scholarly journals Intractable Seizures and Limbic Encephalitis, Unaccounted Complications of Type 1 Diabetes Autoimmunity

Author(s):  
Mohammed A Madkhali ◽  
Jenifer-Kris Hao ◽  
Mohammad Saud Khan ◽  
Himani Sharma ◽  
Alexa Jaume ◽  
...  

Abstract Glutamic acid decarboxylase 65kD autoantibody (GAD65Ab) is frequently detected in patients with refractory epilepsy and stiff person syndrome (SPS). In contrast to T1D, the pathological role of GAD65Ab in neurological disorders is still debatable. As a result, the implementation of possible immunotherapy is usually delayed. This report presents two cases of GAD65Ab associated brain autoimmunity and their different management. We present clinical data and discuss management based on available evidence in the reviewed literature. Both cases presented with acute on chronic neurological symptoms and were GAD65Ab positive. Case 1, a 30-year-old man with a history of early-onset T1D at 14 months, followed by cryptogenic temporal epilepsy at 11 years of age, presented with intractable seizures. Case 2, a 48-year-old woman, presented with a history of recurrent severe headaches, cognitive impairment, decreased memory, and behavioral symptoms. GAD65Ab was detected in both patients’ sera. CSF GAD65Ab was only checked and positive in case 1. Case 2 was diagnosed with limbic encephalitis, treated with immunotherapy, and showed a remarkable clinical improvement. Case 1 with refractory epilepsy failed multiple AEDs and Responsive-Stimulator System (RNS) treatments. He was finally diagnosed with autoimmune epilepsy. The delay in diagnosis resulted in a lost opportunity for early immunotherapy. In conclusion, autoantibody screening and early initiation of immunotherapy should be considered to manage GAD65Ab associated neurological disorders.

Cephalalgia ◽  
1991 ◽  
Vol 11 (6) ◽  
pp. 281-284 ◽  
Author(s):  
L Septien ◽  
JL Pelletier ◽  
F Brunotte ◽  
M Giroud ◽  
R Dumas

The authors report the results of a retrospective controlled study on the incidence of migraine in centro-temporal epilepsy compared to absence epilepsy, partial epilepsy and a group of patients with cranial trauma without epilepsy. The following observations from this series of 129 patients were made. Migraine was present in 63% of the patients with centro-temporal epilepsy (rolandic epilepsy), in 33% with absence epilepsy, in 7% with partial epilepsy and in 9% of the cranial trauma group. These results suggest that the association of centro-temporal epilepsy and migraine is non-fortuitous and also to a lesser degree in absence epilepsy. The role of neurotransmitters in this association is discussed. No decrease in cerebral blood flow was observed in 12 patients with rolandic epilepsy on a Hm-PAO SPECT study.


2021 ◽  
Vol 22 (11) ◽  
pp. 5576
Author(s):  
Jana Amlerova ◽  
Jan Šroubek ◽  
Francesco Angelucci ◽  
Jakub Hort

Epilepsy as a chronic neurological disorder is characterized by recurrent, unprovoked epileptic seizures. In about half of the people who suffer from epilepsy, the root cause of the disorder is unknown. In the other cases, different factors can cause the onset of epilepsy. In recent years, the role of gut microbiota has been recognized in many neurological disorders, including epilepsy. These data are based on studies of the gut microbiota–brain axis, a relationship starting by a dysbiosis followed by an alteration of brain functions. Interestingly, epileptic patients may show signs of dysbiosis, therefore the normalization of the gut microbiota may lead to improvement of epilepsy and to greater efficacy of anticonvulsant drugs. In this descriptive review, we analyze the evidences for the role of gut microbiota in epilepsy and hypothesize a mechanism of action of these microorganisms in the pathogenesis and treatment of the disease. Human studies revealed an increased prevalence of Firmicutes in patients with refractory epilepsy. Exposure to various compounds can change microbiota composition, decreasing or exacerbating epileptic seizures. These include antibiotics, epileptic drugs, probiotics and ketogenic diet. Finally, we hypothesize that physical activity may play a role in epilepsy through the modulation of the gut microbiota.


2019 ◽  
Vol 2 (19) ◽  
pp. 19-23
Author(s):  
A. Savitskaya ◽  
E. G. Filatova

The article presents a review of the literature where, starting with the publication of Ekbom K. (1947), the history of the allocation of cluster headache into a separate form of primary head pain is described, modern diagnostic criteria are presented, the pathogenesis, clinical features of the disease are described. It is shown that due to the rarity of this type of headache and low awareness of doctors, the delay in diagnosis is on average 6–7 years. Patients are prescribed a lot of unnecessary examinations and expert consultations: oculists, ENT doctors, neurosurgeons, psychiatrists, etc. Favorite incorrect diagnoses are migraine and trigeminal neuralgia. The evidence of the leading role of the hypothalamus in the pathogenesis of cluster headache is presented. The principles of therapy are discussed: for the relief of acute attacks, triptans are used as in migraine, the leader of prophylactic therapy is a calcium channel blocker verapamil. In chronic forms and attacks resistant to pharmacological treatment, neurostimulation methods are used.


Author(s):  
Fusun Unal ◽  
Emine Atag ◽  
Leyla Telhan ◽  
Burcu Teber ◽  
Fazilet Karakoc ◽  
...  

Objectives: Our aim was to determine the treatable causes to increase the chance of decannulation success. For this purpose we evaluated the differences between the patients who succesfully decannulated and the patients who still has tracheostomy. Metods: A retrospective cohort study was conducted based on medical records of all pediatric patients with tracheostomy in a single centre. Results: Decannulation was successfully achieved in 59 patients (34.5%) of total 171 patients with tracheostomy between the years 2012-2019. Median duration of tracheostomy was 41.5 and 12 months in patients who remained with tracheostomy and decannulated respectively. Neurological disorders were higher in patients remained with tracheostomy, congenital heart disease and airway abnormalities were higher in decannulated patients. Presence of bacterial colonization (3.8-fold), history of invasive respiratory support following tracheostomy (2.9-fold), and having any neurological disorder and/or comorbidity (5.2-fold) were significantly associated lower rates of decannulation. Almost 33 % of patients had bacterial colonization and colonization rates were higher in patients who needed invasive respiratory support following tracheostomy placement (p<0.001), patients with feeding/swallowing problems (p=0.005) and neurological disorders(0.002). There was significant correlation between duration of tracheostomy and bacterial colonization rates (p=0.008). But after analysing with logistic regression only having a neurological disorder was associated with bacterial colonization (OR= 2.9; 95% Cl: 1.15-7.47 p=0.024). Conclusion: While conducting decanulation assessment, the presence of colonization should be considered. Future prospective researchs are necessary in order to determine the role of chronic colonization on decannulation success.


2021 ◽  
Vol 3 (3) ◽  
pp. 52-54
Author(s):  
Mamdouh H. Kalakatawi ◽  
Nusrat Aziz ◽  
Norhan A. K. Mostafa

Stiffperson syndrome (SPS) is one of the rarest neurological disorders. It is an autoimmune disease with antibodies against glutamic acid decarboxylase - enzyme needed to produce neurotransmitter GABA. This disease is very debilitating and fatal if not managed in time. A 32-year-old man presented with stiffness of abdominal and thoracolumbar paraspinal muscles first noticed 10 months back. There was history of jerkiness of hands. After excluding other mimicking conditions like multiple sclerosis, parkinsonism and axial dystonia, a diagnosis of Stiffperson syndrome was made. Treatment with IVIG therapy along with anti-spasticity drugs resulted in improved patient outcome. SPS is potentially treatable though cannot be cured. Not one treatment fits all patients with SPS and may have to be tailored according to the clinical manifestations. Increasing awareness in medical doctors and identifying the disease early can help the patients escape the disabling effects of SPS and improve the quality of life.


2021 ◽  
Vol 12 ◽  
Author(s):  
Changhong Ren ◽  
Haitao Ren ◽  
Xiaotun Ren ◽  
Weihua Zhang ◽  
Jiuwei Li ◽  
...  

Background: Antibodies against glutamic acid decarboxylase (GAD) are associated with various neurologic conditions described in patients, including stiff person syndrome, cerebellar ataxia, refractory epilepsy, and limbic and extralimbic encephalitis. There have been some case reports and investigations regarding anti-GAD65 antibody-associated encephalitis in adult populations, but pediatric cases are rare. We retrospectively analyzed the clinical data of three anti-GAD65 antibody-positive patients to explore the diversity and clinical features of anti-GAD65 antibody-associated pediatric autoimmune encephalitis.Methods: The clinical data of a series of three patients positive for anti-GAD65 antibody were retrospectively analyzed. GAD65 antibodies were determined in serum and CSF using a cell-based assay.Results: All three patients were female, and the onset ages were 4 years and 9 months, 6 years, and 16 years old. Their clinical phenotypes included autoimmune limbic encephalitis, extralimbic encephalitis, and encephalitis combining limbic and extralimbic encephalitis. The clinical symptoms included seizures, memory deficits, drowsiness, dysautonomia, and headache. All patients had abnormal carinal MRI and EEG. All patients received immunotherapy and had transiently good responsiveness, but one patient then experienced relapse. In follow-up, one patient with extralimbic encephalitis recovered completely, while two patients with limbic involvement had poor outcomes with refractory focal epilepsy.Conclusion: In addition to limbic encephalitis, extralimbic encephalitis is also an important phenotype in patients who are positive for anti-GAD65 antibodies. Early diagnosis and immunotherapy can improve the symptoms. However, patients with limbic encephalitis often have refractory epilepsy in the chronic phase and have a poor long-term outcome.


2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Shuping Chen ◽  
Shubin Wang ◽  
Peijing Rong ◽  
Junling Liu ◽  
Hongqi Zhang ◽  
...  

Neurostimulation procedures like vagus nerve stimulation (VNS) and deep brain stimulation have been used to treat refractory epilepsy and other neurological disorders. While holding promise, they are invasive interventions with serious complications and adverse effects. Moreover, their efficacies are modest with less seizure free. Acupuncture is a simple, safe, and effective traditional healing modality for a wide range of diseases including pain and epilepsy. Thalamus takes critical role in sensory transmission and is highly involved in epilepsy genesis particularly the absence epilepsy. Considering thalamus serves as a convergent structure for both acupuncture and VNS and the thalamic neuronal activities can be modulated by acupuncture, we propose that acupuncture could be a promising therapy or at least a screening tool to select suitable candidates for those invasive modalities in the management of refractory epilepsy.


2019 ◽  
Vol 7 ◽  
pp. 2050313X1985005
Author(s):  
Elysia Tjong ◽  
Rachael Gardner ◽  
Yen-Yi Peng

We report a case of a 60-year-old woman with a history of intractable seizures and isolated delusional psychosis who was later diagnosed with steroid-responsive encephalopathy associated with autoimmune thyroiditis. The patient underwent right temporal lobectomy (epilepsy surgery) 15 years before coming to this clinic, but continued to have focal seizures, resulting in frequent emergency room visits thereafter. After admission for intensive inpatient video electroencephalogram monitoring and subsequent 7 months of close follow-up, both the electroencephalogram abnormalities and isolated delusional psychosis were found to be responsive to immunotherapy. This suggests that her epilepsy may be autoimmune in nature. Steroid-responsive encephalopathy associated with autoimmune thyroiditis was diagnosed after 26 years since the onset of seizures. Performing invasive epilepsy surgery in patients with autoimmune epilepsy cannot reverse the inflammatory process; therefore, it is reasonable to test for autoimmune etiologies before excision surgery on patients with medically intractable epilepsy. This case demonstrates the clinical use of quantitative electroencephalogram in assisting with the diagnosis of steroid-responsive encephalopathy associated with autoimmune thyroiditis and supports that it is a spectrum disorder with protean manifestations.


2018 ◽  
Vol 41 ◽  
Author(s):  
Kevin Arceneaux

AbstractIntuitions guide decision-making, and looking to the evolutionary history of humans illuminates why some behavioral responses are more intuitive than others. Yet a place remains for cognitive processes to second-guess intuitive responses – that is, to be reflective – and individual differences abound in automatic, intuitive processing as well.


2001 ◽  
Vol 120 (5) ◽  
pp. A442-A442
Author(s):  
P TSIBOURIS ◽  
M HENDRICKSE ◽  
P ISAACS

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