been on adult patients, although this encephalopathy does affect children as well. This form of encephalopathy should be considered in the differential diagnosis of children and adults with unexplained neurologic deterioration. We describe the case of a child in whom acute encephalopathy was the presenting symptom of Hashimoto's thyroiditis.     

Long-term treatment of Hashimoto's encephalopathy

Hashimoto's encephalopathy (HE) has been described as an encephalopathy, with acute or subacute onset, accompanied by seizures, tremor, myoclonus, ataxia, psychosis, and stroke-like episodes, with a relapsing/remitting or progressive course. HE patients have positive antithyroid antibodies, are usually in a subclinical hypothyroid state, have elevated cerebral spinal fluid (CSF) protein, and have nonspecific electroencephalogram (EEG) and imaging abnormalities in the absence of CNS infection, tumor, or stroke. The authors present two cases of HE, demonstrating an excellent response to high dose steroids acutely followed by long-term treatment with steroids and other immunomodulatory agents. A review of the literature is also provided.     

桥本氏脑病的临床特点

目的探讨桥本氏脑病(HE)的临床特点。方法对本院收治的11例和文献报道的17例HE患者的临床资料进行回顾性分析。结果本组男5例,女23例;年龄21～81岁;伴有甲状腺功能亢进3例,亚临床甲状腺功能亢进4例,亚临床甲状腺功能减低1例。首发症状为智能下降、癫痫、精神症状、头痛、卒中样发作、行走不稳、行动迟缓、构音和吞咽障碍。主要临床症状依次为智能下降23例(82.1%),癫痫发作14例(50.0%),精神症状14例(50.0%),意识障碍7例(25.0%),头痛7例(25.0%),行动迟缓4例(14.3%),发热3例(10.7%),卒中样发作3例(10.7%),构音/吞咽障碍2例(7.1%),复视1例,伴有心脏受累2例,多发大动脉炎及雷诺病各1例。26例患者查血甲状腺球蛋白抗体及27例查血甲状腺过氧化物酶抗体均显著升高。21例患者行脑电图检查,17例异常,均为全脑的广泛慢波。26例头颅MRI检查17例异常,主要累及皮质和皮质下白质,大多呈边界模糊的斑片状长T1、长T2;T2Flair呈高信号,部分为混杂信号。糖皮质激素治疗均有明显的疗效,1例死亡,其余预后良好。结论 HE多见于中年以上的女性,首发及主要症状以双侧脑皮质或皮质下白质损害症状为主,甲状腺相关抗体指标显著升高;MRI可见分布广泛的斑片状长T1、长T2信号病灶。早期糖皮质激素治疗预后较好。     

Recurrent status epilepticus as the main feature of Hashimoto's encephalopathy

Hashimoto's encephalopathy (HE) is a severe but treatable condition that rarely complicates Hashimoto's thyroiditis. Clinically it is characterized by progressive or relapsing symptoms, including tremor, myoclonus, stroke-like episodes, seizures, impairment of consciousness, and dementia. We describe a patient presenting with recurrent generalized convulsive status epilepticus (GCSE), despite antiepileptic medications, who was successfully treated with methylprednisolone. Our observation confirms that the clinical spectrum of HE at presentation is heterogeneous and diagnosis is often difficult. This case highlights the crucial importance of antithyroid antibody measurement in patients presenting with otherwise unexplained episodes of GCSE with or without adjunctive signs of encephalopathy or thyroiditis.     

桥本脑病二例

目的探讨桥本脑病的临床特点及其治疗方法。方法分析我院收治的2例桥本脑病患者的临床资料。结果两例患者分别以认知功能障碍及癫痫为首发症状,脑脊液检测、脑电图、头颅核磁共振检查未见特异性改变,但是血液甲状腺过氧化物酶抗体(TPOAb)明显增高,例1患者甲状腺功能正常,例2患者有甲亢。2例患者均对皮质类固醇激素敏感。结论临床上有以认知功能障碍、癫痫等症状为突出表现者,须考虑桥本脑病的可能。     

Sporadic hemiplegic migraine presenting as acute encephalopathy

A 10-year-old boy with psychomotor developmental delay and cerebellar vermis atrophy developed right hemiplegia with vomiting, unconsciousness, convulsions, and late-onset fever. Slow delta activity was noted over the left hemisphere on electroencephalography, and neuroimaging revealed swelling of the left temporo-occipital cerebral cortex with restricted diffusivity, successive transient cortical atrophy, and hyperperfusion over the left cerebral hemisphere. Interleukin-6 was elevated in the cerebrospinal fluid. The acute symptoms resolved completely within 3 weeks after onset, but hypoperfusion persisted in the left posterior cortex thereafter. Another episode with transient left hemiplegia appeared 7 months later, followed by recurrence of migraine attacks. Analysis of the CACNA1A gene revealed a mutation of c.1997 C>T (p.T666M). None of his family members had migraine. This case represents an unusual evolution of sporadic hemiplegic migraine with manifestations of acute encephalopathy, for which the role of migraine-related inflammatory process is assumed.     

Hashimoto's encephalopathy in children and adolescents

Hashimoto's encephalopathy is an underdiagnosed, steroid-responsive, progressive or relapsing encephalopathy associated with high titers of serum antithyroid antibodies. Although Hashimoto's encephalopathy is well documented in adults, it is rarely observed or studied in children and adolescents. We describe the clinical and laboratory findings of four children (aged 9-15 years) with Hashimoto's encephalopathy. The clinical features of two patients at presentation included epileptic seizures and confusion. The other presenting signs included breath-holding spells, behavioral problems, psychosis, and ataxia (one patient each). During their presentation, three patients were euthyroid, and one was hyperthyroid. All patients manifested increased antithyroid antibodies, and all improved with steroid treatment. Hashimoto's encephalopathy is rarely suspected at presentation. Therefore, greater awareness of its signs by clinicians is necessary for proper diagnoses.     

Hashimoto's encephalopathy: an anatomicoclinical observation

Hashimoto's encephalopathy (HE) is a rare neurological complication of chronic lymphocytic thyroiditis. As its clinical presentation is aspecific, other etiologies of acute encephalopathy have to be ruled out. We report the case of a 29-year old woman with neuropsychiatric signs preceding coma, myoclonus and epileptic seizures. Clinical and electroencephalographic features were consistent with the diagnosis of new variant of Creutzfeldt-Jakob disease. However, high titres of antithyroid antibodies in serum directed towards the diagnosis of HE. Despite oral steroids, the patient died five months later. Neuropathological findings ruled out spongiform encephalopathy and disclosed aspecific activated microglia. Our observation suggests that this process could be involved in the pathogenesis of HE. Even in the absence of clinical dysthyroidism, HE diagnosis has to be suspected in the settings of acute encephalopathy associated with seric antithyroid antibodies.