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Anti-NMDA Receptor Encephalitis

Anti-NMDAR Encephalitis


Presentation

  • To the best of our knowledge this is the first report of a patient with anti-NMDAR encephalitis first presenting with total insomnia.[ncbi.nlm.nih.gov]
  • Interestingly, each child presented initially with the chief concern of gait disturbance.[ncbi.nlm.nih.gov]
  • We herein present a case of anti-NMDA receptor encephalitis in which the patient initially presented in the psychiatric department.[ncbi.nlm.nih.gov]
  • Infectious disease work-up was negative and anti-NMDA receptor antibodies were present in the CSF and serum.[ncbi.nlm.nih.gov]
  • We present four cases of confirmed anti-NMDA receptor encephalitis; three presented initially with serious psychiatric symptoms and the other developed significant psychiatric symptoms during the initial phase of illness.[ncbi.nlm.nih.gov]
Fever
  • It is an immune-mediated syndrome which presents with a variety of neurological symptoms including headache, fever, personality change and seizures. Most case reports to date are of young adults, it is much less frequently reported in older adults.[ncbi.nlm.nih.gov]
  • She developed disorientation and hallucination after fever and vomiting. Hypothermia, hypoventilation, hypertension, paralytic ileus and hyponatremia were present.[ncbi.nlm.nih.gov]
  • She developed nausea and psychiatric symptoms, followed by fever and an acute progressive disturbance of consciousness.[ncbi.nlm.nih.gov]
  • We present a 31-year-old Caucasian nulliparous patient who was admitted as an emergency with general illness status accompanied by holocranial cephalalgia and fever.[ncbi.nlm.nih.gov]
  • A 31-year-old woman sub-acutely developed psychosis, behavioural changes, amnesia, alternating states of agitation and mutism, fever and epileptic seizures.[ncbi.nlm.nih.gov]
Hyperthermia
  • Keywords: NMDA, receptor, autoimmune, synapse, paraneoplastic, schizophrenia, paraneoplastic disorder, neurologic symptoms, hyperthermia, hypoventilation, hypersexuality, hyperphagia, hypersomnia, Amnesia, Corticosteriods Abstract: Anti-NMDA receptor[doi.org]
  • […] seizure followed by refractory status epilepticus; sedation, MV, and PEG Partial motor seizures in left lower extremity; dystonic movements; hyperthermia 3 F/25 Mature, left ovary, 6cm 6 weeks Hyperthermia STMD, panic attacks, confusion, hallucinations[ncbi.nlm.nih.gov]
  • Tachycardia and bradycardia, blood pressure lability, hyperthermia, hypoventilation, and/or apnea were each reported in most patients with autonomic instability.[ncbi.nlm.nih.gov]
Fatigue
  • Median duration since diagnosis was similar for children (2.42 years, interquartile range 2.12-3.32) and adults (3.55 years, interquartile range 2.08-5.50 years). 3/4 (75%) pediatric and 3/5 (60%) adult patients reported neuropsychiatric symptoms (fatigue[ncbi.nlm.nih.gov]
  • […] receptor encephalitis have the presence of a tumour (most commonly teratoma) CLINICAL PRESENTATION Up to 90% of patients are female The disorder typically progresses over 1-2 weeks An initial non-specific flu-like prodrome (sub-febrile temperature, headache, fatigue[lifeinthefastlane.com]
  • In the pro-dromal phase, affected individuals have a flu-like illness with fever, malaise, headache, and fatigue.[dx.doi.org]
Malaise
  • In the pro-dromal phase, affected individuals have a flu-like illness with fever, malaise, headache, and fatigue.[dx.doi.org]
Hypoventilation
  • Early tumor resection with immunotherapy facilitates recovery from this disease, but central hypoventilation may require long mechanical support.[ncbi.nlm.nih.gov]
  • The phenotype resembles that of the adults, although dysautonomia and hypoventilation are less frequent or severe in children. Ann Neurol 2009;66:11-18.[ncbi.nlm.nih.gov]
  • Keywords: NMDA, receptor, autoimmune, synapse, paraneoplastic, schizophrenia, paraneoplastic disorder, neurologic symptoms, hyperthermia, hypoventilation, hypersexuality, hyperphagia, hypersomnia, Amnesia, Corticosteriods Abstract: Anti-NMDA receptor[doi.org]
  • We report a 62 year old patient who developed psychiatric disorders followed by epilepsy, movement disorders, mutism and hypoventilation. Flair weighted brain MRI sequences showed diffuse high signals in the limbic system.[ncbi.nlm.nih.gov]
Cough
  • During this first round of IVIg treatment, the patient continued to have periods of vital sign instability and developed a new cough, with decreased ability to protect his airway.[dx.doi.org]
  • MV, PEG Incomprehensible speech, choreoathetotic movements, hypersomnia, hyperthermia, autonomic instability 11 4, 7 F/28 1st episode: mature, right ovary, 14cm; 2nd episode: “benign,” left, 2cm; 3rd episode: “benign,” left, 1.7cm 1 month after tumor Cough[ncbi.nlm.nih.gov]
Nausea
  • She developed nausea and psychiatric symptoms, followed by fever and an acute progressive disturbance of consciousness.[ncbi.nlm.nih.gov]
  • 10cm 2 months Headache, hyperthermia STMD, decreased level of consciousness, “epilepsia partialis continua” for 2 weeks; sedation, MV, PEG Restlessness, involuntary movements, hyperthermia 2 F/35 Mature, left ovary, 3.5cm 4 months (autopsy) Headache, nausea[ncbi.nlm.nih.gov]
  • It follows a specific multistep clinical pattern starting with prodromal signs (such as headache, nausea, low-grade fever) and psychosis (hallucinations, delusions, suicidal thoughts) progressing to epileptic seizures, reduced levels of consciousness,[ncbi.nlm.nih.gov]
Diarrhea
  • […] tonic-clonic seizures, partial motor seizures, severe general encephalopathy, PEG Unable to speak and follow commands for 2 months; episodes of “facial twitches” 6 F/24 Mature, right ovary, 1.5cm 3 months (autopsy) Headache, hyperthermia, nausea, vomiting, diarrhea[ncbi.nlm.nih.gov]
Hypertension
  • Hypothermia, hypoventilation, hypertension, paralytic ileus and hyponatremia were present. Neurological examination showed mild consciousness disturbance and bilateral ophthalmoplegia on admission, flaccid paraplegia with leg areflexia on Day 4.[ncbi.nlm.nih.gov]
  • M 29 66 12 Pacific Islander Mental status changes Choreoathetoid movements Tachycardia, hypertension, hyperthermia Y N Unk Y 8 N 170 (92%L, 2%M) Abnormal; dural and meningeal enhancement Diffuse slowing; some left frontal spikes F 67 66 18 Black Behavioral[ncbi.nlm.nih.gov]
  • During the course of the disease of our patients, hypertension and symptoms of sympathetic overactivity occurred frequently, possibly enhancing blood–brain barrier leakiness.[ncbi.nlm.nih.gov]
  • Enalapril was prescribed to manage hypertension; the patient had consistent systolic readings in the 150–170 mm Hg range and diastolic readings in the 80–90 mm Hg range.[dx.doi.org]
Tachycardia
  • Tachycardia and bradycardia, blood pressure lability, hyperthermia, hypoventilation, and/or apnea were each reported in most patients with autonomic instability.[ncbi.nlm.nih.gov]
  • Autonomic instability, evidenced by blood pressure and temperature fluctuations, tachycardia, bradycardia, and even cardiac pauses, is not uncommon.[dx.doi.org]
  • Five patients had signs of autonomic instability, including episodes of mydriasis, tachycardia, tachypnea, diaphoresis, or hypertension, which in one case alternated with hypotension.[ncbi.nlm.nih.gov]
Hypotension
  • Five patients had signs of autonomic instability, including episodes of mydriasis, tachycardia, tachypnea, diaphoresis, or hypertension, which in one case alternated with hypotension.[ncbi.nlm.nih.gov]
Facial Grimacing
  • Examination on the day of admission revealed an awake, alert, and interactive male with focal speech production difficulties, asymmetric facial grimace, hyperactive deep tendon reflexes, and normal sensation.[dx.doi.org]
Delusion
  • A 26-year-old woman presented at 22 weeks of gestation with intermittent headache and an acute episode of bizarre behaviour and grandiose delusions resulting in hospitalisation.[ncbi.nlm.nih.gov]
  • In early phases of the illness, this life-threatening disease is characterized by psychiatric symptoms, such as depression, anxiety, obsessions, hallucinations or delusions.[ncbi.nlm.nih.gov]
  • Here, we present a case of a 15 year-old female adolescent with first-episode psychosis with anti-NMDA receptor encephalitis not related to tumor, which manifested with delusion, hallucinations, panic attacks, agitation, and neurological symptoms, and[ncbi.nlm.nih.gov]
  • ., delusions, hallucinations, disorganized behavior). The disorder is more common in females (80%), in approximately half of whom it is associated with an underlying ovarian teratoma.[ncbi.nlm.nih.gov]
  • Following the diagnosis of uterine cancer, the patient suddenly developed psychosis with abnormal behaviors, delusions, irritability, and forgetfulness. The cerebrospinal fluid tested positive for anti-NMDAR antibodies (encoding the NR1 subunit).[ncbi.nlm.nih.gov]
Impulsivity
  • NMDA receptors are proteins that control electrical impulses in the brain.[antinmdafoundation.org]
  • Similarly, mood dysregulation disorders, disorders of impulse control, and sleep disorders may be identified.[dx.doi.org]
  • In humans with this autoimmune disease, rogue antibodies bind to the NMDA receptors on neurons, impairing their ability to control electrical impulses in the brain.[smithsonianmag.com]
Aggressive Behavior
  • behavior (seen at a psychiatric center); progressive confusion, decreased level of consciousness; general and partial seizures, refractory status epilepticus; sedation, MV, PEG Episodes of “chewing, grimacing”; myoclonic and ballistic movements with[ncbi.nlm.nih.gov]
  • A previously healthy 22-year-old Asian male demonstrated extremely aggressive behavior (eg, he had thrown someone through a glass door on the day of admission) and was initially admitted for psychosis.[doi.org]
  • Baltimore, A 14-Year-Old Girl with Slurred Speech, Aggressive Behavior, and Seizures, Pediatric Annals, 44, 6, (236), (2015). Jessica A. Panzer, Ronan Anand, Josep Dalmau and David R.[doi.org]
Emotional Lability
Paranoid Ideation
  • There is a dearth of information regarding management of psychiatric and behavioral symptoms, including agitation, anxiety, mood dysregulation, depression, disorganized thinking, paranoid ideation, and hallucinations.[dx.doi.org]
Seizure
  • […] balance at seizure offset.[ncbi.nlm.nih.gov]
  • The presence of EDB, which can follow a pattern of well-characterized electroclinical seizures, in our patients was associated with seizures and SE. These findings suggest that EDB could be an evolutive pattern of an SE in NMDAr encephalitis.[ncbi.nlm.nih.gov]
  • A previously healthy 14-year-old girl presenting with generalized tonic clonic seizures and altered behavior.[ncbi.nlm.nih.gov]
  • Of 32 patients or 18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction.[ncbi.nlm.nih.gov]
  • Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis usually presents with psychiatric symptoms, behavioural changes, impaired consciousness, seizures and autonomic instability.[ncbi.nlm.nih.gov]
Catatonia
  • Malignant catatonia is a severe and life-threatening state associated with anti-NMDA receptor encephalitis. It is often inadequately assessed and may not respond to immunosuppressive treatment.[ncbi.nlm.nih.gov]
  • Most patients with anti-NMDAR encephalitis develop a multistage illness that progresses from initial psychiatric symptoms to memory disturbance, seizures, dyskinesia, and catatonia.[ncbi.nlm.nih.gov]
  • Psychiatrists should consider anti-NMDA receptor encephalitis in patients presenting with psychosis and additional features of dyskinesias, seizures and catatonia, particularly where there is no previous history of psychiatric disorder.[ncbi.nlm.nih.gov]
  • […] presented with a clinical picture that resembled delirium and later appeared to present with a conversion reaction and the second patient presented with a first psychotic break followed by the clinical picture of neuroleptic malignant syndrome with catatonia[ncbi.nlm.nih.gov]
  • We describe the case of a 17-year-old male who presented with acute onset of seizures and malignant catatonia with psychosis, agitation, and hypermetabolism, who responded to electroconvulsive therapy (ECT).[ncbi.nlm.nih.gov]
Headache
  • It is an immune-mediated syndrome which presents with a variety of neurological symptoms including headache, fever, personality change and seizures. Most case reports to date are of young adults, it is much less frequently reported in older adults.[ncbi.nlm.nih.gov]
  • A previously healthy 9-year-old girl presented to an emergency department (ED) with headache, dizziness, blurry vision, and abnormal visual perceptions. She was diagnosed with migraine, treated symptomatically, and discharged.[ncbi.nlm.nih.gov]
  • A 26-year-old woman presented at 22 weeks of gestation with intermittent headache and an acute episode of bizarre behaviour and grandiose delusions resulting in hospitalisation.[ncbi.nlm.nih.gov]
  • It is a life threatening condition that typically presents with viral illness, headaches, severe psychiatric symptoms, seizures, behavioural changes, decreasing levels of unconsciousness and progressive unresponsiveness, cognitive impairment, abnormal[ncbi.nlm.nih.gov]
  • Early symptoms may include fever, headache, and feeling tired. This is then typically followed by psychosis which presents with false beliefs (delusions) and seeing or hearing things that others do not see or hear (hallucinations).[en.wikipedia.org]
Confusion
  • Recognition of the syndrome, especially in pediatric populations, is difficult and often undiagnosed and/or confused with neurological disorders with similar clinical features.[ncbi.nlm.nih.gov]
  • A 15 years old female patient presented with fever, changed mental status of confusion, rigidity of the arms and legs, and oral-facial dyskinesias.[ncbi.nlm.nih.gov]
  • PRESENTATION Up to 90% of patients are female The disorder typically progresses over 1-2 weeks An initial non-specific flu-like prodrome (sub-febrile temperature, headache, fatigue) followed by a psychotic stage with bizarre behaviour, disorientation, confusion[lifeinthefastlane.com]
  • Flu-like symptoms Memory deficits, including loss of short-term memory; Sleep disorders; Speech dysfunction – the patient is no longer able to produce coherent language or may be completely unable to communicate Cognitive and behavioural disturbances – confused[antinmdafoundation.org]
  • People are also often agitated or confused.[en.wikipedia.org]
Agitation
  • We describe the case of a 17-year-old male who presented with acute onset of seizures and malignant catatonia with psychosis, agitation, and hypermetabolism, who responded to electroconvulsive therapy (ECT).[ncbi.nlm.nih.gov]
  • Here, we present a case of a 15 year-old female adolescent with first-episode psychosis with anti-NMDA receptor encephalitis not related to tumor, which manifested with delusion, hallucinations, panic attacks, agitation, and neurological symptoms, and[ncbi.nlm.nih.gov]
  • He progressed to a state of reduced alertness and responsiveness, presenting periods of agitation with abnormal dyskinetic movements, periods of autonomic instability and central hypoventilation.[ncbi.nlm.nih.gov]
  • These included encephalitic signs with psychiatric symptoms (agitation, paranoid thoughts, irritability, or hallucinations); seizures; CSF inflammation; and exclusion of viral or bacterial infection.[ncbi.nlm.nih.gov]
  • A 31-year-old woman sub-acutely developed psychosis, behavioural changes, amnesia, alternating states of agitation and mutism, fever and epileptic seizures.[ncbi.nlm.nih.gov]

Workup

  • Workup revealed antibodies against N-methyl-d-aspartate receptor (NMDAR). Immunotherapy treatment led to a very significant improvement with the patient presenting only slight frontal lobe dysfunction after one year of recovery.[ncbi.nlm.nih.gov]
  • The diagnostic workup of this common autoimmune encephalitis includes cerebrospinal fluid analysis, electroencephalography, magnetic resonance imaging (MRI), and fluorodeoxyglucose positron emission tomography (FDG-PET).[ncbi.nlm.nih.gov]
  • Magnetic resonance imaging (MRI) may be used as a component of the workup for encephalopathy. However, the use of MRI in anti-NMDA encephalitis is complicated by wide-ranging reports regarding the frequency of normal MRI findings in this disease.[ncbi.nlm.nih.gov]
  • Example of Workup Prior to Diagnosis of Anti-NMDAR Encephalitis If the diagnosis of anti-NMDAR encephalitis is not considered, the workup often includes an extensive study for infectious diseases.[doi.org]

Treatment

  • […] as second-line treatments, and tumor removal.[ncbi.nlm.nih.gov]
  • These guidelines may promote treatment trials of immunomodulators in autoimmune encephalitides. KEYWORDS: Anti-NMDA receptor encephalitis; Immunotherapy; Treatment[ncbi.nlm.nih.gov]
  • Here, we report our experience with two pediatric patients, in whom no tumors were present during treatment for encephalitis, but in whom ovarian teratomas developed without encephalitis relapse after treatment was completed.[ncbi.nlm.nih.gov]
  • TREATMENT IMPLICATIONS: Early diagnosis and aggressive treatment promote better outcomes. First-line treatment includes immunotherapy. 2015 Wiley Periodicals, Inc.[ncbi.nlm.nih.gov]
  • The mainstays for treatment have been early diagnosis, tumor work-up and removal if found, and initiation of immunosuppressive therapy. Treatment response is often slow and residual symptoms common.[ncbi.nlm.nih.gov]

Prognosis

  • EEG could sensitively reflect the abnormal brain functions of patients and could assist with early clinical diagnosis and prognosis prediction.[ncbi.nlm.nih.gov]
  • Early diagnosis and treatment is essential for recovery and may improve the prognosis.[ncbi.nlm.nih.gov]
  • The prognosis is good with early recognition and treatment, though may relapse. We present a case of NMDA encephalitis in an elderly patient who responded well to immunosuppressive therapy.[ncbi.nlm.nih.gov]
  • Early and aggressive therapy has been shown to improve prognosis although problems with executive functions and memory have continued for several years.[ncbi.nlm.nih.gov]
  • Follow-up visits allowed the analysis of the natural disease course and estimation of prognosis. Seven patients (all women) fulfilled the indicated criteria; 6 of them had NMDAR antibodies.[ncbi.nlm.nih.gov]

Etiology

  • Abstract The California Encephalitis Project (CEP), established in 1998 to explore encephalitic etiologies, has identified patients with N-methyl-D-aspartate receptor (NMDAR) antibodies, the likely etiology of their encephalitis.[ncbi.nlm.nih.gov]
  • Introduction The causes of encephalitis are numerous, and most patients undergo extensive testing to identify infectious etiologies. However, other etiologies need to be considered.[doi.org]
  • Knut the polar bear of the Berlin Zoological Garden drowned in 2011 following seizures and was diagnosed as having suffered encephalitis of unknown etiology after exhaustive pathogen screening.[ncbi.nlm.nih.gov]
  • Extreme delta brush (EDB) is a distinctive EEG pattern that is can be suggestive of the diagnosis; however, the etiology of the EDB remains unclear. Furthermore, there is question with regard to its ictal or interictal nature.[ncbi.nlm.nih.gov]

Epidemiology

  • He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education.[lifeinthefastlane.com]
  • In 2007, the California Encephalitis Project (CEP), which was established to study the epidemiology of encephalitis, began identifying cases of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis.[ncbi.nlm.nih.gov]
  • Interpretation Our study extends epidemiological and clinical knowledge on anti-NMDAR encephalitis.[ncbi.nlm.nih.gov]
  • The California Encephalitis Project (CEP) was initiated in 1998 to identify the etiologic agents and define the clinical and epidemiologic characteristics associated with encephalitis.[doi.org]
  • We present a review of the literature on the disorder, including its clinical presentation, differential diagnosis, epidemiology, treatment and prognosis.[dx.doi.org]
Sex distribution
Age distribution

Pathophysiology

  • The similarities identified between anti-NMDA-R encephalitis and schizophrenia-spectrum illnesses also raise questions about a common underlying pathophysiology particularly in regard to the NMDA-R.[ncbi.nlm.nih.gov]
  • These observations suggest that NMDA receptors may play a role in the pathophysiology of CNH.[ncbi.nlm.nih.gov]
  • Second, it provides valuable insights into the pathophysiology of such anti-NMDAR encephalitis; these insights prove that anti-NMDAR encephalitis is linked not only to hyperglutamatergic signals but also to hypoglutamatergic states.[ncbi.nlm.nih.gov]
  • A review of the literature is provided summarizing the disorder and its known cognitive sequelae, pathophysiology, treatment, and prognostic factors, as well as each patient's relevant history, symptom presentation, and disease course.[ncbi.nlm.nih.gov]
  • Abstract We characterised the pathophysiology of seizure onset in terms of slow fluctuations in synaptic efficacy using EEG in patients with anti-N-methyl-d-aspartate receptor (NMDA-R) encephalitis.[ncbi.nlm.nih.gov]

Prevention

  • All patients received varied forms of chronic immunosuppression to prevent relapses. Complications of chronic immunotherapy occurred in 50% of patients.[ncbi.nlm.nih.gov]
  • No analysis of relapse risk has yet been reported in cases of tumor development after treatment, and at this point, whether or not resection is needed to prevent relapse remains unclear.[ncbi.nlm.nih.gov]
  • In fact, there are no studies to show that these drugs are effective in the acute stage or are useful to prevent relapses. We definitely need multicenter prospective studies to evaluate the efficacy of all these immunotherapies.[dx.doi.org]
  • Immune suppressing medications may be taken for prolonged periods of time (even after recovery) to prevent a relapse.[antinmdafoundation.org]
  • This cellular filter, separating the central nervous system from the circulatory system, normally prevents larger molecules from entering the brain.[en.wikipedia.org]

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