Hashimoto's Encephalopathy Presenting Research Articles

Orthostatic Myoclonus as a Presentation of Hashimoto Encephalopathy.

Orthostatic Myoclonus as a Presentation of Hashimoto Encephalopathy.

Hashimoto's encephalopathy presenting with psychotic symptoms.

Hashimoto's encephalopathy presenting with psychotic symptoms.

Hashimoto's Encephalopathy Presenting with Seizure and Neurocognitive Symptoms: A Case Report

Hashimoto's Encephalopathy Presenting with Seizure and Neurocognitive Symptoms: A Case Report

Hashimoto Encephalopathy Presenting with New Onset Refractory Status Epilepticus and Clinical Hyperthyroidism

NORSE occurs secondary to multiple etiologies including a rare diagnosis of Hashimoto Encephalopathy (HE). The mechanism of HE is not fully understood. Most reported patients with HE are hypothyroid or euthyroid at the time of presentation. Patients with HE commonly present with subacute clinical features, namely behavioral changes, and rarely with the acute presentation of status epilepticus. We report a case of a 14 years old female presenting with New Onset Refractory Status Epilepticus (NORSE) and clinical hyperthyroidism, subsequently developing behavioral symptoms with multiple relapses requiring various treatment modalities including pulse steroid therapy, intravenous immunoglobulin therapy, plasmapheresis and eventually partial thyroidectomy.

Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in 13 children

Objective: To analyze the clinical characteristics, treatment and prognosis of Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in children. Methods: A retrospective analysis was performed on the clinical features, laboratory tests, neuroelectrophysiological examination, imaging, treatment and outcomes of 13 patients with Hashimoto's encephalopathy presenting with isolated cerebellar ataxia, who were admitted to the Department of Pediatric Neurology of Guangzhou Women and Children's Medical Center from January 2016 to May 2021. Results: Among the 13 cases, 6 were males and 7 were females. The onset age was 2.6 (2.0,3.3) years, 9 children had precursor infection or vaccination before the first course of disease. All the 13 children had gait abnormalities or unsteady sitting, 10 had intentional tremor, 6 had dysarthria, 3 had body tremor, 2 had nystagmus, 3 had fatigue, 3 had hypotonia, 2 had vomiting and 1 had irritability. Thyroglobulin antibody (TgAb) was 500.0 (298.9,587.2) kU/L and thyroid peroxidase antibody (TPOAb) was 621.9 (449.6,869.4) kU/L in 13 cases. Autoantibodies were positive in 9 cases, and cerebrospinal fluid leukocytosis was seen in 4 cases. Regarding electroencephalography result, 4 cases had background slowing and 1 case had occasional sharp waves. Among the 3 patients who had relapses, 1 had cerebellar atrophy shown on cranial magnetic resonance imaging (MRI) during the recurrence. All the patients received intravenous immunoglobulin (IVIG) and intensive methylprednisolone therapy during the first onset, followed by the disappearance of the symptoms, 1 patient had repeated episodes which was decreased after immunosuppressive treatment with Rituximab.Followed up for 25.0 (22.5,33.3) months after the last episode, 12 achieved complete remission and 1 had a wide base gait. Conclusions: Trunk ataxia is the common symptom of Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in children.Children with cerebellar ataxia should be tested for TgAb and TPOAb to detect Hashimoto's encephalopathy, avoiding missed diagnosis and treatment delays; IVIG and intensive steroid therapy is effective, and immunosuppressive therapy for patients with multiple relapses could reduce the recurrence.

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

Hashimoto'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.

Autoimmune limbic encephalitis due to Hashimoto’s encephalopathy responding to therapeutic plasma exchange: A case report

AbstractA 66 years‐old lady presented with acute onset confusion and status epilepticus. MRI (Brain) showed T2 shine through at bilateral hippocampi. Cerebrospinal fluid revealed mild lymphocytic pleocytosis. Screening for infections, autoimmune and paraneoplastic conditions were negative except significantly high anti‐TPO antibody and hyperthyroid state. Her seizures needed multiple antiepileptic drugs. Limbic encephalitis was treated with high‐dose steroid but not responded and plasma exchange was proceeded. At two weeks after plasma exchange, her conscious level and seizure frequency were significantly improved. We present a case of Hashimoto's encephalopathy presenting with limbic encephalitis not responding to steroid but to plasma exchange.

Hashimoto's encephalopathy presenting with cognitive impairment and vertical supranuclear gaze palsy

Hashimoto's encephalopathy presenting with cognitive impairment and vertical supranuclear gaze palsy

Corticobasal syndrome like presentation of Hashimoto encephalopathy with cortical ribboning

Abstract Hashimoto encephalopathy (HE) and corticobasal syndrome (CBS) are heterogeneous disorders characterized by variable presentation; seizure, stroke like symptoms, myoclonus, alien limb phenomena, dystonia, limb apraxia, although extrapyramidal features and alien limb phenomena are rare in HE. Here we are reporting a 70 years female who presented with 3 months history of asymmetrical onset rapidly progressive disease associated with alien limb behaviour, myoclonic jerks, dystonia, seizure, speech impairment, asymmetrical extrapyramidal features (left > right), gait ataxia and cortical ribboning on brain imaging without visual disturbances, cognitive decline or neuropsychiatric abnormalities. EEG did not show any periodic discharges or slowing. Neuronal antibody VGKC was negative but she had euthyroid state with high titre of anti TPO antibody 590 IU/ml (normal upto 95 IU/ml). Therefore, diagnosis of Hashimoto encephalopathy in a probable sporadic case of corticobasal syndrome was made. Patient was treated with antiepileptic and IV steroid for 5 days. At the time of discharge she was conscious and followed verbal commands. In every case of rapidly progressive neurodegenerative disease like CBS, investigations for HE should be planned.

A case of Hashimoto's encephalopathy presenting with seizures and cognitive impairment

A case of Hashimoto's encephalopathy presenting with seizures and cognitive impairment

EPA-1360 – Hashimoto's encephalopathy presenting with neuropsychiatric symptoms

Introduction Hashimoto's encephalopathy is a rare neurological disorder of unknown cause associated with thyroid autoimmunity. The disease may present in two types - a sudden vasculitic type or a progressive subacute type associated to cognitive impairment and psychiatric symptoms. Objectives Report a case of a patient with Hashimoto's encephalopathy presenting with neuropsychiatric symptoms. Aims Highlight the diagnostic difficulties of medical disorder presenting with clinical features overlapping psychiatric syndromes. Methods Review of the scientific literature and patient's clinical notes. Results A 61-year old female without previous psychiatric history was referred to psychiatry consultation for depressive symptomatology in the last year. She was prescribed with antidepressants with some initial overall improvement, but gradually developed, during the next nine months, cognitive impairment with loss of skills to be independent and increasing tremor of the extremities. Neurological evaluation was requested which revealed frontal functions impairment. Blood workup only found increased titers of anti-thyroid antibody. EEG showed mild bitemporal slowing, brain MRI was normal and brain SPECT scan revealed fronto-temporo-parietal hypoperfusion. Hashimoto encephalopathy was presumed and corticoids were started with significant improvement of neuropsycological symptoms and tremor. After one year she remains assymptomatic. Conclusions Hashimoto's encephalopathy is a diagnosis of exclusion and clinical suspicion is essential for its identification. This unusual disorder is often unrecognized for a long time because of the multiple and protracted neurocognitive manifestations and normal findings in several different examinations. This case also highlights the importance for close collaboration between psychiatrists and neurologists.

Clinical Manifestations and Treatment Response of Steroid in Pediatric Hashimoto Encephalopathy

Hashimoto encephalopathy is a steroid-responsive encephalopathy associated with elevated titers of antithyroid antibodies. Clinical symptoms are characterized by behavioral and cognitive changes, speech disturbance, seizures, myoclonus, psychosis, hallucination, involuntary movements, cerebellar signs, and coma. The standard treatment is the use of corticosteroids along with the treatment of any concurrent dysthyroidism. Other options are immunoglobulins and plasmapheresis. We described symptoms and outcomes on 3 teenage girls with Hashimoto encephalopathy. Presenting symptoms were seizure or altered mental status. One patient took levothyroxine due to hypothyroidism before presentation of Hashimoto encephalopathy. After confirmation of elevated antithyroid antibodies, all patients were treated with steroids. One patient needed plasmapheresis because of the lack of response to steroids and immunoglobulins. Hashimoto encephalopathy should be considered in any patient presenting with acute or subacute unexplained encephalopathy and seizures. Even though the use of steroids is the first line of treatment, plasmapheresis can rescue steroid-resistant patients.

A Patient with Hashimoto's Encephalopathy Presenting with Convulsive Seizure Alone as the Initial Symptom

A 71-year-old Japanese woman with Sjögren syndrome, Hashimoto’s disease and a 6-month history of cognitive impairment was admitted to our hospital because of consciousness disturbance and convulsion. Her convulsive seizure disappeared by intravenous administration of diazepam following carbamazepine, and conscious level became alert the next day. But, her cognitive function was persistently deteriorated, and a score of mini-mental state examination (MMSE) was 17/30 points. Magnetic resonance imaging (MRI) and single photon emission computed tomography (SPECT) of the brain did not show any abnormal findings. The electroencephalogram showed increased slow waves in bilateral parieto-occipital regions. Serum anti-thyroglobulin antibodies were elevated (1780 U/ml), but thyroid function was within the normal range. In addition, anti-NAE (NH2-terminal of α-enolase) antibodies were positive. We diagnosed Hashimoto’s encephalopathy, and started steroid therapy. Her cognitive function gradually improved after steroid therapy, and convulsive seizure did not recur until 3 months later.We emphasize that Hashimoto’s encephalopathy should be considered even in patients with convulsive seizure of adult onset without thyroid dysfunction.

Autoimmune Thyroiditis-Associated Encephalopathy Without Clinical Steroid-Responsiveness and Recovery After Cyclophosphamide

Autoimmune Thyroiditis-Associated Encephalopathy Without Clinical Steroid-Responsiveness and Recovery After Cyclophosphamide

Hashimoto encephalopathy presenting as progressive myoclonus epilepsy syndrome

We report an 11 year old girl with multi-focal spontaneous myoclonus, generalized seizures and behavioural changes. She was found to have sub-clinical hypothyroidism and elevated anti-thyroid peroxidase antibodies. A diagnosis of Hashimoto encephalopathy (or Steroid Responsive Encephalopathy with Autoimmune Thyroiditis) was made in view of consistent clinical and laboratory features. The patient showed and maintained significant improvement with valproate, clonazepam and long term prednisolone. Other findings included mild cerebral cortical grey matter atrophy on brain magnetic resonance imaging and intermittent slowing with generalized poly-spike-and-wave discharges on electroencephalography. Other causes of progressive myoclonus epilepsy (PME) syndrome including subacute sclerosing panencephalitis, Lafora body disease, Juvenile Neuronal Ceroid Lipofuscinosis, Late onset gangliosidosis, Sialidosis, mitochondrial disorders and coeliac disease were ruled out by appropriate investigations. No other auto-immune abnormality was detected. This case emphasizes that Hashimoto encephalopathy should be considered in the etiologies of PME syndrome in adolescents since it is a treatable entity.

A case of Hashimoto's encephalopathy presenting with seizures and psychosis

Hashimoto's encephalopathy (HE) is a rare, poorly understood, autoimmune disease characterized by symptoms of acute or subacute encephalopathy associated with increased anti-thyroid antibody levels. Here, we report a case of a 14-year-old girl with HE and briefly review the literature. The patient presented with acute mental changes and seizures, but no evidence of infectious encephalitis. In the acute stage, the seizures did not respond to conventional antiepileptic drugs, including valproic acid, phenytoin, and topiramate. The clinical course was complicated by the development of acute psychosis, including bipolar mood, insomnia, agitation, and hallucinations. The diagnosis of HE was supported by positive results for antithyroperoxidase and antithyroglobulin antibodies. Treatment with methylprednisolone was effective; her psychosis improved and the number of seizures decreased. HE is a serious but curable, condition, which might be underdiagnosed if not suspected. Anti-thyroid antibodies must be measured for the diagnosis. HE should be considered in patients with diverse neuropsychiatric manifestations.

Non‐convulsive status epilepticus of frontal origin as the first manifestation of Hashimoto's encephalopathy

Hashimoto's encephalopathy is an often misdiagnosed, life threatening, condition which improves promptly with steroid therapy. Since clinical manifestations are heterogeneous and non-specific, the diagnosis is often difficult. Several case reports of Hashimoto's encephalopathy presenting with partial or generalised seizures are described, but only a few have focused on status epilepticus as the first clinical manifestation. We report two patients presenting with repetitive and prolonged seizures characterised by progressive reduction in contact and reactivity associated with frontal/diffuse polyspike-and-wave activities. This condition, which can be interpreted as a form of non-convulsive status epilepticus (NCSE) of frontal origin, was refractory to antiepileptic drugs but responded promptly to high doses of intravenous steroid treatment. In cases of unexplained encephalopathy with EEG documentation of NCSE, the early recognition and treatment of Hashimoto's encephalopathy may lead to a favourable prognosis. [Published with video sequences].

Hashimoto's encephalopathy presenting with neurocognitive symptoms: a case report

IntroductionHashimoto's encephalopathy is a neurological disorder of unknown cause associated with thyroid autoimmunity. The disease occurs primarily in the fifth decade of life and may present in two types - a sudden vasculitic type or a progressive subacute type associated to cognitive dysfunction, confusion and memory loss.Case presentationWe report the case of a 62-year-old Hispanic woman, previously healthy, who developed a subacute onset of declining upper brain function. Serologic studies demonstrated high levels of antithyroid antibodies. Electroencephalographic and magnetic resonance image findings were consistent with Hashimoto's encephalopathy.ConclusionHashimoto's encephalopathy is a diagnosis of exclusion. This unusual disorder is often under-recognized because of the multiple and protracted neurocognitive manifestations; therefore, it is important to be aware of the clinical manifestations to make a correct diagnosis.

Hashimoto Encephalopathy Presenting as Schizophrenia-like Disorder

Hashimoto encephalopathy (HE) is associated with numerous neuropsychiatric symptoms and responds well to steroid therapy. In the past, only a few cases were reported to present with pure psychiatric syndromes. We describe a case of HE with presenting symptoms like that of schizophrenic patients. We describe a 73-year-old woman with a history of autoimmune thyroiditis. She had psychotic symptoms for 3 years that responded poorly to antipsychotic agents, and she was thus admitted in 2007. The diagnosis of HE was made, although the patient presented neurologic symptoms and signs including abnormal electroencephalography, recent memory impairment, and executive function declination. The psychotic symptoms subsided completely in a few days after high-dose intravenous steroid therapy. The neuropsychiatric manifestation of HE can be similar to typical schizophrenia. Considering the effectiveness of steroid therapy for HE, we suggested HE as an important differential diagnosis for psychotic disorders, particularly for those patients of late onset, with abnormal electroencephalography, history of autoimmune thyroiditis, or poor response to conventional psychiatric treatment, so as to provide prompt and effective treatment for these patients.

Affective psychosis, Hashimoto's thyroiditis, and brain perfusion abnormalities: case report

BackgroundIt has recently become evident that circulating thyroid antibodies are found in excess among patients suffering from mood disorders. Moreover, a manic episode associated with Hashimoto's thyroiditis has recently been reported as the first case of bipolar disorder due to Hashimoto's encephalopathy. We report a case in which Hashimoto's thyroiditis was suspected to be involved in the deteriorating course of mood disorder and discuss potential pathogenic mechanisms linking thyroid autoimmunity with psychopathology.Case presentationA 43-year-old woman, with a history of recurrent depression since the age of 31, developed manic, psychotic, and soft neurological symptoms across the last three years in concomitance with her first diagnosis of Hashimoto's thyroiditis. The patient underwent a thorough medical and neurological workup. Circulating thyroperoxidase antibodies were highly elevated but thyroid function was adequately maintained with L-thyroxine substitution. EEG was normal and no other signs of current CNS inflammation were evidenced. However, brain magnetic resonance imaging evidenced several non-active lesions in the white matter from both hemispheres, suggestive of a non-specific past vasculitis. Brain single-photon emission computed tomography showed cortical perfusion asymmetry particularly between frontal lobes.ConclusionWe hypothesize that abnormalities in cortical perfusion might represent a pathogenic link between thyroid autoimmunity and mood disorders, and that the rare cases of severe Hashimoto's encephalopathy presenting with mood disorder might be only the tip of an iceberg.