Rapidly Progressive Memory Loss, Mood Change, Mutism, and Abnormal Movements

2021 ◽  
pp. 83-85
Author(s):  
Shailee S. Shah ◽  
Marie F. Grill
Keyword(s):  
Cerebrospinal Fluid ◽  
Short Term Memory ◽  
White Blood Cells ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Aspartate Receptor ◽  
Normal Glucose ◽  
Disorganized Speech ◽  
Patient’S History

A 24-year-old woman sought care for 2 weeks of disorientation and short-term memory difficulties, as well as diffuse tremor of all extremities. She returned with further decline in memory and new severe headaches. She had intermittent agitation and emotional outbursts of crying or laughing, insomnia, spells consisting of disorganized speech and episodes of intermittent right gaze deviation with facial twitching and lip smacking. She was nearly mute. Her appetite had decreased and she had not had a bowel movement in several days. She was noted to have significant tachycardia and was intermittently febrile. Within several days she became unresponsive to all external stimuli, with nonpurposeful eye movements and frequent dyskinesias observed, and ultimately required ventilator support. Testing of the cerebrospinal fluid showed 236 white blood cells/µL, mildly increased protein concentration of 50 mg/dL, and normal glucose values. Electroencephalography initially demonstrated generalized slowing and generalized periodic epileptiform discharges and was also notable for an extreme delta brush pattern. Bilateral ovarian masses were identified on pelvic ultrasonography, and subsequent computed tomography of the abdomen and pelvis showed bilateral teratomas. An autoimmune encephalitis autoantibody panel was positive for antibodies targeting the N-methyl-d-aspartate receptor in the serum and cerebrospinal fluid, by both cell-based and immunofluorescence assays. The patient was diagnosed with anti- N-methyl-d-aspartate receptor encephalitis. The patient initially received intravenous methylprednisolone, followed by intravenous immunoglobulin. Benzodiazepines and propranolol were used to manage agitation and dysautonomia. Antiepileptic drugs were initiated for seizures. She required mechanical ventilation and parenteral nutrition given her persistent profound encephalopathic state. She underwent left ovarian cystectomy and right salpingo-oophorectomy. This patient’s history highlights the progressive clinical features characteristic of anti- N-methyl-d-aspartate receptor encephalitis and the long but often complete or near-complete recovery.

scholarly journals Cerebrospinal Fluid IL-17A Could Predict Acute Disease Severity in Non-NMDA-Receptor Autoimmune Encephalitis

2021 ◽  
Vol 12 ◽  
Author(s):  
Michael Levraut ◽  
Véronique Bourg ◽  
Nicolas Capet ◽  
Adrien Delourme ◽  
Jérôme Honnorat ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Disease Severity ◽  
Disease Onset ◽  
Autoimmune Encephalitis ◽  
Demyelinating Diseases ◽  
Acute Disease ◽  
Aspartate Receptor ◽  
Initial Severity ◽  
Csf Concentration

IntroductionMost of our knowledge into autoimmune encephalitis (AE) comes from N-Methyl-D-Aspartate Receptor (NMDAR) encephalitis. The concentrations of cytokines in cerebrospinal fluid (CSF) including IL-17A have been found to be increased and associated with poor outcome. However, data on the cytokine concentration in CSF and its correlation with outcome is lacking for other types of AE.ObjectiveTo report the concentrations of CSF sIL-2R, IL-6, IL-8, IL-10 and IL-17A and to correlate it with acute disease severity and the 1-year outcome in non-NMDAR AE.MethodsWe measured the CSF concentration of each cytokine in 20 AE patients, and compared IL-6 and IL-17A concentrations with 13 patients with CNS demyelinating diseases and 20 non-inflammatory controls. Patients were > 18yr and had at least 1-year clinical follow-up. Intracellular and NMDAR antibody (Ab) -mediated encephalitis were excluded. A mRS ≤ 2 was retained as a 1-year good outcome.ResultsThe IL-17A concentration in CSF was higher in AE patients than in both control groups (p<0.01). No difference was observed in CSF concentration of IL-6 between groups. At disease onset, a high CSF IL-17A concentration correlated with a high modified Rankin Scale (p<0.05), a high Clinical Assessment Scale for Autoimmune Encephalitis score (p<0.001) and ICU admission (p<0.01). There was no correlation between the concentration of all CSF cytokines and the 1-year clinical outcome.ConclusionOur results show that CSF IL-17A could be interesting to assess initial severity in non-NMDAR AE. Thus, CSF IL-17A could be an interesting therapeutic target and be useful to assess early selective immunosuppressive therapy.

scholarly journals Mature ovarian teratoma-associated encephalitis

2022 ◽  
pp. 4-4
Author(s):  
Anita Krsman ◽  
Branislava Baturan ◽  
Dmitar Vlahovic ◽  
Zorica Grujic ◽  
Djordje Petrovic ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Mature Teratoma ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Neurological Status ◽  
Sudden Onset ◽  
Cranial Computed Tomography ◽  
Ovarian Teratoma ◽  
Unusual Behavior ◽  
Clinical Conditions

Introduction. Autoimmune encephalitis associated with ovarian teratoma is a serious and potentially fatal pathology. While this clinical entity is known to neurologists, the available literature rarely mentions the role of a gynecologist in diagnostic imagining and treatment. Although several months have passed from the appearance of the symptoms to surgical treatment, this case shows that even then a complete recovery is possible. Case presentation. The patient was a 28-year-old female, brought to the hospital because a sudden onset of unusual behavior - an acute psychosis with suicidal thoughts and auditory hallucinations. Soon after the admission she became delirious, uncooperative and agitated. Blood check, neurological assessment and cranial computed tomography yielded normal results. Therefore, a psychiatric disorder was suspected. Electroencephalogram revealed a diffuse encephalitic insufficiency. As cerebrospinal fluid was negative for infections, the autoimmune etiology of the disease was suspected. Abdominal computer tomography showed a complex right ovarian mass measuring 50 x 40 x 30 mm, confirmed by vaginal ultrasound. Laparoscopy with right adnexectomy was performed. The pathohistological finding showed a mature teratoma. In the meantime, the result of the cerebrospinal fluid test came positive for anti NMDAR antibodies. Six months after surgery, the patient was in a good mental and neurological status without symptoms. Conclusion. Gynecologists should be aware of the presence of ovarian tumors in encephalitis cases. A timely diagnosis of the underlying gynecological cause of a neurological condition, allows for prompt treatment and can remarkably improve clinical conditions and, thus, be lifesaving.

scholarly journals A critical reflection on our first patient presenting with Anti-Nmethyl- D-aspartate receptor encephalitis

2019 ◽  
Vol 11 (3) ◽  
Author(s):  
Susanne Buechner ◽  
Igor Florio ◽  
Gabriele J. Sixt ◽  
Francesco Teatini
Keyword(s):  
Treatment Guidelines ◽  
Autoimmune Encephalitis ◽  
Complete Recovery ◽  
Intravenous Immunoglobulins ◽  
High Dose ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
One Year

One of the best characterized autoimmune encephalitis is the Anti-Nmethyl-D-aspartate receptor (NMDAR) encephalitis, which may occur in the presence of cancer. First- and second-line immunotherapy and oncological investigations are suggested. We present here a case of an 18-year-old female who was our first patient suffering from Anti-NMDAR encephalitis more than 9 years ago. She was satisfactorily treated with intravenous immunoglobulins and high dose steroid therapy. After more than one year the patient had a relapse. First-line immunotherapy was repeated; however, a complete recovery was achieved only after plasmapheresis. Afterwards, she continued maintenance immunotherapy with steroids for two years and with Azathioprine for about five years associated to regular oncological assessment. In the last years our therapeutical approach of Anti-NMDARencephalitis has significantly changed. Nevertheless, established treatment guidelines are still missing and the role of long-term maintenance immunotherapy is largely unexplored. In addition, oncological revaluation might be indicated in selected patients.

Behavioral and Cognitive Changes Followed by Coma

2021 ◽  
pp. 86-89
Author(s):  
Anastasia Zekeridou
Keyword(s):  
Cerebrospinal Fluid ◽  
Short Term Memory ◽  
Epileptiform Activity ◽  
Memory Loss ◽  
Lymph Node Biopsy ◽  
Short Term ◽  
Cognitive Changes ◽  
Term Memory ◽  
Acid Receptor ◽  
Fluid Analysis

A 60-year-old woman sought care for behavioral and cognitive changes over 1 week. She became irritable, could not run her daycare facility, and had short-term memory loss and. She was admitted to the hospital, where decreased consciousness developed over 2 to 3 days. She became comatose and needed to be intubated because of an aspiration event. Her clinical examination at admission showed short-term memory and attention deficits and generalized hyperreflexia without any meningeal signs or myoclonic jerks. She also had evidence of left axillary and retroclavicular lymphadenopathy. Magnetic resonance imaging of the brain showed T2 and fluid-attenuated inversion recovery bilateral hyperintensities in the caudate nuclei and putamina, without any diffusion abnormalities. Cerebrospinal fluid analysis showed lymphocytic, increased protein, and cerebrospinal fluid -restricted oligoclonal bands. Electroencephalography showed diffuse slowing with no epileptiform activity. Neural autoantibody testing was positive for immunoglobulin G antibodies to α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor in the serum and cerebrospinal fluid by indirect tissue immunofluorescence and antigen-specific cell-based assays. Because of suspicion for paraneoplastic neurologic disease, positron emission tomography of the body was performed and showed hypermetabolic axillary, retropectoral, and retroclavicular lymph nodes. Lymph node biopsy showed metastatic melanoma of unknown primary. A diagnosis of paraneoplastic anti- α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis was made. The patient received treatment for melanoma (vemurafenib and cobimetinib) and encephalitis (intravenous methylprednisolone) and plasma, followed by rituximab. She remained intubated and unresponsive but subsequently recovered substantially. By 3 months the patient had minimal short-term memory loss; at 2-year follow-up her function had returned to baseline and her cancer was in remission. Anti-α‎-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor encephalitis can be severe, necessitating a protracted immunotherapy course and cancer treatment.

scholarly journals Anti-AMPA-Receptor Encephalitis Presenting as a Rapid-Cycling Bipolar Disorder in a Young Woman with Turner Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Giuseppe Quaranta ◽  
Angelo Giovanni Icro Maremmani ◽  
Giulio Perugi
Keyword(s):  
Bipolar Disorder ◽  
Turner Syndrome ◽  
Ampa Receptor ◽  
Short Term Memory ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Rapid Cycling ◽  
Case Description ◽  
Autoimmune Conditions ◽  
Paraneoplastic Disorder

Background.Autoimmune encephalitis is a disorder characterised by the subacute onset of seizures, short-term memory loss, and psychiatric and behavioural symptoms. Initially, it was recognised as a paraneoplastic disorder, but recently a subgroup of patients without systemic cancer was identified.Case Description.We describe a 20-year-old woman with Turner syndrome presenting with a treatment-resistant rapid cycling bipolar disorder with cognitive impairment. She was diagnosed with anti-AMPA-receptor encephalitis. She showed marked improvement after starting memantine and valproic acid.Conclusion.This case description emphasises the importance of timely recognition of autoimmune limbic encephalitis in patients with psychiatric manifestations and a possible predisposition to autoimmune conditions, in order to rule out malignancy and to quickly initiate treatment.

A Case of Trimethoprim-Sulfamethoxazole-Induced Aseptic Meningitis Masquerading as Septic Shock

2021 ◽  
Author(s):  
Vishaka R Hatcher ◽  
Robert M Brooks ◽  
Karen B Barker ◽  
Christopher A Coop
Keyword(s):  
Septic Shock ◽  
Adverse Reaction ◽  
Aseptic Meningitis ◽  
White Blood Cells ◽  
Autologous Stem Cell Transplant ◽  
Cell Transplant ◽  
Elevated Protein ◽  
Normal Glucose ◽  
Patient’S History ◽  
Csf Findings

ABSTRACT Trimethoprim-sulfamethoxazole-induced aseptic meningitis (TSIAM) is a rare adverse reaction to a commonly prescribed antibiotic. We describe a case of severe TSIAM which resembled septic shock. A 30-year-old male with relapsed Hodgkin’s lymphoma 25 days status post autologous stem cell transplant presented to our clinic for evaluation of trimethoprim-sulfamethoxazole (TMP-SMX) hypersensitivity. After review of patient’s history and records, we had a low suspicion for a TMP-SMX adverse reaction and conducted an oral challenge to one 160 mg/800 mg tab of TMP-SMX. Four hours later, the patient developed vomiting, lightheadedness, and disorientation with progression to rigors, fever, tachycardia, and hypotension. He was admitted for fluid resuscitation and broad-spectrum antibiotic coverage for neutropenic fever and possible septic shock. A lumbar puncture performed due to complaints of headache, photophobia, and neck pain showed 375 white blood cells/µL with 73% neutrophil predominance, normal glucose (75 mg/dL), and elevated protein (101 mg/dL); additional cerebrospinal fluid (CSF) studies were negative for infectious etiologies. Fever and headache resolved by hospital day 4, at which time patient was discharged home. We believe this case represents TSIAM given the characteristic timing of symptom onset, CSF findings, and timing of symptom resolution without other clear etiology found on extensive infectious evaluation. It is important for allergists to recognize TSIAM, including its potential presentation as shock, in order to appropriately diagnose and counsel patients who seek evaluation for TMP-SMX adverse reactions.

scholarly journals Clinical Relevance of Cerebrospinal Fluid Antibody Titers in Anti-N-Methyl-d-Aspartate Receptor Encephalitis

2021 ◽  
Vol 12 (1) ◽  
pp. 4
Author(s):  
Meng-Ting Cai ◽  
Yang Zheng ◽  
Sa Wang ◽  
Qi-Lun Lai ◽  
Gao-Li Fang ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Psychiatric Symptoms ◽  
Autoimmune Encephalitis ◽  
Memory Deficits ◽  
Clinical Severity ◽  
Weak Correlation ◽  
Aspartate Receptor ◽  
Nmdar Encephalitis ◽  
Significant Difference ◽  
The Relationship

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is the most common autoimmune encephalitis. To date, there has been no study on the relationship between antibody (Ab) titers and clinical phenotype. This study aims to clarify the relationship between cerebrospinal fluid Ab titers and clinical manifestations of anti-NMDAR encephalitis at onset. Seventy-six consecutive patients with a definite diagnosis were enrolled. The relationship between Ab titers and different onset symptoms including psychiatric symptoms, seizures, and memory deficits were analyzed. We further investigated the correlation between Ab titers and clinical severity as assessed by the modified Rankin scale (mRS) and the clinical assessment scale for autoimmune encephalitis (CASE), respectively. The Ab titers had a median value of 1:10 (range 1:1–1:100). There was no significant difference in titers among various clinical factors including gender and combination of tumor and other diseases (each p > 0.05). Patients presenting with psychiatric symptoms at onset had higher titers than those with seizures (p = 0.008) and memory deficits (p = 0.003). The mRS scores revealed a significant but weak correlation with Ab titers (r = 0.243, p = 0.034), while CASE scores did not correlate with the titers (p = 0.125). Our findings indicated that the Ab titers were associated with the type of onset symptoms, with a higher level of patients with psychiatric symptoms. Regarding the clinical severity, the titers showed a weak correlation with the mRS, but no correlation with the CASE.

scholarly journals Anti-Alpha-Amino-3-Hydroxy-5-Methyl-4-Isoxazolepropionic Acid Receptor Encephalitis: A Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Tian-Yi Zhang ◽  
Meng-Ting Cai ◽  
Yang Zheng ◽  
Qi-Lun Lai ◽  
Chun-Hong Shen ◽  
...  
Keyword(s):  
Medial Temporal Lobe ◽  
Short Term Memory ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Abnormal Behavior ◽  
High Dose ◽  
First Line ◽  
Acid Receptor ◽  
Cancer Breast ◽  
Fluid Attenuated Inversion Recovery

Anti-alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) encephalitis, a rare subtype of autoimmune encephalitis, was first reported by Lai et al. The AMPAR antibodies target against extracellular epitopes of the GluA1 or GluA2 subunits of the receptor. AMPARs are expressed throughout the central nervous system, especially in the hippocampus and other limbic regions. Anti-AMPAR encephalitis was more common in middle-aged women and most patients had an acute or subacute onset. Limbic encephalitis, a classic syndrome of anti-AMPAR encephalitis, was clinically characterized by a subacute disturbance of short-term memory loss, confusion, abnormal behavior and seizure. Magnetic resonance imaging often showed T2/fluid-attenuated inversion-recovery hyperintensities in the bilateral medial temporal lobe. For suspected patients, paired serum and cerebrospinal fluid (CSF) testing with cell-based assay were recommended. CSF specimen was preferred given its higher sensitivity. Most patients with anti-AMPAR encephalitis were complicated with tumors, such as thymoma, small cell lung cancer, breast cancer, and ovarian cancer. First-line treatments included high-dose steroids, intravenous immunoglobulin and plasma exchange. Second-line treatments, including rituximab and cyclophosphamide, can be initiated in patients who were non-reactive to first-line treatment. Most patients with anti-AMPAR encephalitis showed a partial neurologic response to immunotherapy.

Autoimmune encephalitis and Morvan’s syndrome

2020 ◽  
pp. 6393-6396
Author(s):  
Camilla Buckley ◽  
Angela Vincent
Keyword(s):  
Short Term Memory ◽  
Age At Onset ◽  
Memory Loss ◽  
Autoimmune Encephalitis ◽  
Psychological Disturbance ◽  
Term Memory ◽  
Voltage Gated ◽  
Men 2 ◽  
Morvan’S Syndrome ◽  
Autoimmune Limbic Encephalitis

Since its first recognition in 2001, hundreds of patients have been identified with autoimmune limbic encephalitis (LE) associated with antibodies that immunoprecipitate voltage-gated potassium channel (VGKC)-complex proteins. Preliminary epidemiology suggests that it is more common in men (2:1) and that the median age at onset is 65 years. The phenotype has been recognized mainly in patients over the age of 18 years at onset. The classic presentation is with subacute onset of short-term memory loss, seizures, disorientation, with psychological disturbance or hallucinations. Additional features that may occur are sleep disturbance, autonomic dysfunction, and neuromyotonia, but these would be more typical of Morvan’s syndrome.

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