Hashimoto encephalopathy causing drug-resistant status epilepticus treated with plasmapheresis

Hashimoto encephalopathy is a rare, clinically heterogenous condition. Its treatment is based on corticosteroids. A previously normal 12-year-old boy was admitted to our pediatric emergency department with status epilepticus. He experienced a recurrence of status epilepticus after pentobarbital withdrawal, and required repeated resumptions of drug-induced coma. He manifested acute personality changes. His limbic encephalitis markers were normal, but his level of anti-thyroid peroxidase antibody was high. A diagnosis of Hashimoto encephalopathy was considered. Our patient responded to plasmapheresis instead of corticosteroid treatment. This case report is the first, to the best of our knowledge, of plasmapheresis because of Hashimoto encephalopathy in a child.     

A case of 18 q-syndrome associated with status epilepticus

A 15-year-old boy with 18 q-syndrome manifesting a status epilepticus is reported. He has been already diagnosed as epilepsy because of grand mal seizures at six months earlier, and abnormal EEG findings. Unilateral status epilepticus developed at 15 years of age, which were characterized by alternative repetition of horizontal nystagmus to the right and clonic convulsion of the right (mainly upper) extremities every several minutes. Ictal EEG showed continuous 2 Hz high voltage slow waves superimposed by spikes and polyspikes which transformed to localized, irregular spike discharges in the left occipital region at the end of the status. The chromosomal study revealed a partial deletion of the long arm of No. 18. He had severe mental retardation, and a typical karyotype for 18 q-syndrome with reduced prominence of the midface region, short stature and whorls on all finger tips. The immaturity of the brain probably relates to this kind of unilateral status epilepticus.     

Cortico-thalamic activation in generalized status epilepticus, a PET study

In a patient with a refractory generalized convulsive status epilepticus, the ictal distribution of regional cerebral glucose was assessed with positron emission tomography (PET). Synchronized seizure activity in the EEG was associated with bilateral metabolic activation of medial sensorimotor regions, anterior cingulate cortex, striatum and thalamus. This pattern with focal cortical activation supports the concept that a cortical focus may drive epilepsy, while the thalamus mediates synchronization of neuronal activity as reflected in the EEG.     

以癫痫持续状态起病的桥本脑病1例分析

目的 探讨以癫痫持续状态为首发表现的桥本脑病的临床特征。方法 回顾性分析1例以癫痫持续状态为首发表现的桥本脑病患者的临床资料。结果 临床呈癫痫持续状态,入院时神志不清,间断抽搐伴瞳孔散大,间歇期仍昏迷,余无明显局灶定位体征; 血气pH值6.807,乳酸24 mmol/L,头颅CT、MRI检查均未见异常,2次腰穿脑脊液常规、生化均正常,自身免疫性脑炎抗体8项均阴性,甲状腺功能检测提示轻度甲减,抗甲状腺球蛋白抗体和抗甲状腺过氧化物酶抗体升高,诊断桥本脑病,给予丙种球蛋白及甲强龙冲击治疗后病情迅速并明显好转,用药次日患者神志转清楚,癫痫持续状态得到有效控制。结论 桥本脑病临床以癫痫持续状态为首发者少见,故遇到不明原因的脑病患者,需注意甲状腺功能及相关抗体检查,以尽快明确诊断,尽早治疗,最大程度地改善其预后。     

A case of Hashimoto's encephalopathy: association with sensory ganglionopathy

We report a case of Hashimoto's encephalopathy with prominent unsteadiness of gait. Nerve conduction studies and electromyographic findings were consistent with a sensory ganglionopathy. Symptoms and clinical findings resolved with high-dose corticosteroid therapy and paralleled the levels of anti-microsomal antibodies. Sensory ganglionopathy seems to be another aspect of the broad clinical syndrome labeled Hashimoto's encephalopathy.     

A case of Hashimoto's encephalopathy associated with Graves' disease]

A 23-year-old woman with Graves' disease was first admitted to a hospital because of generalized convulsion, consciousness disturbance, and tachycardia. Investigations showed biochemical hyperthyroidism and positive thyroid binding inhibitory immunoglobulin (TBII). She was treated with anticonvulsant and antithyroid therapy. One month later the patient developed the second episode of generalized convulsion, followed by cognitive deficits especially concerning short-term memory, when she was transferred to our hospital. Although thyroid function was normalized at that time, anti-thyroid peroxidase (TPO) antibodies level were elevated to 5,850 U/ml in the serum, and 4.9 IU/ml in CSF. CSF protein was elevated to 133 mg/dl. Brain SPECT showed global decrease of cerebral perfusion, and EEG demonstrated diffuse slowing of the background rhythm without any signs of epileptic activity, although brain MRI was normal. Based on a diagnosis of Hashimoto's encephalopathy associated with Graves' disease, therapy with steroids was started, which definitely improved her clinical symptoms. Anti-TPO antibodies disappeared in CSF and decreased in the serum after the treatment. Hashimoto's encephalopathy should always be kept in mind in patients with Hashimoto's disease and Graves' disease.     

Changes in brain glucose metabolism and connectivity in somatoform disorders: an 18F-FDG PET study

European Archives of Psychiatry and Clinical Neuroscience - Somatoform disorders (SFD) are defined as a syndrome characterized by somatic symptoms which cannot be explained by organic reasons....     

A case of status epilepticus with psychomotor seizures

The authors describe in the light of an own case the clinical manifestations and electroencephalographic findings in status epilepticus with psychomotor seizures.     

Idiopathic late-onset absence status epilepticus: A case report with an electroclinical 14 years follow-up

Late-onset absence status epilepticus (ASE) may be observed in adult and elderly patients as a late complication of idiopathic generalized epilepsy or de novo, usually related to benzodiazepines withdrawal, alcohol intoxication or psychotropic drugs initiation, but without history of epilepsy. EEG may be highly heterogeneous, varying from the 3 to 3.5 Hz spike-wave discharges typical of idiopathic generalized epilepsy to asymmetric irregular sharp and slow wave complexes. We report the clinical and neurophysiologic 14 years follow-up of a now 86 years-old woman, in whom we observed – at the age of 72 – an idiopathic late-onset ASE, with a good clinical response to lamotrigine monotherapy, but with the persistence over years of the same interictal 3–3.5 Hz spike-wave epileptic activity at EEG. This case is singular because, with the available long follow-up, indicates that idiopathic generalized epilepsy may also occur in the elderly, with a late-onset ASE presentation. In this condition, it is particularly important to underline the essential role of EEG (urgent and ambulatory) for the diagnosis, management and monitoring of the disease.     

A case of acute thallotoxicosis successfully treated with double-filtration plasmapheresis

The authors report a 48-year-old Chinese woman who presented with acute peripheral neuritis with progressive alopecia. Laboratory examinations disclosed a high blood concentration of thallium (97 microg/L) versus a normal value (0.9 microg/L), and she was diagnosed as having acute thallotoxicosis. After her hospitalization, the cutantest of dimercaptopropansulfonate sodium was positive and the patient refused to take Prussian blue because it caused constipation. She rapidly entered remission after assistance via double-filtration plasmapheresis (DFPP), suggesting the potential efficacy of DFPP for thallotoxicosis.     

Metabolic brain network in the Chinese patients with Parkinson's disease based on 18F-FDG PET imaging

The objective of this study was to validate Parkinson's disease-related pattern (PDRP) as a measure of network biomarker of Parkinson's disease (PD) in Chinese population by using ¹⁸F-fluorodeoxyglucose (FDG) and Positron Emission Tomography (PET). Resting-state brain FDG PET imaging was performed in a cohort of 33 PD patients and 33 age/gender-matched healthy controls to identify a PDRP. PDRP expression was then computed in a new cohort of 30 PD patients and 30 healthy controls using a voxel-based network quantification algorithm. Differences in PDRP expression were compared across groups and correlations with severities of PD were investigated. As a result, we identified a PDRP characterized by relative increases in pallidothalamic, pontine, and cerebellar metabolism, associated with concurrent metabolic decreases in the premotor and posterior parietal areas. PDRP expression in each of the two PD groups was significantly elevated relative to that of the healthy controls (P < 0.001). Receiver operating characteristic (ROC) analysis revealed that the PDRP-based discrimination for PD patients and controls had high sensitivity and specificity (both = 93.9%) in the derivation cohort, which declined slightly in the validation cohort (both = 90.0%) at the same diagnostic threshold. Moreover, PDRP scores correlated positively with Hoehn and Yahr scores (r ≥ 0.590, P ≤ 0.001) and Unified Parkinson's Disease Rating Scale motor scores (r ≥ 0.646, P < 0.001) in both patient groups. In conclusion, PDRP is highly reproducible in Chinese cohorts based on FDG PET imaging. Network activity of PDRP can differentiate PD patients from healthy controls and correlates with the severities of the disease.     

Generalized tonic-clonic status epilepticus in a child with cat-scratch disease and encephalopathy.

Cat-scratch disease occasionally can be associated with encephalopathy and present as status epilepticus. However, the majority of these patients recover without sequelae, as shown by our patient and previous reports. Cat-scratch disease with encephalopathy should be considered in the differential diagnosis of status epilepticus in children.     

Increased brain metabolism after acute administration of the synthetic cannabinoid HU210: a small animal PET imaging study with 18F-FDG

Cannabis use has been shown to alter brain metabolism in both rat models and humans although the observations between both species are conflicting. In the present study, we examined the short term effects of a single-dose injection of the synthetic cannabinoid agonist HU210 on glucose metabolism in the rat brain using small animal (18)F-2-fluoro-deoxyglucose (FDG) Positron Emission Tomography (PET) 15 min (Day 1) and 24h (Day 2) post-injection of the agonist in the same animal. Young adult male Wistar rats received an intra-peritoneal injection of HU210 (100 μg/kg, n=7) or vehicle (n=5) on Day 1. Approximately 1mCi of (18)F-FDG was injected intravenously into each animal at 15 min (Day 1) and 24h (Day 2) post-injection of HU210. A 5-min Computer Tomography (CT) scan followed by a 20-min PET scan was performed 40 min after each (18)F-FDG injection. Standardised Uptake Values (SUVs) were calculated for 10 brain regions of interest (ROIs). Global increased SUVs in the whole brain, hence global brain metabolism, were observed following HU210 treatment on Day 1 compared to the controls (21%, P<0.0001), but not in individual brain regions. On Day 2, however, no statistically significant differences were observed between the treated and control groups. At the 24h time point (Day 2), SUVs in the HU210 treated group returned to control levels (21-30% decrease compared to Day 1), in all ROIs investigated (P<0.0001). In the control group, SUVs did not differ between the two acquisition days in all brain regions. The present results suggest that high-dose HU210 increases brain glucose metabolism in the rat brain shortly after administration, in line with normalised human in vivo studies, an effect that was no longer apparent 24 h later.     

Nonconvulsive status epilepticus causing prolonged stupor after intraventricular hemorrhage: report of a case.

We describe the case of an octogenarian woman who experienced a severe alteration of mental state due to non-convulsive status epilepticus (NCSE) complicating an intraventricular hemorrhage. Our report emphasizes that NCSE may be the cause of unexplained neurological deterioration in elderly patients with acute brain injury.     

A methodology for assessing treatment response in Hashimoto's encephalopathy: a case study demonstrating repeated computerized neuropsychological testing

Hashimoto's encephalopathy, a steroid-responsive encephalopathy, presents with cognitive deterioration, psychiatric symptoms, and seizures and is often associated with elevation of anti-thyroid peroxidase antibodies or thyroid dysfunction. Because of the absence of a gold-standard diagnostic test and serological marker of disease, improvement in cognitive impairment is an important sign when assessing response to corticosteroid treatment. The purpose of this case study is to present a methodology for tracking neurocognitive functioning using computerized testing (CNS Vital Signs) in a 14-year-old girl with Hashimoto's encephalopathy. Computerized neuropsychological testing was done prior to commencing corticosteroid treatment, as well as at 7 follow-up assessments over 1 year. Improvement in cognitive, psychological, and behavioral functioning heralded a response to treatment, which was sustained during tapering of medication. Computerized neuropsychological testing seems to be a viable tool for tracking cognitive functioning (when rapid assessments are desired) and assessing response to corticosteroid treatment in Hashimoto's encephalopathy.     

Hashimoto's encephalopathy with selective involvement of the nucleus accumbens: a case report

Hashimoto's encephalopathy (HE) is an acute or subacute relapsing disorder usually affecting euthyroid patients with evidence of autoimmune thyroiditis. The neurological manifestations are non-specific, with subacute cognitive impairment, movement disorders, generalized seizures, focal neurological symptoms such as stroke-like episodes, or psychiatric disturbances. Autoimmune phenomena are likely to play an etiological role. Magnetic resonance imaging (MRI) findings are usually normal or show non-specific changes. We report the case of an 11-year-old girl with autoimmune thyroiditis who presented acutely with a complex neuropsychiatric disorder in association with MRI evidence of focal involvement of the nucleus accumbens (NA). The NA, a ventral striate nucleus, is part of a complex dopaminergic network. Lesions to the NA result in several psychiatric symptoms, such as attention-deficit hyperactivity disorders. In this patient, we observed alternating phases of stupor and hyperkinetic-anxious behavior, with marked instability. The pathogenetic mechanism and the anatomic and functional correlations are briefly discussed.