scholarly journals Steroid-Responsive Epilepsia Partialis Continua with Anti-Thyroid Antibodies: A Spectrum of Hashimoto's Encephalopathy

2014 ◽  
Vol 6 (2) ◽  
pp. 166-170 ◽  
Author(s):  
Hiroki Masuda ◽  
Masahiro Mori ◽  
Shoichi Ito ◽  
Toshiyuki Yagishita ◽  
Satoshi Kuwabara
2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Pelin Nar Senol ◽  
Aylin Bican Demir ◽  
Ibrahim Bora ◽  
Mustafa Bakar

Hashimoto’s encephalopathy is a rare disease which is thought to be autoimmune and steroid responsive. The syndrome is characterized by cognitive impairment, encephalopathy, psychiatric symptoms, and seizures associated with increased level of anti-thyroid antibodies. The exact pathophysiology underlying cerebral involvement is still lesser known. Although symptoms suggest a nonlesional encephalopathy in most of the cases, sometimes the clinical appearance can be subtle and may not respond to immunosuppressants or immunomodulatory agents. Here we report a case who presented with drowsiness and amnestic complaints associated with paroxysmal electroencephalography (EEG) abnormalities which could be treated only with an antiepileptic drug.


2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Murugan Selvaraj Karthik ◽  
Kulothungan Nandhini ◽  
Viswanath Subashini ◽  
Ramasamy Balakrishnan

Hashimoto’s encephalopathy (HE) is a rare autoimmune disorder with neurological and neuropsychiatric manifestations and elevated titres of anti-thyroid antibodies. Here we are reporting a case of HE in a 19-year-old girl who presented with seizure-like episodes, confusion, and behavioural disturbances with catatonic symptoms such as posturing, echopraxia, echolalia, and ambivalence. Patient did not respond to antipsychotics and anticonvulsants. On further investigation, patient was found to have high serum anti-TPO antibodies of about 1261 U/mL with euthyroid status, which supported a suspicion of HE. Our consultant neurologist confirmed the diagnosis and she was started on injection of methylprednisolone 750 mg OD. Since patient started showing clinical improvement, her antipsychotic medications were tapered off. On follow-up, patient has recovered and is functioning well. Since HE is a diagnosis of exclusion, very high anti-TPO antibodies and good response to steroids supported the diagnosis of HE in this patient after excluding other etiological possibilities. This case has been reported because the clinical presentation was predominantly neurobehavioural manifestations which is uncommon with HE.


Reumatismo ◽  
2018 ◽  
Vol 70 (4) ◽  
pp. 268-269
Author(s):  
C. Cosso ◽  
M. Ghio ◽  
M. Cutolo

Hashimoto’s encephalopathy (HE) is an autoimmune form of encephalopathy, associated with autoimmune thyroiditis. Its prevalence is estimated to be 2:100,000. HE is characterized by behavioral changes, mental confusion, dysarthria, ataxia, psychosis, paranoia, convulsions, hallucinations, headache and hyperthermia. Elevated thyroid antibodies are necessary for diagnosis and the disease responds dramatically to glucocorticoid therapy. We describe a patient with HE and panniculitis, an association reported twice in the literature.


2020 ◽  
Vol 11 (3) ◽  
pp. 198-199
Author(s):  
Tommaso Ercoli ◽  
Federica Maria Genitori ◽  
Giovanni Defazio ◽  
Antonella Muroni

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Lilibet Urias-Uribe ◽  
Emmanuel Valdez-Solis ◽  
Claudia González-Milán ◽  
Claudia Ramírez-Rentería ◽  
Aldo Ferreira-Hermosillo

We present the case of a patient with previous psychiatric illness, acutely exacerbated by thyroid storm due to Graves’ disease, in whom treatment with antipsychotics induced catatonia. These associations are extremely rare and may be confused with Hashimoto’s encephalopathy, especially in the presence of anti-thyroid antibodies in cerebrospinal fluid. The treatment consists in the control of the triggering disease (in this case the resolution of the thyrotoxicosis) and the use of benzodiazepines. However, in some cases, the resolution of psychiatric symptoms is partial and may require the use of electroconvulsive therapy.


Hashimoto's encephalopathy is a relapsing encephalopathy occurring in association with Hashimoto's thyroiditis (HT) with high titers of anti-thyroid antibodies. The mechanism of pathogenesis is unknown. Auto-antibodies to α-enolase have been found to be associated with Hashimoto's encephalopathy. Recently, the crucial role of neuro-inflammation in the development of psychological disorders including depression and anxiety has received more attention. Because the majority of patients with Hashimoto's encephalopathy respond to steroids or immuno-suppressant treatment, this condition is now also referred to as “steroid-responsive encephalopathy.” Initial treatment is usually with oral prednisone (50–150 mg/day) or high-dose IV methyl-Prednisolone (1 g/day) for 3-7 days. Thyroid hormone treatment is also included if required. This chapter explores Hashimoto's encephalopathy.


Author(s):  
Yasser Aladdin ◽  
Bader Shirah

AbstractHashimoto's encephalopathy is a rare immune-mediated disorder characterized by subacute encephalopathy with elevated thyroid antibodies. Hashimoto's encephalopathy is also known as steroid-responsive encephalopathy associated with autoimmune thyroiditis. We report a rare presentation of Hashimoto's encephalopathy presenting with acute neuropsychiatric disturbances, rapidly progressive dementia, seizures, and extrapyramidal failure. Neuroimaging revealed multifocal vasculitides of major cerebral vessels that support the autoimmune vasculitic theory as the underlying pathogenesis for Hashimoto's encephalopathy. Unfortunately, permanent irreversible cerebral damage has already ensued before her presentation to our center, which rendered steroid therapy ineffective. Serological testing for Hashimoto's thyroiditis must be in the investigation of all rapidly progressive dementias as early diagnosis and timely management of autoimmune thyroiditis may salvage sizable and eloquent cerebral tissues. The rarity of the condition should not preclude the investigation of Hashimoto's disease even in the presence of normal levels of thyroid hormones. Delayed diagnosis may result in irreversibly catastrophic encephalopathy in patients who once presented with potentially curable dementia.


2017 ◽  
Vol 6 (1) ◽  
pp. 136-142 ◽  
Author(s):  
Lokesh A. Rukmangadachar ◽  
Sudeepta Dandapat ◽  
Esther N. Bit-Ivan ◽  
Yen-Yi Peng

2020 ◽  
Vol 8 (1) ◽  
pp. 131
Author(s):  
Abarna Lakshmi R. ◽  
Rajganesh Ravichandran ◽  
Jaya Shree D. ◽  
Raveena P. B. ◽  
Nikhil Cherian Sam ◽  
...  

Hashimoto’s encephalopathy (HE) is a rare neuroendocrine disorder with high titers of the thyroid antibodies. HE is more common to women than in men with a ratio of approximately 5:1. The estimated prevalence of HE was 2 per 100,000 people. We present a case of Hashimoto’s encephalopathy in a 24-year-old male patient known case of hypothyroidism admitted with the complaints of unconsciousness, and further developed seizure during admission. Brain magnetic resonance imaging (MRI) was normal, electroencephalogram (EEG) revealed slow diffusion, and the serum thyroid function test showed a high concentration of anti-thyroid antibodies. The patient had a good recovery after the management with steroid therapy.  


Author(s):  
Eoin P. Flanagan ◽  
Richard J. Caselli

The historical term “Hashimoto’s encephalopathy” describes a serologically diverse spectrum of autoimmune encephalopathies that tend to be highly steroid responsive and unassociated with cancer. Patients typically present with subacute cognitive decline and serological evidence of autoimmunity that includes but is not limited to thyroid antibodies. An inflammatory spinal fluid and, in a subset of patients, MRI signal abnormalities are supportive. Patients often respond promptly to corticosteroids but relapses are typical. The continued discovery of novel neural-specific-autoantibodies such as voltage-gated-potassium-channel-autoantibodies associated with autoimmune encephalopathies highlights why the term “Hashimoto’s” is misleading. The authors advocate the more general term “autoimmune encephalopathy.”


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