Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0751295 (memory loss)
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A case of presumed Hashimoto's encephalopathy (HE) is presented. The presentation included memory loss, delusions, functional decline and culminated in a generalized seizure. Anti-thyroid antibodies were detected and symptoms resolved with prednisolone. Patients with HE may present with prominent neuropsychiatric symptoms, attract psychiatric diagnoses and present to psychiatric services. Primarily a diagnosis of exclusion, HE should be considered in cases of encephalopathy in which standard investigations are negative.
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PMID:Reversible dementia with psychosis: Hashimoto's encephalopathy. 1710 12

Both severe thyrotoxicosis and hypothyroidism may affect brain function and cause a change in consciousness, as seen with a thyroid storm or myxedema coma. However, encephalopathy may also develop in patients with autoimmune thyroid diseases independent of actual thyroid function level, and this is known as Hashimoto's encephalopathy. Although most patients are found to have Hashimoto's thyroiditis, less frequently they have Graves' disease. Clinical manifestations include epilepsy, disturbance of consciousness, cognitive impairment, memory loss, myoclonus, hallucinations, stroke-like episodes, tremor, involuntary movements, language impairment, and gait impairment. Hashimoto's encephalopathy is a relatively rare disease. As a good response can be obtained with corticosteroid therapy, early diagnosis and treatment is very beneficial for patients. Here we report three patients with Hashimoto's encephalopathy with typical manifestations of hallucinations that were associated with hypothyroidism, hyperthyroidism, and euthyroid status, respectively. They all showed a dramatic response to methylprednisolone pulse therapy.
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PMID:Hashimoto's encephalopathy: report of three cases. 2544 53

A 48-year-old male patient presented with personality changes and progressive memory loss over 2 years with initially suspected Hashimoto's encephalopathy. Strategy of diagnostic workup of early onset dementia included dementia from neurodegenerative, neuroinflammatory, metabolic/toxic, and psychiatric origin. The patient's neurological exam was normal. MRI revealed a leukencephalopathy, predominantly in the frontal periventricular white matter, without notable changes over 2 years. On neurophysiological examination, prolonged central conduction times and a sensorimotor polyneuropathy were noted. Neuropsychological impairment included disorientation in place and a reduced short time memory. Behavioral alterations were predominated by sudden mood changes and disinhibition. Cerebrospinal fluid was normal. Despite presence of thyroid autoantibodies, glucocorticosteroid treatment did not improve the dementia. A metachromatic leukodystrophy was diagnosed by decreased arylsulfatase-A activity in leucocytes/fibroblasts and identification of a compound heterozygous mutation in the ARSA gene: c.542T>G (exon 3) and the novel mutation c.1013T>C (exon 6). Pathogenic function was suggested by bioinformatic mutation search. In a patient with early onset dementia, strategic diagnostic workup including genetic assessment revealed an adult-onset metachromatic leukodystrophy with a novel mutation in the arylsulfatase A gene.
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PMID:Late-Onset Metachromatic Leukodystrophy with Early Onset Dementia Associated with a Novel Missense Mutation in the Arylsulfatase A Gene. 2689 Jul 52

Hashimoto encephalopathy (HE) is characterized by heterogeneous neurological symptoms. HE is diagnosed based on three criteria-the presence of antithyroid antibodies, neurological symptoms from the cerebrum and/or cerebellum, and a positive response to immunotherapy. We clinically analyzed 18 patients (3 men, 15 women; age range, 38-81years) diagnosed with HE in our hospital from May 2013 to January 2016. Eleven patients showed sensory abnormalities such as strong pain, deep muscle pain, dysesthesia, paresthesia, or neuralgia. Surprisingly, the majority of the pain was distributed in a manner that was not explainable anatomically. Seventeen patients showed motor disturbances, such as weakness, paresis of extremities, or dexterity movement disorder, and eight patients showed give-way weakness, which is disruption of continuous muscle contraction. Other symptoms indicative of brain-related anomalies such as tremor, dystonia, involuntary movements, cerebellar ataxia, parkinsonism, memory loss, and chronic fatigue were also seen. In most patients, such motor, sensory, or higher brain functions were markedly improved with immunosuppressive therapies such as prednisolone, azathioprine, or immunoadsorption therapy. Although give-way weakness and anatomically unexplainable pain are typically considered as being psychogenic in origin, the presence of these symptoms is indicative of HE. HE exhibits diffuse involvement of the entire brain and thus, these symptoms are explainable. We propose that physicians should not diagnose somatoform disorders without first excluding autoimmune encephalopathy.
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PMID:[Clinical Features and Treatment of Hashimoto Encephalopathy]. 2766 88

Autoimmune encephalopathies are clinically and immunologically heterogeneous disorders. At least 20 types of autoimmune encephalopathies have been discovered, with the most common type being Hashimoto encephalopathy. In clinical situations, we often observe that patients with autoimmune encephalopathy are misdiagnosed because they exhibit signs similar to those observed in functional psychogenic movement, conversion, or somatoform disorders. We clinically examined over 100 patients with autoimmune encephalopathy. These patients primarily demonstrated motor disturbances, mostly with give-way weakness, sensory abnormalities, and involuntary movements such as tremor entrainment, dystonia, or coarse involuntary movement. In addition, we observed memory loss, psychogenic non-epileptic seizures, epilepsy, and autonomic symptoms in our patients. To diagnose autoimmune encephalopathies, we propose that a combination of neurological symptoms indicating "diffuse brain damage" be used. "Diffuse brain damage" is a proof of several symptoms, such as give-way weakness; motor symptoms such as paralysis, smoothness disorder of exercise, involuntary movements, and difficulty to sustain; abnormal sensations such as pain, abnormal perception of various parts, and impaired vibration sensation; deterioration of higher order functions such as memory and learning ability; and impairment of the visual processing system and various visual abnormalities. As patients with autoimmune encephalitis exhibit diffuse involvement, the presence of these symptoms was entirely understandable. Over three such abnormal findings could indicate diffuse brain damage. Owing to the regular understanding in neurology, most patients tend to be diagnosed with somatoform disorders. Thus, physicians should not diagnose somatoform disorders without first excluding autoimmune encephalopathy.
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PMID:[Motor Symptoms of Autoimmune Encephalopathies]. 2928 42

Hashimoto's encephalopathy (HE) is an uncommon neurological disorder of unknown etiology, found in association with thyroid autoimmunity, mostly uncommon in males. The disease occurs primarily in the fifth decade of life and may occur in two forms; a sudden vasculitic type or a progressive subacute type associated with cognitive dysfunction, confusion, and memory loss. We report a case of a 51-year-old Sri Lankan gentleman with no comorbidities who was presented with one episode of the generalized tonic- clonic seizure (GTCs) followed by prolonged agitation and disorientation. His EEG showed generalized slowing while CT scan and MRI brain were unremarkable. CSF examination showed high protein level with normal cell count and glucose. Routine serologic examination showed very high thyroid-stimulating hormone (TSH) level, with significantly high antithyroid antibodies. He was diagnosed as a case of Hashimoto's encephalopathy and treated with a high dose of steroid and showed remarkable improvement.
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PMID:Sudden-onset Encephalopathy: Do not ignore the Possibility of Hashimoto's Encephalopathy. 3264 88