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Clinical Rheumatology Dec 2023
Topics: Humans; Tonic Pupil; Sjogren's Syndrome
PubMed: 37518192
DOI: 10.1007/s10067-023-06707-4 -
Clinical Neurology and Neurosurgery Nov 2023Anti-contactin-associated protein-like 2 (CASPR2) is classically associated with limbic encephalitis (LE), Morvan syndrome and peripheral nerve hyperexcitability (PNH)....
INTRODUCTION
Anti-contactin-associated protein-like 2 (CASPR2) is classically associated with limbic encephalitis (LE), Morvan syndrome and peripheral nerve hyperexcitability (PNH). Additional clinical features have been previously recognized.
OBJECTIVE
To describe a cohort of patients with anti-CASPR2-associated neurological syndromes from a tertiary referral centre.
METHODS
Retrospective analysis of patients with positive serum anti-CASPR2 antibodies in the period between 2014 and 2021.
RESULTS
Nineteen patients were identified, 11 (57.9%) male, with a median age at symptom onset of 49.0 (31.3-63.0) years and a median time to diagnosis of 1.0 (0.0-1.8) years. The most common clinical syndromes were LE (7 cases, 36.8%), Morvan syndrome (4, 21.1%) and PNH (2, 10.5%). Six patients presented with atypical phenotypes (31.6%), comprising dysautonomia (orthostatic hypotension and Adie's Pupil), motor tics/stereotypies, obsessive-compulsive disorder, and brainstem involvement. The most common presenting symptoms were seizures (31.6%), PNH (21.1%) and cognitive dysfunction (15.8%). One LE patient had a disease duration of 2,5 years and was initially diagnosed with dementia. CSF was normal in most cases. Brain MRI showed temporal lobe hyperintensities in 4 LE cases (57.1%). All PNH cases had myokymic discharges of fasciculations in the electromyography. Two patients had associated thymoma and 1 had lung adenocarcinoma. Eight patients (42.1%) received treatment during the acute phase and 26.3% maintenance treatment. Approximately half of the treated cases improved or stabilised, with 4 (21.1%) deaths in the whole cohort.
CONCLUSION
Anti-CASPR2-associated neurological disorders may present with isolated atypical phenotypes, a slowly progressive clinical course, and with normal CSF or imaging findings.
Topics: Female; Humans; Male; Autoantibodies; Limbic Encephalitis; Retrospective Studies; Seizures; Syndrome
PubMed: 37797365
DOI: 10.1016/j.clineuro.2023.107994 -
Medical Journal, Armed Forces India Dec 2023A 30-year-old male patient presented to the eye department with complaints of blurring of vision of right eye at distance and near for a duration of 1.5 months. Ocular...
A 30-year-old male patient presented to the eye department with complaints of blurring of vision of right eye at distance and near for a duration of 1.5 months. Ocular examination revealed Anisocoria with enlarged pupil in the right eye. On instillation of 0.1% pilocarpine, there was a pronounced miosis in the dilated pupil seen at 30 min associated with an improvement in distance and near vision. On slit lamp examination, vermiform movements were seen in the affected pupil on shining the slit from temporal aspect. Fundus examination was within normal limits. Systemic examination revealed absent deep tendon reflexes. Based on the clinical features, a diagnosis of Holmes-Adie syndrome was reached and the patient was started on 0.1% pilocarpine eye drops. This case highlights the importance of thorough systemic examination and investigations in all cases of anisocoria.
PubMed: 38144644
DOI: 10.1016/j.mjafi.2022.01.008 -
Journal of Neuro-ophthalmology : the... Dec 2023
Topics: Humans; Tonic Pupil; Adie Syndrome; Pupil; Autoantibodies
PubMed: 35439231
DOI: 10.1097/WNO.0000000000001598 -
Acta Neurologica Taiwanica Sep 2023Ross syndrome is a rare disorder of unknown etiology, characterized by the triad of segmental anhidrosis, tonic pupil, and areflexia/hyporeflexia. Ross syndrome is...
Ross syndrome is a rare disorder of unknown etiology, characterized by the triad of segmental anhidrosis, tonic pupil, and areflexia/hyporeflexia. Ross syndrome is thought to be a limited and selective ganglioneuropathy. Its etiology has not been fully elucidated. Autonomic findings may also accompany. We wanted to present our 25-year-old patient who was diagnosed with Ross syndrome and presented with complaints of inability to sweat, heat intolerance, headache, diarrhea and chronic cough. Keyword: cough, tonic pupil, anhidrosis, compensatory.
Topics: Humans; Adult; Tonic Pupil; Hypohidrosis; Cough; Pupil Disorders; Adie Syndrome; Reflex, Abnormal
PubMed: 37674425
DOI: No ID Found -
Journal of Neuro-ophthalmology : the... Mar 2024
Topics: Humans; Tonic Pupil; BNT162 Vaccine; COVID-19 Vaccines; COVID-19; Adie Syndrome
PubMed: 36166780
DOI: 10.1097/WNO.0000000000001670 -
Journal of the European Academy of... Sep 2023
Topics: Humans; Tonic Pupil; Scleroderma, Localized; Adie Syndrome
PubMed: 37170937
DOI: 10.1111/jdv.19181 -
American Heart Journal Plus :... Feb 2024Patients who survive critical illness endure complex physical and mental health conditions, referred to as post-intensive care syndrome (PICS). The University of...
OBJECTIVE
Patients who survive critical illness endure complex physical and mental health conditions, referred to as post-intensive care syndrome (PICS). The University of Michigan's post-intensive cardiac care outpatient long-term outreach (PICCOLO) clinic is designed for patients recently admitted to the coronary care unit (CCU). The long-term goal of this clinic is to understand post-CCU patients' needs and design targeted interventions to reduce their morbidity and mortality post-discharge. As a first step toward this goal, we aimed to define the post-discharge needs of CCU survivors.
DESIGN SETTING PARTICPANTS
We retrospectively reviewed case-mix data (including rates of depression, PTSD, disability, and cognitive abnormalities) and health outcomes for patients referred to the PICCOLO clinic from July 1, 2018, through June 30, 2021 at Michigan Medicine.
RESULTS
Of the 134 referred patients meeting inclusion criteria, 74 (55 %) patients were seen in the PICCOLO clinic within 30 days of discharge. Patients seen in the clinic frequently screened positive for depression (PHQ-2 score ≥3, 21.4 %) and cognitive impairment (MOCA 26, 38.8 %). Further, patients also reported high rates of physical difficulty (mean WHODAS 2.0 score 28.4 %, consistent with moderate physical difficulty). Consistent with medical intensive care unit (ICU) patients, CCU survivors experience PICS.
CONCLUSION
This work highlights the feasibility of an outpatient care model and the need to leverage information gathered from this care model to develop treatment strategies and pathways to address symptoms of PICS in CCU survivors, including depression, cognitive impairment, and physical disability.
PubMed: 38434252
DOI: 10.1016/j.ahjo.2024.100363