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Scientific Reports Jan 2020Intrinsically photosensitive retinal ganglion cells (ipRGCs) control non-visual light responses (e.g. pupillary light reflex and circadian entrainment). Patients with...
BACKGROUND
Intrinsically photosensitive retinal ganglion cells (ipRGCs) control non-visual light responses (e.g. pupillary light reflex and circadian entrainment). Patients with diabetic retinopathy (DR) show reduced ipRGC function, as inferred by abnormalities in the post illumination pupil response (PIPR). We explored whether ipRGC function in DR is associated with circadian outputs and sleep/wake behavior.
METHODS
Forty-five participants (15 without diabetes, 15 with type 2 diabetes (T2D) and no DR, 15 with T2D and DR) participated. ipRGC function was inferred from the PIPR (pupil size following stimulus offset). Circadian outputs were melatonin amplitude (overnight urinary 6-sulfatoxymelatonin (aMT6s)) and timing (dim light melatonin onset (DLMO)), and evening salivary cortisol levels. Sleep/wake patterns were measured with wrist actigraphy and insomnia symptoms were assessed subjectively.
RESULTS
Patients with T2D and DR had smaller PIPR and lower urinary aMT6s than other groups (p < 0.001). In adjusted regression models, smaller PIPR was associated with lower urinary aMT6s (β = 4.552, p = 0.005). Patients with DR were more likely to have no detectable DLMO (p = 0.049), higher evening salivary cortisol, greater insomnia symptoms and greater sleep variability compared to other groups. Sleep duration, efficiency and rest-activity rhythms were similar.
CONCLUSION
Reduced ipRGC function in DR is associated with circadian dysregulation and sleep disturbances, although a causal relationship cannot be established in this cross-sectional study. Prospective mechanistic and intervention studies examining circadian and sleep health in these patients are warranted.
Topics: Adie Syndrome; Aged; Cells, Cultured; Circadian Clocks; Cross-Sectional Studies; Diabetes Mellitus, Type 2; Diabetic Retinopathy; Female; Humans; Hydrocortisone; Male; Melatonin; Middle Aged; Reflex, Pupillary; Retinal Ganglion Cells; Sleep Disorders, Circadian Rhythm; Sleep Initiation and Maintenance Disorders
PubMed: 32005914
DOI: 10.1038/s41598-020-58205-1 -
BMJ Case Reports Jan 2020A 40-year-old healthy man presented with a 4-month history of photophobia, blurred vision and a right dilated pupil. Examination revealed a right pupil that was not...
A 40-year-old healthy man presented with a 4-month history of photophobia, blurred vision and a right dilated pupil. Examination revealed a right pupil that was not reactive to light but constricted strongly to a near target and slowly redilated when he looked back in the distance. Pharmacological testing with dilute pilocarpine 0.1% resulted in constriction of the right pupil but no change in the left pupil. This also resulted in resolution of his photophobia and blurry vision. Neurological examination was otherwise normal, and a diagnosis of Adie's tonic pupil was made. The main differential diagnosis to consider for a large pupil is a third nerve palsy, pharmacological mydriasis, tonic pupil and local iris processes, such as iris sphincter tears from trauma. Knowledge of the key features of these conditions can avoid wasted resources from unnecessary testing. Dilute pilocarpine 0.1% three times a day can be considered as a pharmacological therapy for symptomatic relief.
Topics: Adie Syndrome; Adult; Diagnosis, Differential; Humans; Male; Photophobia; Pilocarpine; Tonic Pupil
PubMed: 31900299
DOI: 10.1136/bcr-2019-233136 -
Frontiers in Neurology 2019We report a woman with unilateral Adie's pupil associated with peripheral sensorimotor polyneuropathy, elevated anti-Hu antibody, and primary mediastinal small cell...
We report a woman with unilateral Adie's pupil associated with peripheral sensorimotor polyneuropathy, elevated anti-Hu antibody, and primary mediastinal small cell carcinoma (SCC). To our knowledge, this is the first report of Adie's pupil in a patient with mediastinal SCC. Although rare, Adie's pupil and sensorimotor polyneuropathy may be the first manifestation of cancer. Its rapid recognition facilitates an early diagnosis and treatment.
PubMed: 31849812
DOI: 10.3389/fneur.2019.01236 -
Journal of Orthopaedic Surgery (Hong... 2020The primary objective of this review was to determine whether tranexamic acid (TXA) reduces transfusion rates in patients undergoing surgery for hip fractures. The... (Meta-Analysis)
Meta-Analysis
AIMS
The primary objective of this review was to determine whether tranexamic acid (TXA) reduces transfusion rates in patients undergoing surgery for hip fractures. The secondary objective was to assess the effects of TXA on mortality and thromboembolic events in the same cohort.
METHODS
A systematic review of electronic databases was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We included randomized controlled trials comparing perioperative TXA in patients treated surgically for hip/proximal femoral fractures against placebo. The primary outcome was the proportion of patients requiring blood transfusion. Secondary outcomes were blood loss, mortality, and complications. Meta-analysis was performed using inverse variance and random effects model.
RESULTS
The pooled data from 10 studies involving 842 patients showed that the proportion of patients requiring blood transfusion was significantly less in the TXA group (risk ratio (RR) 0.72, 95% confidence interval (CI) 0.59-0.88). There was no difference between TXA and control groups when comparing mortality (RR 1.17, 95% CI 0.65-2.10), deep venous thrombosis (RR 1.14, 95% CI 0.43-3.06), pulmonary embolism (RR 0.53, CI 0.09-3.02), acute coronary syndrome (RR 1.52, CI 0.18-12.98), cerebrovascular events (RR 0.78, CI 0.16-3.68), or wound complications (RR 1.61, CI 0.51-5.13).
CONCLUSION
There is evidence that TXA reduces the proportion of patients requiring blood transfusions when undergoing hip fracture surgery. However, the small sample size and low event rates for adverse effects preclude any definitive conclusions from being established regarding adverse effects. Future trials should be powered to further assess potential complications and determine the ideal dosage and regime.
Topics: Antifibrinolytic Agents; Blood Loss, Surgical; Blood Transfusion; Hip Fractures; Humans; Tranexamic Acid
PubMed: 31835969
DOI: 10.1177/2309499019887995 -
Vestnik Oftalmologii 2019Disorders and abnormalities of pupil reactions comprise important part in the clinical practice of both ophthalmologists and neurologists. The present article presents a...
Disorders and abnormalities of pupil reactions comprise important part in the clinical practice of both ophthalmologists and neurologists. The present article presents a historical perspective on one of such pathologies - Adie syndrome, and discusses its etiology, pathogenesis and clinical symptomatology. The article also describes a clinical case of one patient with comorbidity.
Topics: Adie Syndrome; Humans
PubMed: 31573561
DOI: 10.17116/oftalma201913504183 -
Rinsho Shinkeigaku = Clinical Neurology Oct 2019We describe a 60-year-old woman with medically refractory left mesial temporal lobe epilepsy accompanied by Ross syndrome. The patient had a partial triad of Ross...
We describe a 60-year-old woman with medically refractory left mesial temporal lobe epilepsy accompanied by Ross syndrome. The patient had a partial triad of Ross syndrome with hypohydrosis only on her right side (contralateral to the epileptic seizure focus), Adie's tonic pupil on the right, and areflexia while her seizures used to be medically refractory. However, her hypohidrosis and Adie's tonic pupil have completely changed in terms of laterality following nearly complete seizure freedom resutling from left temporal lobectomy. This unique change in laterality in Ross syndrome is most likely caused by remote effects of the near-absent epileptic acitivity, and it also may contribute to understanding the pathophysiological mechanism of Ross syndrome.
Topics: Drug Resistant Epilepsy; Epilepsies, Partial; Epilepsy, Temporal Lobe; Female; Functional Laterality; Humans; Hypohidrosis; Middle Aged; Neurosurgical Procedures; Postoperative Complications; Reflex, Abnormal; Syndrome; Temporal Lobe; Tonic Pupil
PubMed: 31564703
DOI: 10.5692/clinicalneurol.cn-001315 -
The Turkish Journal of Pediatrics 2019Demirsoy U, Alparslan B, Şen MC, Anık Y, Akansel G, Görür G, Gürel B, Aksu G, Çorapçıoğlu F. More than Ophelia syndrome: Multiple paraneoplastic syndromes in...
Demirsoy U, Alparslan B, Şen MC, Anık Y, Akansel G, Görür G, Gürel B, Aksu G, Çorapçıoğlu F. More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma. Turk J Pediatr 2019; 61: 139-141. Paraneoplastic syndromes in Hodgkin lymphoma (HL) can be seen with different symptoms and organ findings within a significant time before definite diagnosis of the primary disease. Achalasia, Holmes-Adie pupil, and limbic encephalitis are rarely reported paraneoplastic components in pediatric HL. In this report, we present an 11-year-old girl who had all these three paraneoplastic components synchronously before HL was identified.
Topics: Adie Syndrome; Child; Esophageal Achalasia; Female; Hodgkin Disease; Humans; Limbic Encephalitis; Paraneoplastic Syndromes
PubMed: 31559737
DOI: 10.24953/turkjped.2019.01.024 -
Journal of Family Medicine and Primary... Apr 2019Ross syndrome is diagnosed by the presence of the characteristic triad of segmental anhidrosis, depressed deep tendon reflex, and tonic pupils. It is a rare,...
Ross syndrome is diagnosed by the presence of the characteristic triad of segmental anhidrosis, depressed deep tendon reflex, and tonic pupils. It is a rare, misdiagnosed autonomic disorder with less than 80 cases reported in the world literature. Two representative cases of Ross syndrome are presented with their laboratory correlates and relevant review of literature. Both cases (aged 35 and 58) presented with complaint of decreased sweating over one half of the face and ipsilateral upper limb and trunk and contralateral lower limb. There was compensatory increased sweating and hyperpigmentation over the remaining parts of the body. The duration of symptoms was 2 years and 15 days. The patients had variegated skin color as per the above distribution and hyporeflexia in lower limbs. One patient also had Holmes-Adie pupil. Iodine test showed hypohidrosis in the described areas, which was confirmed by skin biopsy in both cases. The patients were treated symptomatically with incomplete relief. The authors aim to highlight this rare disorder that can be one of the causes of pathological sweating encountered in general practice and the challenges in its management.
PubMed: 31143750
DOI: 10.4103/jfmpc.jfmpc_151_19 -
Neurological Sciences : Official... Aug 2019Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neuropathy which affects mainly large myelinated axons and has a typically mild autonomic dysfunction...
BACKGROUND
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neuropathy which affects mainly large myelinated axons and has a typically mild autonomic dysfunction mainly from postganglionic nerve fiber involvement.
CASE REPORT
We report here an acute onset CIDP initially diagnosed as Guillain-Barré syndrome (GBS), unresponsive to treatment with intravenous immunoglobulin (IVIg), which later responded to plasmapheresis and corticoids. The patient had a markedly distal demyelination, prominent cranial nerve involvement and, interestingly, bilateral fixed dilated pupils. Despite complete clinical recovery, this neurological sign remained.
CONCLUSIONS
Tonic pupils have previously been described in different neurologic conditions, including GBS, but not yet in acute onset CIDP or in variants with predominantly distal demyelination. It differs from the classical Adie's pupil because it lacks the light-near dissociation. This case report expands the range of possible autonomic signs in acute onset CIDP, which could help physicians establish optimal treatment strategies earlier on.
Topics: Diagnostic Errors; Female; Guillain-Barre Syndrome; Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Tonic Pupil; Young Adult
PubMed: 30997592
DOI: 10.1007/s10072-019-03890-8 -
The Canadian Journal of Neurological... Mar 2019Lewis-Sumner syndrome (LSS) is a demyelinating peripheral neuropathy described in 1982.
INTRODUCTION
Lewis-Sumner syndrome (LSS) is a demyelinating peripheral neuropathy described in 1982.
METHODS
We reviewed the charts of nine LSS patients in neurological care for their symptoms, response to different treatment regimens, and pattern of nerve involvement.
RESULTS
One patient had an Adie's pupil. Every patient studied had median nerve involvement. Seven of nine patients required intravenous immunoglobulin (IVIg) therapy and all showed improvement with IVIg. Four of nine patients received oral steroid therapy and had some improvement. Two of nine patients received azathioprine to little effect. Two of nine patients experienced significant trauma while receiving neurological follow-up and their symptoms worsened to a clinically significant degree afterward.
DISCUSSION
We noticed a possible association between trauma and symptom severity in cases of LSS with preexisting neurological follow-up. We hypothesize that physical trauma exacerbates LSS. To our knowledge, this is an unreported phenomenon.
Topics: Adult; Electrodiagnosis; Female; Humans; Immunoglobulins, Intravenous; Male; Middle Aged; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Severity of Illness Index; Wounds and Injuries
PubMed: 30724154
DOI: 10.1017/cjn.2018.389