scholarly journals Hashimoto's Encephalopathy Masquerading as Rapidly Progressive Dementia and Extrapyramidal Failure

2022 ◽  
Author(s):  
Yasser Aladdin ◽  
Bader Shirah
Keyword(s):  
Autoimmune Thyroiditis ◽  
Delayed Diagnosis ◽  
Thyroid Antibodies ◽  
Cerebral Damage ◽  
Serological Testing ◽  
Progressive Dementia ◽  
Rare Presentation ◽  
Hashimoto’S Encephalopathy ◽  
Immune Mediated ◽  
Underlying Pathogenesis

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.

scholarly journals Hashimoto’s encephalopathy in a patient with septal panniculitis: a case report

Reumatismo ◽  
2018 ◽  
Vol 70 (4) ◽  
pp. 268-269
Author(s):  
C. Cosso ◽  
M. Ghio ◽  
M. Cutolo
Keyword(s):  
Case Report ◽  
Autoimmune Thyroiditis ◽  
Behavioral Changes ◽  
Glucocorticoid Therapy ◽  
Thyroid Antibodies ◽  
Mental Confusion ◽  
Hashimoto’S Encephalopathy

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.

Can Anti-Thyroid Antibodies Influence the Outcome of Primary Chronic Immune Thrombocytopenia in Children?

2020 ◽  
Vol 20 (3) ◽  
pp. 351-355 ◽  
Author(s):  
Paola Giordano ◽  
Maurizio Delvecchio ◽  
Giuseppe Lassandro ◽  
Federica Valente ◽  
Valentina Palladino ◽  
...  
Keyword(s):  
Autoimmune Thyroiditis ◽  
Immune Thrombocytopenia ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Clinical Signs ◽  
Thyroid Antibodies ◽  
Chronic Itp ◽  
Immune Mediated

Background: Immune thrombocytopenia (ITP) is an acquired immune mediated disorder characterized by isolated thrombocytopenia. Pediatric ITP patients can develop autoantibodies such as anti-thyroglobulin (TG) and anti-thyroperoxidase (TPO), even in the absence of clinical signs of autoimmune disease. Objective: The purpose of this article is to provide a review about: 1) the prevalence of positivity of anti-thyroid antibodies (TPO and TG) in pediatric patients with chronic ITP; 2) the role of autoimmune thyroiditis on the outcome of chronic ITP. Method: The authors individually completed a review of the literature for this article. Retrospective and prospective clinical studies with pediatric cohorts were considered. Results: From the analysis of data, we found 4 papers which included studies only on pediatric population, and which corresponded to selected criteria. Pediatric ITP patients have been shown to have a statistically significant prevalence of anti-thyroid antibodies over healthy controls (11.6-36% versus 1.2-1.3%). No correlation has been found between the platelet count and the prevalence of positive anti-thyroid antibodies at any time of the follow up. Conclusion: The results of our bibliographic research demonstrated that: a) pediatric patients with chronic ITP tend to have a statistically significant prevalence of anti-thyroid antibodies positivity respect to general pediatric population; b) there are no clear data about the role of autoimmune thyroiditis as prognostic factor for chronic course of ITP in pediatric age.

scholarly journals Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting as Confusion, Dysphasia, and Myoclonus

2012 ◽  
Vol 2012 ◽  
pp. 1-2 ◽  
Author(s):  
S. A. Ryan ◽  
C. Kennedy ◽  
H. J. Harrington
Keyword(s):  
Clinical Features ◽  
Autoimmune Thyroiditis ◽  
Complete Response ◽  
Altered Level ◽  
Hashimoto’S Encephalopathy ◽  
The Core ◽  
Immune Mediated ◽  
Level Of Consciousness ◽  
Laboratory Results ◽  
Core Features

Steroid response encephalopathy associated with autoimmune thyroiditis (SREAT), or Hashimoto’s encephalopathy, is a rare disorder believed to be immune-mediated. It is most often characterized by a subacute onset of confusion with altered level of consciousness, seizures, and myoclonus. We describe the case of a 48-year-old gentleman who presented with confusion and dysphasia. Specific clinical features and laboratory results led to a diagnosis of Hashimoto’s encephalopathy. This case highlights the core features of this condition and the potential for complete response to steroid therapy.

Imperforate hymen mimicking acute appendicitis in an adolescent woman: a rare presentation

2021 ◽  
Vol 14 (3) ◽  
pp. e238547
Author(s):  
Victoria Rose Russell ◽  
Mohamed Ibrahim ◽  
Georgina Phillips ◽  
Tom Setchell ◽  
Sanjay Purkayastha
Keyword(s):  
Abdominal Pain ◽  
Acute Appendicitis ◽  
Iliac Fossa ◽  
Female Genital Tract ◽  
Delayed Diagnosis ◽  
Rare Presentation ◽  
Left Iliac Fossa ◽  
Imperforate Hymen ◽  
Retrograde Menstruation ◽  
Low Incidence

Imperforate hymen is a rare congenital malformation of the female genital tract. The condition poses several diagnostic challenges owing to its low incidence and often atypical presentation. Classical symptoms include amenorrhoea and cyclical abdominal pain. Delayed diagnosis leads to potentially irreversible and lifechanging sequelae including infertility, endometriosis and renal failure. A premenarchal 13-year-old girl with a background of chronic constipation presented with symptoms mimicking acute appendicitis. The underlying cause was imperforate hymen and retrograde menstruation. The diagnosis was made during diagnostic laparoscopy. As with this patient, pre-existing symptoms are often troublesome long before the true diagnosis is made. This case report highlights the importance of recognising imperforate hymen as a potential cause of acute abdominal pain in premenarchal adolescent girls. The clinical picture may present as right or left iliac fossa pain. Early identification reduces the risk of adverse complications and avoids unnecessary and potentially harmful interventions.

scholarly journals Paroxysmal Amnesia Attacks due to Hashimoto’s Encephalopathy

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Pelin Nar Senol ◽  
Aylin Bican Demir ◽  
Ibrahim Bora ◽  
Mustafa Bakar
Keyword(s):  
Cognitive Impairment ◽  
Antiepileptic Drug ◽  
Rare Disease ◽  
Psychiatric Symptoms ◽  
Thyroid Antibodies ◽  
Immunomodulatory Agents ◽  
Clinical Appearance ◽  
Hashimoto’S Encephalopathy ◽  
Eeg Abnormalities ◽  
Cerebral Involvement

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.

scholarly journals Hashimoto’s Encephalopathy Presenting with Unusual Behavioural Disturbances in an Adolescent Girl

2017 ◽  
Vol 2017 ◽  
pp. 1-3 ◽  
Author(s):  
Murugan Selvaraj Karthik ◽  
Kulothungan Nandhini ◽  
Viswanath Subashini ◽  
Ramasamy Balakrishnan
Keyword(s):  
Clinical Presentation ◽  
Autoimmune Disorder ◽  
High Serum ◽  
Thyroid Antibodies ◽  
Antipsychotic Medications ◽  
Hashimoto’S Encephalopathy ◽  
Neuropsychiatric Manifestations ◽  
Euthyroid Status ◽  
Very High

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.

scholarly journals A novel melanoma therapy stirs up a storm: ipilimumab-induced thyrotoxicosis

2015 ◽  
Vol 2015 ◽  
Author(s):  
Christine Yu ◽  
Inder J Chopra ◽  
Edward Ha
Keyword(s):  
Adverse Effects ◽  
T Lymphocyte ◽  
Autoimmune Thyroiditis ◽  
Cytotoxic T Lymphocyte ◽  
Alternative Therapy ◽  
Thyroid Storm ◽  
Iopanoic Acid ◽  
Immune Mediated ◽  
Thyroid Function Testing ◽  
Function Testing

Summary Ipilimumab, a novel therapy for metastatic melanoma, inhibits cytotoxic T-lymphocyte apoptosis, causing both antitumor activity and significant autoimmunity, including autoimmune thyroiditis. Steroids are frequently used in treatment of immune-related adverse events; however, a concern regarding the property of steroids to reduce therapeutic antitumor response exists. This study describes the first reported case of ipilimumab-associated thyroid storm and implicates iopanoic acid as an alternative therapy for immune-mediated adverse effects. An 88-year-old woman with metastatic melanoma presented with fatigue, anorexia, decreased functional status, and intermittent diarrhea for several months, shortly after initiation of ipilimumab – a recombinant human monoclonal antibody to the cytotoxic T-lymphocyte-associated antigen 4 (CTLA4). On arrival, she was febrile, tachycardic, and hypertensive with a wide pulse pressure, yet non-toxic appearing. She had diffuse, non-tender thyromegaly. An electrocardiogram (EKG) revealed supraventricular tachycardia. Blood, urine, and stool cultures were collected, and empiric antibiotics were started. A computed tomography (CT) angiogram of the chest was negative for pulmonary embolism or pneumonia, but confirmed a diffusely enlarged thyroid gland, which prompted thyroid function testing. TSH was decreased at 0.16 μIU/ml (normal 0.3–4.7); free tri-iodothyronine (T3) was markedly elevated at 1031 pg/dl (normal 249–405), as was free thyroxine (T4) at 5.6 ng/dl (normal 0.8–1.6). With iopanoic acid and methimazole therapy, she markedly improved within 48 h, which could be attributed to lowering of serum T3 with iopanoic acid rather than to any effect of the methimazole. Ipilimumab is a cause of overt thyrotoxicosis and its immune-mediated adverse effects can be treated with iopanoic acid, a potent inhibitor of T4-to-T3 conversion. Learning points While ipilimumab more commonly causes autoimmune thyroiditis, it can also cause thyroid storm and clinicians should include thyroid storm in their differential diagnosis for patients who present with systemic inflammatory response syndrome. Immune-related adverse reactions usually occur after 1–3 months of ipilimumab and baseline thyroid function testing should be completed before initiation with ipilimumab. Conflicting data exist on the use of prednisone for treatment of CTLA4 adverse effects and its attenuation of ipilimumab's antitumor effect. Iopanoic acid may be considered as an alternative therapy in this setting.

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