scholarly journals Case Report: Anti-flotillin 1/2 Autoantibody-Associated Atypical Dementia

2021 ◽  
Vol 12 ◽  
Author(s):  
Niels Hansen ◽  
Claudia Bartels ◽  
Winfried Stöcker ◽  
Jens Wiltfang ◽  
Charles Timäus

Flotillin proteins are involved in neurodegeneration and T-cell immunity. Here, we report the case of 65-year-old woman who presented with dementia, depressive symptoms, and a patient history involving speech problems. As diagnostics methods we applied magnetic resonance imaging, clinical examination, extensive neuropsychological testing, and cerebrospinal fluid analysis. Neuropsychological testing revealed major cognitive decline in attentional, executive, and memory functions together with impaired activities of daily living. The cerebrospinal fluid showed elevated phosphorylated tau protein 181. We identified serum autoantibodies against the flotillin 1/2 complex. Immunotherapy entailing four cycles of high-dose steroids resulted in less cognitive dysfunction along with reduced depressive symptoms in the second follow-up after starting steroids. In conclusion: probable autoimmune-mediated dementia associated with anti-flotillin 1/2 complex autoantibodies expands the phenotypic spectrum of anti-flotillin 1/2 antibody disease.

2021 ◽  
Vol 9 ◽  
pp. 232470962110121
Author(s):  
Reshmi Mathew ◽  
Michael Chahin ◽  
Carmen Isache

Neisseria gonorrhoeae, a gram-negative coccus, is a major cause of morbidity among sexually active individuals. Hematogenous spread of N gonorrhoeae from the initial site of infection is thought to occur in only 0.5% to 3% of infected patients. Disseminated gonococcal infections can rarely lead to serious sequelae, such as endocarditis or meningitis. In this article, we present a case that demonstrates a rare finding of disseminated gonococcal infection leading to N gonorrhoeae meningitis, complicated by hydrocephalus. The patient in this case initially presented with intermittent polyarthralgias for two years and later developed a rash. Cultures from blood and joint aspirate were negative. Urine nucleic acid amplification test for N gonorrhoeae was also negative. He was initially started on steroids for what was believed at first to be an autoimmune polyarthritis. The patient later developed acute encephalopathy. Head imaging revealed hydrocephalus. Cerebrospinal fluid analysis was consistent with bacterial meningitis. Blood, joint, and mucosal membrane studies failed to isolate the causative organism, but his cerebrospinal fluid grew N gonorrhoeae. He was treated with high-dose intravenous ceftriaxone for two weeks with rapid improvement in his mental status and resolution of his joint pains and rash.


Author(s):  
Lorenzo Mari ◽  
Anita Shea

ABSTRACT A 4 yr old, intact female cocker spaniel was presented for investigation of acute, progressive lethargy/hypersomnia; vestibular signs; and cataplexy. A narcolepsy-cataplexy episode with associated hypertension and bradycardia was triggered during examination. There was no evidence of arrhythmia on electrocardiography during the episode. Hematology, serum biochemistry, and thoracic and abdominal imaging were unremarkable. MRI of the brain and cerebrospinal fluid analysis were compatible with meningoencephalitis of unknown origin affecting the mesencephalon, pons and rostral medulla oblongata. The dog was started on immunosuppressive treatment with prednisolone and cytosine arabinoside, which was subsequently switched to cyclosporine. Narcolepsy-cataplexy episodes could initially still be triggered by offering food; however, they gradually became shorter and less frequent until they completely subsided along with all other clinical signs after 3 wk. No relapse occurred over a 32 mo follow-up period from the diagnosis. Repeated MRI revealed marked reduction in the lesion size; cerebrospinal fluid analysis revealed no abnormalities. Although very rare, symptomatic narcolepsy/cataplexy can occur in dogs and can be secondary to brainstem encephalitis. Cardiovascular changes can occur in association with narcolepsy/cataplexy and should be considered when dealing with patients presenting with these specific clinical signs.


2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S242-S243
Author(s):  
Sarah I Collens ◽  
Douglas R Wilcox ◽  
Shibani Mukerji ◽  
Farrah J Mateen ◽  
Isaac H Solomon

Abstract Background Eastern equine encephalitis (EEE) is a mosquito-borne viral infection with significant neurological morbidity and mortality. The clinical presentation and patient outcomes after treatment with IVIG, high-dose steroids, or standard of care alone in EEE remains unclear. Methods A retrospective observational study of patients admitted to two tertiary academic medical centers in Boston, Massachusetts with EEE from 2005 to 2019. Results Of 17 patients (mean [SD] age, 50 [26] years; 10 (59%) male, and 16 (94%) White race), 17 patients had fever (100%), 15 had encephalopathy (88%), and 12 had headache (71%). Eleven of 14 patients with cerebrospinal fluid (CSF) cell count differential had a neutrophil predominance (mean [SD], 60.6% of white blood cells [22.8]) with an elevated protein level (mean [SD], 112 mg/dL [48.8]). Affected neuroanatomical regions included the basal ganglia (n=9/17), thalamus (n=7/17), and mesial temporal lobe (n=7/17). A total of 11 patients (65%) received IVIG; 8 (47%) received steroids. Of the patients who received IVIG, increased time from hospital admission to IVIG administration correlated with worse long-term disability as assessed by modified Rankin Score (mRS) (r=0.72, p=0.02); steroid use was not associated with mRS score. The mortality was 12%. Figure 1. Imaging Characteristics: Typical Pattern of MRI Involvement and Affected Neuroanatomical Regions in Patients with Eastern Equine Encephalitis. All images displayed are the T2-FLAIR sequence. (A) Representative images of pattern of typical neuroanatomical region involved in one patient with demonstrated involvement of the temporal lobe and pons, temporal lobe and midbrain, and basal gangial by T2-FLAIR hyperintensity (panels left to right). (B) Representative images of patients with mild (mRS 0–2), moderate (mRS 3–4), and severe (mRS 5–6) disability score at discharge. (C) Representative images of one patient over course of hospitalization at days 1, 4, and 10 after admission. (D) Quantification of neuroanatomical region involvement in initial MRI of patients with EEE as determined by T2-FLAIR hyperintensity. An area was scored as abnormal only once per patient. Figure 2. Outcomes in Patients with Eastern Equine Encephalitis. Patient disability by modified Rankin Score (mRS) of EEE patients at admission to the hospital, discharge from the hospital, and last recorded follow-up (A). Time to IVIG administration compared to mRS at discharge (B), and most recent clinical follow-up (C). Table 1. Demographics, Clinical Characteristics, and Laboratory Data in Patients with Eastern Equine Encephalitis. Abbreviations: CSF = cerebrospinal fluid, WBC = white clood count, EEG = electroencephalogram, ALT = alanine aminotransferase, AST = aspartate transaminase. Demographic data was collected for all patients with confirmed EEE. Altered mental status included any description of encephalopathy, confusion, or difficulty with attention. Seizures were defined as clinical events with a high-degree of suspicion to be true seizures, and were entirely comprised of generalized tonic-clonic seizures. Conclusion Clinicians should suspect EEE in immunocompetent patients with early subcortical neuroimaging abnormalities and CSF neutrophilic predominance. This study suggests a lower mortality than previously reported, but a high morbidity rate in EEE. IVIG as an adjunctive to standard of care may be considered early during hospitalization. Disclosures All Authors: No reported disclosures


Author(s):  
Harald Hegen ◽  
Charlotte E. Teunissen ◽  
Finn Sellebjerg ◽  
Hayrettin Tumani ◽  
Florian Deisenhammer

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