Focal neurological presentation in Hashimoto's encephalopathy mimicking a vascular occlusion of the middle cerebral artery

Alazzeh, Ahmad; Jaroudi, Sarah; Gooch, Matthew; Peiris, Alan N.

doi:10.1136/bcr-2017-219933

Cited by 4 publications

(3 citation statements)

References 20 publications

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“…Obviously, constricted pupils are caused by opioid intoxication more often than by isolated pontine damage. But maybe less obviously, the whole range of possible metabolic disorders (depending on their severity) may also cause asymmetrical neurological abnormalities that may be misleading [21,[24][25][26][27][28][29]. Moreover, neurological abnormalities may have been pre-existing and may thus falsely suggest acute structural brain damage.…”

Section: Discussionmentioning

confidence: 99%

The diagnostic value of the neurological examination in coma of unknown etiology

et al. 2021

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Background Identifying the cause of non-traumatic coma in the emergency department is challenging. The clinical neurological examination is the most readily available tool to detect focal neurological deficits as indicators for cerebral causes of coma. Previously proposed clinical pathways have granted the interpretation of clinical findings a pivotal role in the diagnostic work-up. We aimed to identify the actual diagnostic reliability of the neurological examination with regard to identifying acute brain damage. Methods Eight hundred and fifty-three patients with coma of unknown etiology (CUE) were examined neurologically in the emergency department following a predefined routine. Coma-explaining pathologies were identified retrospectively and grouped into primary brain pathology with proof of acute brain damage and other causes without proof of acute structural pathology. Sensitivity, specificity and percentage of correct predictions of different examination protocols were calculated using contingency tables and binary logistic regression models. Results The full neurological examination was 74% sensitive and 60% specific to detect acute structural brain damage underlying CUE. Sensitivity and specificity were higher in non-sedated patients (87/61%) compared to sedated patients (64%/59%). A shortened four-item examination protocol focusing on pupils, gaze and pyramidal tract signs was only slightly less sensitive (67%) and more specific (65%). Conclusions Due to limited diagnostic reliability of the physical examination, the absence of focal neurological signs in acutely comatose patients should not defer from a complete work-up including brain imaging. In an emergency, a concise neurological examination should thus serve as one part of a multimodal diagnostic approach to CUE.

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Section: Discussionmentioning

confidence: 99%

The diagnostic value of the neurological examination in coma of unknown etiology

et al. 2021

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“…Majority of cases of SREAT run a fluctuating course with features such as cognitive impairment [ 9 ], seizures including status epilepticus [ 10 ] and myoclonus, tremor, ataxia, sleep disturbance, headache [ 11 ], depression, or psychosis [ 1 , 2 , 4 , 5 , 11 – 13 ]. Occurrence of focal neurological deficits has been described as well [ 8 , 14 ]. Recently, it has drawn much interest as a reversible cause of dementia [ 15 , 16 ].…”

Section: Discussionmentioning

confidence: 99%

“…To date, there are no accepted guidelines on treatment of SREAT. Therapeutic options used include intravenous or oral steroids [ 2 , 4 , 5 , 17 ], immune modulators such as azathioprine [ 14 ], methotrexate, cyclophosphamide, and intravenous immunoglobulin [ 12 ]. Our patient showed a dramatic improvement following intravenous steroid therapy.…”

Section: Discussionmentioning

confidence: 99%

Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting with Fever and Confusion

Liyanage

Munasinghe

Paramanantham

2017

Case Reports in Neurological Medicine

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Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) is a diagnostic conundrum as it may present with a myriad of nonspecific clinical features and laboratory and neuroimaging investigations are not diagnostic. We report a case of a 65-year-old female who presented with an acute febrile illness associated with headache and confusion, tangential thoughts, and loose association. Based on neutrophil leukocytosis in the full blood count and elevated inflammatory markers, she was commenced on empirical intravenous antibiotics suspecting meningoencephalitis. Further evaluation found a very high titer of both anti-thyroid peroxidase (anti-TPO) antibodies and anti-thyroid globulin antibodies. She was clinically and biochemically euthyroid. EEG showed right sided frontal intermittent rhythmic delta activity (FIRDA). Cranial MRI revealed age related cerebral atrophy and nonspecific periventricular white matter changes. A diagnosis of SREAT was made and she was treated with intravenous methylprednisolone followed by oral prednisolone. Her condition improved dramatically within 48 hours of starting steroids. SREAT is a diagnosis of exclusion in patients with a central nervous system disorder. There are no specific clinical features or investigative findings. Elevated anti-TPO antibodies are considered a hallmark of SREAT and steroid responsiveness supports the diagnosis. Prompt diagnosis and treatment reverses the neurological dysfunction in most cases.

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Clinical presentations and treatment outcomes of Hashimoto encephalopathy at Siriraj Hospital – Thailand’s largest national tertiary referral center

Dumrikarnlert,

Thakolwiboon,

Senanarong

2023

BMC Neurol

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Background Hashimoto encephalopathy has multiple clinical presentations, and other than the presence of thyroid antibody, laboratory and imaging investigations are all non-specific. Data specific to the clinical presentations and treatment outcomes of patients with Hashimoto encephalopathy in Thailand remain scarce. Objectives To retrospectively investigate the clinical presentations and treatment outcomes of patients with Hashimoto encephalopathy at Siriraj Hospital. Methods Patients who presented with acute encephalopathy at our center during July 2012-March 2017 were evaluated for eligibility. The inclusion criteria were positive anti-thyroperoxidase (anti-TPO) or anti-thyroglobulin (anti-Tg) in serum with negative neuronal antibody in serum or cerebral spinal fluid (CSF). Clinical presentations, symptom duration, laboratory results of thyroid status and thyroid autoantibody, CSF study, and clinical outcomes were collected. Results Of the 204 patients who presented with encephalopathy, 31 (15.2%) were positive for the anti-TPO or anti-Tg antibody. Of those, 13 patients met the diagnostic criteria for Hashimoto encephalopathy. Clinical presentations included cognitive impairment (76.9%), clouding of consciousness (46.2%), and behavior change (30.8%). The neuropsychiatric presentations were visual hallucination (30.8%), auditory hallucination (15.4%), delusion (7.7%), and mood disturbance (23.1%). Other clinical presentations included seizure (38.5%), abnormal movement (23.1%), sleep disturbance (38.5%), ataxia (46.2%), stroke-like episode (15.4%), and fever (15.4%). Most patients (76.9%) had onset within < 3 months. Regarding outcomes, 1 patient who did not receive corticosteroid died from status epilepticus and septic shock. Among the 12 patients who received corticosteroid, 9 (75%) had marked improvement, 1 (8.3%) had slight improvement, and 2 (16.6%) had no clinical improvement. Seven patients (53.9%) had normal thyroid function, 4 patients (30.8%) had subclinical hypothyroidism, and 2 patients (15.4%) had subclinical hyperthyroidism. Conclusions The results of this study revealed cognitive impairment, neuropsychiatric symptoms, seizure, ataxia, and sleep disturbance to be common manifestations of Hashimoto encephalopathy. This condition should always be considered in individuals with subacute onset of unexplained cognitive impairment or cerebellar ataxia. Laboratory and neuroimaging investigations were all found to be nonspecific in Hashimoto encephalopathy. Most patients responded well to treatment, so clinical suspicion and early diagnosis and treatment will lead to improved patient outcomes.

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Focal neurological presentation in Hashimoto's encephalopathy mimicking a vascular occlusion of the middle cerebral artery

Cited by 4 publications

References 20 publications

The diagnostic value of the neurological examination in coma of unknown etiology

The diagnostic value of the neurological examination in coma of unknown etiology

Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting with Fever and Confusion

Clinical presentations and treatment outcomes of Hashimoto encephalopathy at Siriraj Hospital – Thailand’s largest national tertiary referral center

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