Subacute Cognitive Decline Research Articles

Clinical Features and Electroencephalogram Analysis ofBrain Network Functional Connectivityin Anti-Leucine-RichGlioma-Inactivated1Antibody Encephalitis.

To summarize the clinical manifestations, laboratory findings, and magnetic resonance imaging (MRI) characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis (anti-LGI1 antibody encephalitis) and explore the electroencephalogram (EEG) features. We retrospectively analyzed the medical history of 16 patients diagnosed with anti-LGI1 antibody encephalitis at the First Hospital of Hebei Medical University from 2021 to 2023. EEGs of patients with anti-LGI1 antibody encephalitis and healthy individuals were analyzed. Based on Video-EEG signal analysis of EEG δ, θ, α, β frequency bands, weighted phase lag index values were calculated to form brain network matrices, studying differences in coherence between brain regions of patients with anti-LGI1 antibody encephalitis and healthy individuals. Patients with anti-LGI1 antibody encephalitis often presented with subacute onset seizures and cognitive decline. Routine test of cerebrospinal fluid was mostly normal. Serum testing revealed hyponatremia in 62.50% of patients, along with positive serum antinuclear antibodies, decreased vitamin B12, and abnormal cytokines such as interleukin-6. Head MRI revealed abnormal lesions related to the disease in seven cases (43.75%), mainly located in the unilateral or bilateral frontal and temporal lobes of the hippocampus. The EEG mainly showed generalized and focal slow waves, sometimes with focal discharges. Brain network functional connectivity analysis found a significant weakening of functional connections in the frontal-temporal lobe in the δ and β frequency bands. Intravenous pulse corticosteroids and intravenous immunoglobulin are first-line immunotherapies for anti-LGI1 antibody-related encephalitis. The disease recovery and cognitive decline improved in most patients. Anti-LGI1 antibody encephalitis is characterized by seizures and cognitive dysfunction. Serum may show abnormalities in immune indicators such as cytokines. Head MRI mainly reveals abnormal signals in the frontal-temporal lobes and the hippocampus. EEG brain network connectivity analysis reveals characteristic weakening of functional connections in the frontal-temporal lobe in the δ and β frequency bands.

Relapsing meningitis and limbic encephalitis in anti-AQP4-Ab-associated neuromyelitis optica spectrum disorder.

neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease mainly affecting optic nerves and the spinal cord. Due to the potentially irreversible tissue damage, prevention of relapses is of utmost importance. We describe the atypical clinical course and pathology results of a patient with anti-aquaporin-4 antibody (anti-AQP4-Ab)-associated NMOSD who developed aseptic meningitis followed by limbic-encephalitis-like presentation with extensive brain lesions upon treatment with rituximab and tocilizumab. The patient developed subacute cognitive decline with magnetic resonance imaging (MRI) evidence of extensive brain white matter lesions. In the hypothesis of an opportunistic brain infection, she underwent brain biopsy of the temporal pole. Pathology results revealed typical NMOSD findings with complement activation, supporting the hypothesis of an atypical presentation of anti-AQP-Ab-associated NMOSD. Accordingly, treatment with the complement-targeting drug eculizumab was started, leading to a dramatic clinical and MRI improvement. aseptic meningitis and limbic encephalitis could represent a rare phenotype of anti-AQP4-Ab-associated NMOSD.

Kleine Levin syndrome - presentation of five cases.

Kleine Levin syndrome (KLS) is a rare entity. It presents with subacute onset episodic hypersomnia, cognitive decline, altered perception, and occasional hyperphagia and hypersexuality with full recovery during the interepisodic period. Five cases presented with episodic hypersomnia and met the diagnostic criteria of KLS. The majority of the cases were females (3/5) in whom KLS is even rarer. The initial presentation was in the age range of 10-25 years. Cognitive dysfunction (5/5), derealization (4/5), viral prodrome (3/5), hyperphagia (3/5), and hypersexuality (2/5) were other clinical presentations. A differential diagnosis of atypical depression is the major challenge, and thorough history taking would help in differentiation. Treatment with stimulants (modafinil) and mood stabilizers (lithium) proved effective. A high degree of suspicion should be kept for cases of episodic hypersomnolence for early diagnosis and management of KLS.

Cognitive decline as the main manifestation of diabetic striatal disease but without involuntary movements: a case report

Diabetic striatopathy (DS) is a rare central nervous system complication of diabetes mellitus, characterized mainly by non-ketotic hyperglycemia and lateralized involuntary movements. Patients with diabetic striatopathy manifested solely by subacute cognitive decline were rarely reported. In this paper, we report a patient with DS who presented solely with subacute cognitive decline without involuntary movements, and cranial CT showed bilateral high density in the basal ganglia. In contrast, SWI showed microhemorrhages in the right caudate nucleus head. After one week of treatment, including glycemic control, the patient showed significant improvement in cognitive function, while a repeat cranial CT showed improved hyperdensity in the right basal ganglia region. 1 month later, at telephone follow-up, the patient’s symptoms did not recur.

Dramatic Improvement of MRI Abnormalities of HIV Dementia after Suppressive Antiretroviral Therapy (S25.002)

<h3>Objective:</h3> Not applicable. <h3>Background:</h3> A 59-year-old man diagnosed with HIV 26 years prior and a long history of antiretroviral therapy (ART) non-adherence presented with subacute cognitive decline. A neurologic workup including cerebrospinal fluid testing showed no evidence of opportunistic infections. He was admitted to an assisted care facility where he restarted ART. However, he remained viremic and was referred to the National Institutes of Health (NIH). On admission to the NIH, he had an HIV plasma viral load of 28,056 copies/mL and CD4 T-cell count of 5 cells/μL. He was started on a new ART regimen with increased central nervous system penetration (etravirine, dolutegravir, darunavir/cobicistat, tenofovir/emtricitabine). MRI revealed extensive leukoencephalopathy and volume loss, consistent with HIV encephalopathy. Comprehensive neuropsychological testing revealed significant deficits in all cognitive domains, including learning, memory, attention, executive function, and psychomotor speed. The patient was followed for two years, during which time he achieved and sustained viral suppression (&lt;40 copies/mL) and reached a CD4 T-cell count of 197 with ART. A repeat MRI showed profound improvement in the white matter signal abnormality. Repeat neuropsychological testing demonstrated notable improvements in several tests of attention, information processing, and psychomotor speed, but continued difficulty in most other cognitive domains. <h3>Design/Methods:</h3> Not applicable. <h3>Results:</h3> Not applicable. <h3>Conclusions:</h3> While current ART regimens are highly effective in reducing the incidence of HIV-associated neurologic complications, their role in reversing the MRI abnormalities seen in HIV encephalopathy remains unclear. In this case, we tracked HIV encephalopathy in the current era of effective ART with serial MRI evaluations. Our results provide a rare example of dramatic reduction of periventricular white matter hyperintensities and improved performance in a subset of cognitive domains. Providers encountering white matter abnormalities in patients with treated HIV should consider this legacy effect in evaluating whether disease is progressive, or as in this case, improved. <b>Disclosure:</b> Ms. Dietrich has nothing to disclose. Miss Laidlaw has nothing to disclose. Dr. Hammoud has nothing to disclose. Dr. Pau has nothing to disclose. Dr. Smith has nothing to disclose.

Cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 positivity: A case report of a treatment dilemma and a possible relationship (P1-12.003)

<h3>Objective:</h3> To examine the treatment dilemma utilizing high-dose steroids in a patient with cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 positivity. Additionally, to question a possible relationship between HHV-6 and CAARI. <h3>Background:</h3> Cerebral amyloid angiopathy-related inflammation (CAARI) has been described as a distinct syndrome with subacute cognitive decline or seizure symptoms. Previous studies have established diagnostic criteria for this entity and proposed a treatment strategy. However, the precipitating factors behind developing CAARI in a subset of patients with CAA are unclear. We present a case of CAARI in a patient with no underlying diagnosis of dementia that was found to be positive for HHV-6 in serum and CSF and treated successfully with steroids. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> An 83-year-old woman presented with a first-time generalized tonic-clonic seizure with postictal confusion. On presentation, her systolic blood pressure was elevated at 150–170, and she was tachycardic to 160 with a temperature of 38.4. A contrasted MRI brain showed multifocal vasogenic edema, sulcal FLAIR hyperintensity, and innumerable lobar microhemorrhages. Black blood contrast MRI brain angiogram was unremarkable, with no evidence of vasculitis. At this point, the patient had clinically improved but remained slightly encephalopathic with word-finding difficulties. Continuous EEG monitoring showed diffuse background slowing. Initial CSF studies revealed an isolated mildly elevated protein of 49 and negative cytology. Therefore, based on the clinical and radiographic evidence, a decision was made to undergo five days of high-dose methylprednisolone for probable CAARI. However, days later, HHV-6 in CSF resulted positive with 249 copies/ml, and a serum sample showed 1100 copies/ml. <h3>Conclusions:</h3> The concurrence of cerebral amyloid angiopathy-related inflammation (CAARI) and HHV-6 in serum and CSF might yield a diagnostic and therapeutic challenge. However, the treatment with high-dose pulsatile steroids with taper did improve the overall clinical picture. Further studies are needed to investigate a potential correlation. <b>Disclosure:</b> Dr. Alshawa has nothing to disclose. Dr. Makkieh has nothing to disclose. Miss Alsakka has nothing to disclose. Dr. Torres-Melendez has nothing to disclose.

Fatal thrombolysis-related intracerebral haemorrhage associated with amyloid-β-related angiitis in a middle-aged patient – case report and literature review

BackgroundAmyloid-β-related angiitis (ABRA) is a rare complication of cerebral amyloid angiopathy, characterized by amyloid-β deposition in the leptomeningeal and cortical vessels with associated angiodestructive granulomatous inflammation. The clinical presentation is variable, including subacute cognitive decline, behavioural changes, headaches, seizures and focal neurological deficits, which may mimic other conditions. Here, we present a case with fatal thrombolysis-related haemorrhage associated with ABRA in a middle-aged patient.Case presentationA 55-year-old man was admitted to hospital with sudden onset left-sided cheek, arm and hand sensory loss, blurred vision, and worsening headache, with a National Institutes of Health Stroke Scale (NIHSS) score of 3. An acute CT head scan showed no contraindications, and therefore the decision was made to give intravenous thrombolysis. Post-thrombolysis, he showed rapid deterioration with visual disturbances, headache and confusion, and a repeat CT head scan confirmed several areas of intracerebral haemorrhage. No benefit from surgical intervention was expected, and the patient died four days after the first presentation. Neuropathological examination found acute ischemic infarcts of three to five days duration in the basal ganglia, insular cortex and occipital lobe, correlating with the initial clinical symptoms. There were also extensive recent intracerebral haemorrhages most likely secondary to thrombolysis. Furthermore, the histological examination revealed severe cerebral amyloid angiopathy associated with granulomatous inflammatory reaction, consistent with ABRA.ConclusionsPresentation of ABRA in a middle-aged patient highlighted the difficulties in recognition and management of this rare condition. There is emerging evidence that patients with CAA may have increased risk of fatal intracerebral haemorrhages following thrombolysis. This may be further increased by a coexisting CAA-related inflammatory vasculopathy which is potentially treatable with steroid therapy if early diagnosis is made.

Co-Occurring NMDA-Receptor and Anti-GAD65 Antibodies in the CSF of a Patient With Encephalitis: Case Report

Objective We describe a 58-year-old woman who presented with rapid cognitive changes and was found to have concurrent CSF NMDA and GAD65 receptor antibodies. Background Antibodies against NMDA and GAD65 receptors are associated with highly morbid autoimmune encephalitides. One case of co-occurring NMDA-R and anti-GAD65 antibodies in a patient with progressive cognitive changes and type I diabetes has previously been described. Herein, we describe a case of a previously high-functioning woman who experienced rapidly progressive cognitive changes secondary to autoimmune encephalitis (AE) with co-existent NMDAR and GAD65 antibodies. Design/Methods NA. Results A 58-year-old woman with adult-onset insulin-dependent diabetes mellitus, hypertension, and prior left-sided Bell's palsy presented to medical care for subacute cognitive decline characterized initially by inattention and difficulty with activities of daily living, progressing to profound global aphasia and seizures. Diagnostic testing revealed GAD65 antibody positivity in serum (33 nmol/L) and CSF (17.3 nmol/L) and negative serum but positive CSF NMDAR antibody. CSF showed pleocytosis (37 nucleated cells, 97% lymphocytes), elevated glucose (109 mg/dL), and normal protein (41 mg/dL). EEG showed right temporal epileptiform discharges. MRI was unrevealing. She was treated with IV steroids, IVIg, and rituximab, and has slowly improved on follow-up. Conclusions Co-existent CSF anti-NMDAR and anti-GAD65 AE is not well-described in the literature. While this patient's serum GAD65 titer was in a range that correlates with neurologic autoimmunity, it was not as high as is typically reported in AE, and could simply be a marker of her insulin-dependent diabetes. However, it was also definitively positive from the CSF, which usually correlates well with GAD65-associated AE. Clinically, the patient had AE features that can be seen associated with either NMDAR and/or GAD65 antibodies. It is interesting to consider whether the GAD65 positivity is most likely to correlate with the patient's adult-onset diabetes, her AE, or both.

Cerebral amyloid angiopathy-related inflammation with posterior reversible encephalopathy syndrome-like presentation: a case report

BackgroundCerebral amyloid angiopathy-related inflammation (CAA-RI), which presents with acute or subacute cognitive or functional decline, focal or multifocal neurologic deficits, new onset of seizures, or a combination of seizures and neurologic deficits, shares clinical and radiologic similarities with posterior reversible encephalopathy syndrome (PRES). Differential diagnosis is critical because the treatment principle for these 2 conditions differs greatly. Here, we present a case of PRES-like CAA-RI and the strategy used to discriminate between the 2 conditions.Case presentationA patient with probable CAA-RI was first thought to suffer from PRES. Initial high-dose methylprednisolone therapy caused rapid improvement of the neurologic symptoms but abrupt discontinuation of corticosteroids resulted in clinical relapse and deterioration. Subsequent reinitiation of high-dose methylprednisolone followed by tapering off of oral prednisone led to clinical and radiologic recovery at the 3-month follow-up.ConclusionsWe suggest that in cases where it is difficult to distinguish between CAA-RI and PRES solely based on magnetic resonance imaging, a good response to corticosteroids and an apolipoprotein E (ApoE) ε4/ε4 genotype are critical for establishing a diagnosis of CAA-RI. If there is clinical deterioration, sudden withdrawal of high-dose corticosteroid during the active phase of CAA-RI should be avoided.

131 Concurrent presentation of cutaneous vasculitis and Creutzfeldt-Jakob disease

A previously well and independent 84-year-old man presented with subacute behavioural disturbance and cognitive decline, progressing over one month to a bedbound encephalopathic state with myoclonus. Examination at that time revealed global cognitive dysfunction, dysarthria, dysphasia, myoclonus and motor pyramidal signs. MRI study revealed widespread high signal on T2-weighted imaging in the hemi- spheric deep white matter, as well as in the right caudate nucleus. EEG revealed frequent periodic triphasic complexes. CSF examination was positive for 14–3–3 and RTQuIC, with S100b 1.2mg/ml, confirm- ing a diagnosis of sporadic Creutzfeldt-Jacob Disease (CJD).During his admission, we noted the emergence of a vasculitic skin rash over his torso spreading bilater- ally over three days. Punch biopsy confirmed the presence of an acute leucocytoclastic vasculitis not suggestive of a drug-associated rash. Further immunological staining did not detect any abnormal prion protein. The rash persisted until he sadly died 4 weeks after admission.In the literature, there are only 3 reported cases of cutaneous manifestations of CJD. The emergence of an acute vasculitic rash in the context of confirmed CJD in our case may suggest immune dysregulation associated with the latter’s underlying pathophysiology, a direct manifestation of prion protein (despite negative staining) or an unrelated concurrent presentation.katieyoganathan@gmail.com

A Case of Cerebral Amyloid Angiopathy-Related Inflammation (CAA-RI) Presenting as a Generalized Tonic Clonic Seizure

Cerebral Amyloid Angiopathy (CAA) is characterized by amyloid beta-peptide deposits within the small to medium-sized vessels of the brain and leptomeninges. CAA is an important cause of intracerebral hemorrhage in older adults. Cerebral Amyloid Angiopathy Related Inflammation (CAA-ri) is, however, a rare variant of CAA that results from an autoimmune response to the deposits and is characterized by acute or subacute encephalopathy, headache, or focal neurological deficits. We present a case of a 62-year-old female who presented with a generalized tonic-clonic seizure witnessed by a family member. The event was preceded by a worsening of her dementia in the past few months. The patient had features suggestive of CAA-ri on Magnetic Resonance Imaging of the brain and was treated with high dose IV steroids. With an improvement in her cognitive symptoms and no further seizure episodes, she was discharged on oral steroids. Although CAA is well studied and well documented, its subset CAA-ri is uncommon with its clinical course and complications mentioned infrequently in medical literature. In conclusion, CAA-ri is underdiagnosed because of its rarity and remains a potentially treatable cause of subacute cognitive decline and seizures demanding further research in this area.

Atypical euthyroid presentation of Steroid Responsive Encephalopathy with Associated Thyroiditis

Introduction&#x0D; Steroid-responsive encephalopathy with associated thyroiditis (SREAT) is an autoimmune disease associated with antithyroid antibodies. Its clinical features are variable, ranging from sudden focal neurologic deficits to progressive sub-acute cognitive decline and thus, can mimic a host of other neurological and psychiatric conditions.&#x0D; Case Presentation&#x0D; We present the case of a previously healthy 62-year-old female with rapid onset neurocognitive and functional decline. EEG and MRI findings were consistent with an encephalopathy of unknown origin. Serologic findings revealed elevated antithyroid antibodies but were otherwise insignificant.&#x0D; Conclusion&#x0D; Steroid-responsive encephalopathy with associated thyroiditis is typically a diagnosis of exclusion but should always be considered in cases of encephalopathy of unknown origin. The disorder is often underdiagnosed due to its variable presentation and poorly understood pathophysiology, but prognosis can be significantly improved with greater physician awareness and prompt management with corticosteroids.

Anti-LGI-1 Encephalitis with Worsening Symptoms Associated with Hyponatremia

Anti-leucine-rich glioma-inactivated 1 (LGI-1) encephalitis is clinically characterized by subacute cognitive decline and frequent seizures including fasciobrachial dystonic seizures. A 67-year-old man visited the emergency department due to the recurrent episodes of seizures of 4 days duration. At the time of the visit, he showed hyponatremia at Na 121 mg/dL. His symptoms were relieved by 3% sodium chloride and water restriction. Here we report a case of anti-LGI-1 encephalitis with worsening symptoms associated with hyponatremia.

Abstract WP483: Grouped Cortical Micro Bleeds a Unique Radiological Marker in Inflammatory Cerebral Amyloid Angiopathy

Background: Inflammatory Cerebral Amyloid Angiopathy (iCAA) is a rare cause of rapidly progressive cognitive decline. Prior studies have proposed radiological diagnostic criteria and demonstrated evidence of inflammation related to Aβ. Though lacking any therapeutic trials, various authors have suggested a single pulse IV Methyl Prednisolone (MPS) therapy as curative in most patients. Methods: We studied clinical, radiological and neuropathological features of 2 of biopsy proven iCAA cases over the past 5 years correlating their neuropathological and radiological features. We also reviewed published literature for radiological findings of proven cases of ICAA Clinical features Case 1: 75/female presented with visual hallucinations, her neurological examination was un-remarkable &gt;Case 2: 84/female presented with language and memory difficulties her neurological examination was remarkable for word finding and object naming difficulties Radiological Features: Gadolinium enhanced brain MRI with fluid attenuated inversion recovery (FLAIR) and susceptibility weighted imaging (SWI) were obtained Patient 1: SWI artifact s in bilateral cerebral hemispheres basal ganglia and cerebellum suggestive of cortical micro-bleeds (CMBs ) there was a cluster of CMBs in the right parietal region associated with FLAIR changes and gadolinium enhancement Patient 2 numerous scattered cortical micro-bleeds (CMBs). There was a large cluster of CMBs in left temporal lobe along with FLAIR changes and gadolinium enhancement Biopsy: B oth patient underwent lesional biopsy corresponding to the area of clustered CMBs Neuropathology: demonstrated histopathological and electron microscopic evidence of perivascular inflammation and amyloid deposition consistent with iCAA Review of literature : In a total of 7 brain MRI s in 3 recently published case series 4 had reported CMBs of which 3 patients were identified representing MRI findings consistent with Grouped CMBs associated with FLAIR changes Conclusion: iCAA is a rare and treatable cause of subacute cognitive decline. We suggest grouped cortical micro bleeds (GCMBs) associated with FLAIR changes as a unique radiological feature of this disorder.

A 33-Year-Old Man with Progressive Diffuse and Focal Neuropsychiatric Signs without Localizing Correlates on Brain Imaging: A Systematic Approach to Diagnosis.

Introduction:Novel expensive diagnostic tests are rapidly emerging. However, the answer to the most complex clinical presentations is often inferred from a systematic approach to the differential diagnosis. This is especially the case in neuropsychiatric disorders that present with a mix of neurologic and psychiatric symptoms. This case report fills a gap in the literature by providing a systematic differential diagnosis of such neuropsychiatric presentations associated with non-focal brain imaging.Case Presentation:A 33-year-old African-American man presented with confusion, weakness, and tremors. He initially noted memory problems and over the following six months progressively became confused, developed speech difficulties and left sided weakness and tremors. On exam, he was predominantly abulic but with intermittent and extreme mood lability. He lacked insight and his attention was poor. He had mild facial weakness and spastic hemiparesis with action tremors on the left side. Magnetic Resonance Imaging of the brain demonstrated non-specific diffuse parenchymal volume loss. His serum and cerebrospinal fluid studies were positive for Rapid Plasma Reagin and Veneral Disease Research Laboratory tests, respectively, suggesting a diagnosis of paretic neurosyphilis.Conclusion:This is a case of a young man with neurosyphilis who presented with progressive subacute cognitive decline, associated with focal neurological signs but no focal lesions on brain imaging. Neurosyphilis is often misdiagnosed on medicine, psychiatry, and neurology inpatient units. In this report, we present an approach to conceptualize similar cases and provide a differential diagnosis that will help reach an accurate diagnosis more efficiently. Further, it raises awareness regarding neurosyphilis, a devastating but easily treatable condition.

Treatable causes of adult-onset rapid cognitive impairment

ObjectivesAcute and subacute cognitive decline, defined collectively as rapid cognitive impairment (RCI), is attributed to diverse disorders and brings great challenges for differential diagnosis. In this study we investigated the RCI patients to determine the underlying causes and the cognitive outcome of the treatable RCI. Patients and MethodsWe reviewed medical records of consecutively hospitalized patients (n = 346) with significant and new cognitive dysfunction between January 2014 and December 2015. Based on the duration of their cognitive dysfunction, patients were divided into two groups with the RCI (< 12 months) and the chronic cognitive impairment (CCI, ≥ 12 months), respectively. Etiologies of the RCI and the CCI were analyzed; the cognitive outcomes of the RCI patients with the treatable disorders were assessed in the follow-up visits. ResultsPotentially treatable or reversible causes were identified in 134 (72%) of 187 RCI patients and in 34 (21%) of 159 CCI patients. The causes in the 134 (72%) RCI patients were immune/inflammation (50, 37%), infection (30, 22%), vascular diseases (29, 22%), neoplasm (16, 12%), metabolic/toxic disorders (7, 5%), and others (2, 1%). The treatable disorders found in both the RCI and the CCI patients were vascular diseases, autoimmune encephalitis, viral encephalitis, inflammatory demyelinating diseases, Hashimoto encephalopathy, neurosyphilis, hydrocephalus, and Vitamin B12 deficiency. Total 114 RCI patients with the treatable disorders were followed up for 6∼39 (median 21) months. Poor cognitive outcomes were found in 24 (21%) of the 114 followed-up patients, comprising patients with infection (1, 3%), immune/inflammation (12, 25%), vascular diseases (8, 28%), and metabolic/toxic disorders (3, 43%). ConclusionsTreatable or reversible causes are common underlying RCI. Poor outcomes with severe cognitive deficits are considerably present in the treatable RCI patients and result in permanent dementia.

Epstein–Barr virus associated CNS lymphoproliferative disorder after long-term immunosuppression

The incidence of Epstein–Barr virus (EBV)associated lymphoproliferative disorders has increased with greater use of immunomodulatory therapies. We present a woman who developed subacute cognitive decline and unilateral weakness while taking...

Autoimmune Epilepsy.

The field of autoimmune epilepsy has evolved substantially in the last few decades with discovery of several neural autoantibodies and improved mechanistic understanding of these immune-mediated syndromes. A considerable proportion of patients with epilepsy of unknown etiology have been demonstrated to have an autoimmune cause. The majority of the patients with autoimmune epilepsy usually present with new-onset refractory seizures along with subacute progressive cognitive decline and behavioral or psychiatric dysfunction. Neural specific antibodies commonly associated with autoimmune epilepsy include leucine-rich glioma-inactivated protein 1 (LGI1), N-methyl-D-aspartate receptor (NMDA-R), and glutamic acid decarboxylase 65 (GAD65) IgG. Diagnosis of these cases depends on the identification of the clinical syndrome and ancillary studies including autoantibody evaluation. Predictive models (Antibody Prevalence in Epilepsy and Encephalopathy [APE2] and Response to Immunotherapy in Epilepsy and Encephalopathy [RITE2] scores) based on clinical features and initial neurological assessment may be utilized for selection of cases for autoimmune epilepsy evaluation and management. In this article, we will review the recent advances in autoimmune epilepsy and provide diagnostic and therapeutic algorithms for epilepsies with suspected autoimmune etiology.

Sleep disturbances are common in patients with autoimmune encephalitis.

Autoimmune encephalitis (AE) is increasingly recognized as an important cause of subacute cognitive decline, seizures, and encephalopathy, with an ever-broadening clinical phenotype. Sleep disturbances are reported in AE patients, including rapid eye movement sleep behavior disorder, hypersomnia, fragmented sleep, and sleep-disordered breathing; however, the prevalence of sleep disturbances and contributions to outcomes in AE patients remain unknown. There is a need to determine the prevalence of sleep disturbances in AE patients, and to clarify the relationship between specific autoantibodies and disruptions in sleep. Clinical history, results of serum and cerebrospinal fluid testing, electroencephalography, and neuroimaging were reviewed from 26 AE patients diagnosed and managed at our tertiary care hospital. Polysomnography was performed in patients with clinical indications, yielding data from 12 patients. The median age of AE patients was 53years (range 18-83). Autoantibodies against intracellular antigens (including Ma and Hu autoantibodies) were identified in 6/26 (23%) patients, while autoantibodies against cell-surface neuronal antigens (including NMDAR and LGI1) were identified in 20/26 (77%) patients. New sleep complaints were reported by 19/26 (73%) AE patients, including gasping or snoring (9/19, 47%), dream enactment behavior (6/19, 32%), insomnia (5/19, 29%), hypersomnia (4/19, 21%), other parasomnias (4/19, 21%), and dream-wake confusional states (2/19, 11%). Dream enactment behaviors were particularly common in AE associated with LGI1 autoantibodies, reported in 4/7 (57%) patients. Polysomnography showed reduced total sleep time, stage 3 and rapid eye movement sleep, and prominent sleep fragmentation. Sleep disturbances are common in AE, warranting active surveillance in affected patients. Improved identification and treatment of sleep disorders may reduce morbidity associated with AE and improve long-term outcomes.

Periodic EEG patterns in sporadic Creutzfeld-Jakob-Disease can be benzodiazepine-responsive and be difficult to distinguish from non-convulsive status epilepticus

PurposePeriodic discharges in EEG and a history of rapidly progressive dementia are known to be associated with sporadic Creutzfeld-Jakob Disease (sCJD). Doubts regarding this rare but fatal diagnosis can arise, when episodic symptoms (seizures, psychiatric features, speech disturbances) are present and the EEG shows epileptiform discharges within this periodicity. This scenario may indicate non-convulsive status epilepticus (NCSE), which is – in contrast to sCJD – a treatable and frequent condition. MethodsHerein we report a small retrospective study of 4 elderly patients, who suffered from sCJD, but due to episodic symptoms and benzodiazepine-responsive epileptiform discharges in combination to a subacute cognitive decline, NCSE was considered as a potential differential diagnosis and therefore treated aggressively. ResultsUltimately, this treatment was not successful and sCJD was diagnosed in all cases. Hence, there was no diagnostic and prognostic value of abolishing periodic discharges in EEG via benzodiazepines in differentiating sCJD from NCSE in our series. ConclusionThese findings indicate that periodic discharges in sCJD can be responsive to benzodiazepines andnot necessarily help to differentiate differentiation between sCJD and NCSE of other causes. We argue that an aggressive anticonvulsive treatment in this scenario should be considered cautiously, especially for invasive options like general anesthesia.