Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dementias which are either reversible or avoidable are discussed in the light of the literature. The frequency is between 6 and 32%. The most important etiological groups are immunological vasculopathies, hyperlipidemia, some types of encephalitis and, mainly, progressive dementia of the insane, benign tumors and in particular meningioma, low pressure hydrocephalus, intoxications due to drugs, industrial products and alcohol, metabolic disturbances, encephalopathy in dialysed patients, ileo-jejunal-bypass encephalopathy and encephalopathy due to neoplasms. Dementias are also seen in endocrinological disturbances and particularly in hypothyroidism. Vitamin B12 and folate deficiency, as well as epilepsy, may be causes of dementia. Depression may mimic a state of dementia. Some features of reversible dementias are listed, including in particular the somewhat more rapid onset, the younger age of patients, and accompanying neurological symptoms such as headache, gait disturbances, ataxia, polyneuropathy, myoclonus or epileptic fits.
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PMID:[Reversible and preventable dementias]. 361 87

A case of 43-year-old woman with Hashimoto's encephalopathy who experienced three relapses closely associated with the menstrual cycle is reported. In April 1992, she began to experience occasional tremors in her arms. Three months later, she experienced a generalized seizure and was transferred to our hospital. Hashimoto's thyroiditis was diagnosed on the basis of high thyroid microsomal titer and mild hypothyroidism. Neurological findings in admission included action tremor in both hands, myoclonus in all extremities, cerebellar ataxia, confusion, and hyperreflexia. Cerebrospinal fluid showed elevated protein level without pleocytosis. Electroencephalogram showed diffuse slowing and magnetic resonance imaging of brain was normal. Hashimoto's encephalopathy was diagnosed from these findings. These episodes of remission and exacerbation were observed during the admission. Her symptoms started at ovulation, worsened during the luteal phase, and improved when menstruation started. After the third relapse, she was treated with oral thyroxine for hypothyroidism and with an estrogen and progesterone combination to regulate the menstrual cycle. Her thyroid function gradually became euthyroid and she did not experience any subsequent relapses. The relation between the relapsing course and menstrual cycle suggests that the periodic alteration of gonadotrophic and/or gonadal hormones or the menstrual regulating center itself in the brain may be an important factor of pathogenetic mechanism of the disorder.
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PMID:[A case of Hashimoto's encephalopathy with a relapsing course related to menstrual cycle]. 829 82

A 25-year-old woman presented with a subacute confusional state, headaches, unsteadiness, myoclonus, seizures, and an amnesic syndrome as a manifestation of Hashimoto's encephalopathy. Investigations showed biochemical hypothyroidism, raised thyroid microsomal antibodies, and weakly positive antineuronal antibodies. A T2-weighted MRI of the brain showed bilateral symmetric areas of increased signal in the mesial temporal lobes and hippocampi that had a low signal intensity on T1-weighted imaging. Despite clinical and radiologic improvement after steroid and thyroid hormone replacement therapy, a severe amnesic syndrome with associated localized MRI abnormalities persists.
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PMID:Amnesic syndrome with bilateral mesial temporal lobe involvement in Hashimoto's encephalopathy. 1068 Aug 14

A 71-year-old woman with Hashimoto's disease was admitted to our hospital because of involuntary movement, gait disturbance, and mental decline. Her consciousness was alert but her orientation about time and place was disturbed. She was mentally ill (HDS-R; 12/30, MMSE; 14/30), and could not walk because of truncal ataxia. Myoclonus was present in the upper extremities. Laboratory examinations showed hypothyroidism and very high titers of antithyroid antibodies (ATA) in serum. Head MRI showed no abnormal lesions. On electroencephalogram (EEG), the basic rhythm was slow and bursts of irregular slow waves (4-6 Hz) were present. Her conditions gradually ameliorated so that she was discharged. However, she was hospitalized again because of sudden worsening of the illness: her consciousness got disturbed and the myoclonus became marked. As the result, she got bed-ridden. At the time, thyroid function was almost normal, suggesting that the deterioration could not be attributed to hypothyroidism. The EEG findings were quite different from the former: complex of multiple spikes and slow waves was continuously present. Examination of the cerebrospinal fluid (CSF) revealed an elevated level of protein and IgG (cell 1/m3, protein 101 mg/dl, sugar 60 mg/dl ,Cl 124 mEq/l, IgG 20.4 mg/dl). IgG index was 0.57 and Q albumin (CSF-albumin/serum-albumin ratio) was 15.2 (9.0>) . After the second admission, she recovered from the bed-ridden state but was still unable to walk or communicate. She continued to need complete support for all daily lives. The diagnosis was made as Hashimoto's encephalopathy (HE), from the following points: 1) encephalopathy not due to hypothyroidism, 2) very high titers of ATA, 3) elevated CSF protein. The effectiveness of steroid therapy was so amazing that the neurological problems faded away very soon. Finally she completely recovered. As well as the clinical manifestations, the EEG findings were improved. At the stage in which excellent clinical improvement had achieved, head MRI revealed that T2/FLAIR high lesions without Gd-enhancement temporarily appeared diffusely and extensively in the cerebral white matter. They thereafter almost disappeared on follow-up MRI. Judging from the lesions being non-enhanced and reversible, they supposedly reflected brain edema. It is important to keep it in mind that MRI findings may change abruptly and drastically in HE. ATA was detected not only in serum but also in CSF. Either titer thereafter decreased along with the healing of the illness. Concerning the reason why ATA is present in CSF, we supposed leakage from blood through the disrupted blood-brain barrier, for the following reasons: 1) IgG index was normal, 2) Q albumin was elevated, 3) the change of CSF-ATA titer was almost parallel to that of serum-ATA titer, and 4) the white matter lesions, which presumably reflected brain edema, were observed.
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PMID:[Reversible white matter lesions and antithyroid antibodies in the cerebrospinal fluid in Hashimoto's encephalopathy: a case report]. 1538 4

Hashimoto's encephalopathy (H.E.) is probably of autoimmune etiology, and manifests with seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms, myoclonus. It is presumed to be autoimmune in origin with high serum titers of antithyroid peroxidase antibodies (anti-TPA). Thyroid function might often be normal. The diagnosis is arrived at by excluding other toxic, metabolic and infectious causes of encephalopathies, supportive clinical profile, elevated thyroid antibodies and optimum steroid response. We present the characteristic phenotypic manifestations, magnetic resonance imaging and electroechography observations and response to immunomodulation with follow-up in three cases of H.E. All the three cases manifested with subacute to chronic progressive encephalopathy, cerebellar dysfunction, seizures, behavioral abnormalities and oculomotor disturbances and had evidence of hypothyroidism, elevated titers of anti-TPA and positive thyroid anti-microsomal antibodies. Atypical and uncommon presentations are known. This report emphasizes that a high index of suspicion is often required in cases with "investigation negative encephalopathy" for early diagnosis of H.E.
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PMID:Steroid-responsive encephalopathy in autoimmune thyroiditis: Clinical spectrum and MRI observations in three cases. 2202 37

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.
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PMID:Hashimoto encephalopathy presenting as progressive myoclonus epilepsy syndrome. 2284 Feb 75

Neurological disorders associated with glutamic acid decarboxylase (GAD) antibodies are rare pleomorphic diseases of uncertain cause, of which stiff-person syndrome (SPS) is the best-known. Here, we described nine consecutive cases of neurological disorders associated with anti-GAD, including nine patients with SPS and three cases with cerebellar ataxia. Additionally, four had hypothyroidism, three epilepsy, two diabetes mellitus and two axial myoclonus.
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PMID:Neurological disorders associated with glutamic acid decarboxylase antibodies: a Brazilian series. 2299 Jul 19

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.
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PMID:Clinical manifestations and treatment response of steroid in pediatric Hashimoto encephalopathy. 2405 54

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

Statins and hypothyroidism, independently, can rarely cause rhabdomyolysis. The combination of them especially with concurrent intake of drugs such as diltiazem increases the risk of rhabdomyolysis. Hashimoto's encephalopathy is a rare condition associated with Hashimoto's thyroiditis and some patients with that can present with a stroke like picture. An elderly male who has been on atorvastatin for three years and on diltiazem for a week presented with sudden onset inability to walk and confusion. On examination muscle tenderness was noticed and creatine kinase levels indicated rhabdomyolysis which we attributed to atorvastatin. Patient developed a seizure and myoclonus of masseters. Considering this, his confusion and his neutrophilia and high C-reactive protein levels, empirical antibiotics with dexamethasone were started and the patient responded to that. His cerebrospinal fluid and blood culture reports that arrived later did not show sepsis. After going home also his CK (creatine kinase) levels remained high; TSH (thyroid-stimulating hormone) level test was done and hypothyroidism was diagnosed. His antithyroid peroxidase antibody levels were also very high. We retrospectively think he had Hashimoto's encephalopathy as well. His lipid profile and TSH and CK values returned to normal in that order after a few months of levothyroxine therapy.
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PMID:Atorvastatin-Diltiazem Combination Induced Rhabdomyolysis Leading to Diagnosis of Hypothyroidism. 2848 44


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