scholarly journals General Paresis in a Child with Unusual Presentation

2020 ◽  
Vol 18 (2) ◽  
Author(s):  
Muhamad Azamin Anuar ◽  
Wan Alina Khadijah ◽  
Asrar Abu Bakar ◽  
Mossad Abdelhak Shaban Mohamed ◽  
Taufiq Hidayat Hasan
Keyword(s):  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathy ◽  
Access Point ◽  
Acute Onset ◽  
Recurrent Tonsillitis ◽  
Tertiary Centre ◽  
Paravertebral Muscles ◽  
Normal Tone ◽  
Autoimmune Conditions ◽  
History Of

Introduction: Acute onset quadriparesis can be a manifestation of a variety of neurological, metabolic or autoimmune conditions. Rarely, it could be one of many clinical presentations of juvenile dermatomyositis which belongs to the group of idiopathic inflammatory myopathies of childhood. We report the case of a 9-year old girl who presented with global myopathy over a two-month period. Case report: A 9-year-old girl referred with a 2 months history of generalised muscular pain and weakness. There were no significant history of preceded illness, trauma or excessive strenuous exercises. She had no other systemic complaints such as fever or skin rash. Past medical history was unremarkable except for recurrent tonsillitis. Physical examination revealed a child with normal alertness and behaviour. She has notable generalised swelling of all four limbs. Her neurological examination revealed normal tone however her power was definitely reduced in all four limbs (Graded 3/5). She demonstrated signs of proximal myopathy. Subsequent investigations revealed high Creatinine Kinase (CK) levels of 6470U/L, ESR 84mm/hr with deranged transaminases and positive serum mycoplasma and CMV IgM. Her MRI brain and spine along with lumbar puncture results were normal. She was referred to tertiary centre for further evaluation as her weakness progressed. An MRI muscle demonstrated marked inflammation in all four limbs including paravertebral muscles. Her muscle biopsy showed inflammatory myopathy leading to a diagnosis of juvenile dermatomyositis (JDM). She is now showing sustained clinical improvements following a course of immunoglobulin and corticosteroids therapy. Discussion: The diagnosis of JDM is generally considered in patients with rash-associated muscle weakness. Essentially, it involves assessment of muscle, skin, lung and cardiac involvement on top of baseline list of investigations that has been outline by Single Hub and Access point for pediatric Rheumatology in Europe (SHARE). This case reflects that JDM is possible despite non-existent of skin involvement.  

scholarly journals Rare occurrence of central diabetes insipidus with dermatomyositis in a young male

2020 ◽  
Vol 2020 ◽  
Author(s):  
Aishah Ekhzaimy ◽  
Afshan Masood ◽  
Seham Alzahrani ◽  
Waleed Al-Ghamdi ◽  
Daad Alotaibi ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Inflammatory Myopathy ◽  
Young Male ◽  
Central Diabetes Insipidus ◽  
Endocrine Disorders ◽  
Extensive Literature ◽  
List Type ◽  
Autoimmune Conditions ◽  
History Of ◽  
Autoimmune Condition

Summary Central diabetes insipidus (CDI) and several endocrine disorders previously classified as idiopathic are now considered to be of an autoimmune etiology. Dermatomyositis (DM), a rare autoimmune condition characterized by inflammatory myopathy and skin rashes, is also known to affect the gastrointestinal, pulmonary, and rarely the cardiac systems and the joints. The association of CDI and DM is extremely rare. After an extensive literature search and to the best of our knowledge this is the first reported case in literature, we report the case of a 36-year-old male with a history of CDI, who presented to the hospital’s endocrine outpatient clinic for evaluation of a 3-week history of progressive facial rash accompanied by weakness and aching of the muscles. Learning points: Accurate biochemical diagnosis should always be followed by etiological investigation. This clinical entity usually constitutes a therapeutic challenge, often requiring a multidisciplinary approach for optimal outcome. Dermatomyositis is an important differential diagnosis in patients presenting with proximal muscle weakness. Associated autoimmune conditions should be considered while evaluating patients with dermatomyositis. Dermatomyositis can relapse at any stage, even following a very long period of remission. Maintenance immunosuppressive therapy should be carefully considered in these patients.

Z-line degradation and phagocytosis of sarcomeric fragments in myonecrosis

1983 ◽  
Vol 41 ◽  
pp. 790-791
Author(s):  
Melinda L. Estes ◽  
Samuel M. Chou
Keyword(s):  
Vastus Lateralis ◽  
Inflammatory Myopathy ◽  
Muscle Disease ◽  
White Female ◽  
Inflammatory Cell Infiltrate ◽  
Limb Weakness ◽  
Muscle Diseases ◽  
History Of ◽  
The Right ◽  
Mononuclear Inflammatory Cell

Many muscle diseases show common pathological features although their etiology is different. In primary muscle diseases a characteristic finding is myofiber necrosis. The mechanism of myonecrosis is unknown. Polymyositis is a primary muscle disease characterized by acute and subacute degeneration as well as regeneration of muscle fibers coupled with an inflammatory infiltrate. We present a case of polymyositis with unusual ultrastructural features indicative of the basic pathogenetic process involved in myonecrosis.The patient is a 63-year-old white female with a one history of proximal limb weakness, weight loss and fatigue. Examination revealed mild proximal weakness and diminished deep tendon reflexes. Her creatine kinase was 1800 mU/ml (normal < 140 mU/ml) and electromyography was consistent with an inflammatory myopathy which was verified by light microscopy on biopsy muscle. Ultrastructural study of necrotizing myofiber, from the right vastus lateralis, showed: (1) degradation of the Z-lines with preservation of the adjacent Abands including M-lines and H-bands, (Fig. 1), (2) fracture of the sarcomeres at the I-bands with disappearance of the Z-lines, (Fig. 2), (3) fragmented sarcomeres without I-bands, engulfed by invading phagocytes, (Fig. 3, a & b ), and (4) mononuclear inflammatory cell infiltrate in the endomysium.

Omental Infarction: A Case Treated with Laparoscopic Surgery

2019 ◽  
pp. 1-3
Author(s):  
Bertrand Ng ◽  
Arafat Yasser
Keyword(s):  
Diabetes Mellitus ◽  
Laparoscopic Surgery ◽  
Hospital Stay ◽  
Acute Cholecystitis ◽  
Inflammatory Markers ◽  
Acute Onset ◽  
Laparoscopy Surgery ◽  
History Of ◽  
Background History ◽  
The Right

Omental infarct is a rare cause of an acute abdomen that arises from an interruption of blood supply to the omentum. Here, we present a case of omental infarct in a 67-year-old gentleman with background history of diabetes mellitus who present unusually with a severe acute onset right hypochondrium pain. Examination revealed that he was tender to touch at the right and was having localized guarding. His inflammatory markers were normal. He was successfully treated with laparoscopy surgery and he was subsequently discharged the following day. Omental infarct cases with right hypochondrium pain can sometimes mimicked acute cholecystitis and management includes laparoscopic surgery which can hasten symptoms resolution and reduces hospital stay, however recommendation for surgery has to be balanced with anesthetics risk and complication of the surgery itself.

Young Male with Bilateral ICA Dissection: Beyond CADISS Trial

2021 ◽  
pp. 251660852098428
Author(s):  
Vikas Bhatia ◽  
Chirag Jain ◽  
Sucharita Ray ◽  
jay Kumar
Keyword(s):  
Management Strategies ◽  
Young Male ◽  
Acute Onset ◽  
The Body ◽  
Artery Dissection ◽  
Cervical Artery Dissection ◽  
Stroke Study ◽  
History Of

Objective: To report a case of young male with stroke and bilateral internal carotid artery (ICA) dissection. Background: Cervical Artery Dissection in Stroke Study trial has provided some insight on management of patients with ICA dissection. However, there is a need to modify the management strategies as per specific clinical scenario. Design/Methods: Case report and literature review. Results: A 45-year-old male presented with 1 month old history of acute onset numbness of right half of the body with slurring of speech. Computed tomography angiography showed complete occlusion of left cervical ICA just beyond origin with presence of fusiform dilatation and spiral flap in right extracranial cervical ICA. The patient was started on antiplatelets and taken for endovascular procedure using 2-mesh-based carotid stents. Patient was discharged after 3 days on antiplatelet therapy. At 1-year follow-up, there were no fresh symptoms. Conclusion: This case emphasizes the role of successful endovascular management of carotid dissection in a young male. These clinical situations may not be fully represented in trials, and a case-based approach is required.

scholarly journals Bone mineral density and explanatory factors in children and adults with juvenile dermatomyositis at long term follow-up; a cross sectional study

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Henriette Schermacher Marstein ◽  
Kristin Godang ◽  
Berit Flatø ◽  
Ivar Sjaastad ◽  
Jens Bollerslev ◽  
...  
Keyword(s):  
Bone Turnover ◽  
Inflammatory Markers ◽  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathy ◽  
Disease Onset ◽  
Whole Body ◽  
Mineral Density ◽  
Z Scores

Abstract Background Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children and adolescents. Both the disease and its treatment with glucocorticoids may negatively impact bone formation. In this study we compare BMD in patients (children/adolescence and adults) with long-standing JDM with matched controls; and in patients, explore how general/disease characteristics and bone turnover markers are associated with BMD. Methods JDM patients (n = 59) were examined median 16.8y (range 6.6–27.0y) after disease onset and compared with 59 age/sex-matched controls. Dual-energy X-ray absorptiometry (DXA) was used to measure BMD of the whole body and lumbar spine (spine) in all participants, and of ultra-distal radius, forearm and total hip in participants ≥20y only. Markers of bone turnover were analysed, and associations with outcomes explored. Results Reduced BMD Z-scores (<−1SD) were found in 19 and 29% of patients and 7 and 9% of controls in whole body and spine, respectively (p-values < 0.05). BMD and BMD Z-scores for whole body and spine were lower in all patients and for < 20y compared with their respective controls. In participants ≥20y, only BMD and BMD Z-score of forearm were lower in the patients versus controls. In patients, BMD Z-scores for whole body and/or spine were found to correlate negatively with prednisolone use at follow-up (yes/no) (age < 20y), inflammatory markers (age ≥ 20y) and levels of interferon gamma-induced protein 10 (IP-10) (both age groups). In all patients, prednisolone use at follow-up (yes/no) and age ≥ 20y were independent correlates of lower BMD Z-scores for whole body and spine, respectively. Conclusion In long-term JDM, children have more impairment of BMD than adults in spine and whole-body. Associations with BMD were found for both prednisolone and inflammatory markers, and a novel association was discovered with the biomarker of JDM activity, IP-10.

scholarly journals CNS demyelination associated with immune dysregulation and a novel CTLA-4 variant

2021 ◽  
pp. 135245852096389
Author(s):  
Stefania Kaninia ◽  
Alexandros Grammatikos ◽  
Kathryn Urankar ◽  
Shelley A Renowden ◽  
Nikunj K Patel ◽  
...  
Keyword(s):  
T Cell Activation ◽  
Cell Activation ◽  
Cytotoxic T Lymphocyte ◽  
Checkpoint Inhibitors ◽  
Novel Treatments ◽  
First Case ◽  
Self Tolerance ◽  
Autoimmune Conditions ◽  
History Of ◽  
Cns Demyelination

Background: The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T-cell activation and promotes self-tolerance. Case: We report the first case of biopsy-proven central nervous system inflammatory demyelination in the context of primary immunodeficiency and a novel CTLA-4 variant. Conclusion: This case has significant implications for the development of novel treatments for autoimmune conditions including multiple sclerosis and further emphasises the need for caution with clinical use of CTLA-4 immune checkpoint inhibitors in those with a history of inflammatory demyelination.

scholarly journals A Rare Case of Spontaneous Arachnoid Cyst Rupture Presenting as Right Hemiplegia and Expressive Aphasia in a Pediatric Patient

Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 78
Author(s):  
Anne Bryden ◽  
Natalie Majors ◽  
Vinay Puri ◽  
Thomas Moriarty
Keyword(s):  
Cognitive Processing ◽  
Arachnoid Cyst ◽  
Memory Loss ◽  
Acute Onset ◽  
Word Finding ◽  
Outpatient Visits ◽  
The Family ◽  
History Of ◽  
Cyst Rupture ◽  
The Brain

This study examines an 11-year-old boy with a known history of a large previously asymptomatic arachnoid cyst (AC) presenting with acute onset of right facial droop, hemiplegia, and expressive aphasia. Shortly after arrival to the emergency department, the patient exhibited complete resolution of right-sided hemiplegia but developed headache and had persistent word-finding difficulties. Prior to symptom onset while in class at school, there was an absence of reported jerking movements, headache, photophobia, fever, or trauma. At the time of neurology consultation, the physical exam showed mildly delayed cognitive processing but was otherwise unremarkable. The patient underwent MRI scanning of the brain, which revealed left convexity subdural hematohygroma and perirolandic cortex edema resulting from ruptured left frontoparietal AC. He was evaluated by neurosurgery and managed expectantly. He recovered uneventfully and was discharged two days after presentation remaining asymptomatic on subsequent outpatient visits. The family express concerns regarding increased anxiety and mild memory loss since hospitalization.

scholarly journals A two-year retrospective study of infants with Erb-Duchenne's palsy at a tertiary centre in Rajasthan, India

2018 ◽  
Vol 7 (2) ◽  
pp. 700
Author(s):  
Rakesh Appasaheb Hasabe ◽  
Dnyaneshwar S. Diwane ◽  
Sushant S. Chandawar
Keyword(s):  
Brachial Plexus Injury ◽  
Single Mother ◽  
Shoulder Dystocia ◽  
Fetal Macrosomia ◽  
Live Births ◽  
Tertiary Centre ◽  
Vaginal Deliveries ◽  
History Of ◽  
The Mean ◽  
Care History

Background: Brachial plexus injury (BPI) has prevalence of between 0.5 and 4.4/1000 live births. It is commonly believed to be attributed to extensive lateral traction during difficult neck delivery. This paper aims to look at one aspect of birth injuries, Erb-Duchenne palsy, its incidence and contributing factors.Methods: All infants sustaining Erb-Duchenne palsy during birth were identified at Department of OBGY, NIMS Hospital, Jaipur. The notes of the infants and mothers were reviewed. Special attention was given to the known risk factors for birth trauma such as ethnicity, parity, antenatal care, history of diabetes etc.Results: During the two-year period 2013 to 2015, there were 6 infants diagnosed with Erb-Duchenne palsy, giving an incidence of 0.79 in 1000 live births. Out of six, four were primigravidae. All pregnancies were singleton, cephalic/vertex undergoing vaginal deliveries; none required forceps or ventouse deliveries. The mean infant birthweight was 4.378 kg with a median value of 4.48 kg (range 3.51-4.78). Four infants were classified as macrosomic i.e. birthweights greater than 4 kg, four infants had birthweights of 4 kg to 5 kg; none weighed over 5 kg. Three of the deliveries were documented as difficult shoulder delivery/shoulder dystocia. Five of the infants had APGAR scores that were less than seven.Conclusions: This study suggested that Erb-Duchenne palsy is strongly associated with fetal macrosomia and shoulder dystocia. Diabetes was not significant in causing macrosomia as only single mother was found diabetic. Fetal macrosomia contributed to shoulder dystocia in the majority of cases.

scholarly journals Attributes of turnaround rural hospitals: Case study findings and research opportunities

2017 ◽  
Vol 6 (6) ◽  
pp. 8
Author(s):  
Asa B. Wilson
Keyword(s):  
Theory Of The Firm ◽  
Access Point ◽  
Hospital Performance ◽  
Rural Hospitals ◽  
Rural Hospital ◽  
Classical Economics ◽  
The Status ◽  
History Of ◽  
Closure Conversion ◽  
The Impact

Background: Rural and Critical Access Hospitals (CAHs) have a history of operating challenges and closure-conversion threats. The history is reviewed including the supportive public policy provisions and administrative tactics designed to maintain a community’s hospital as the hub and access point for health services. Limited research indicates that rural facilities are not strategic in their responses to challenges. A question emerges regarding the enduring nature of operating difficulties for these facilities, i.e., no understanding with explanatory value.Objective: The author, as the CEO in six rural hospitals designated as turnaround facilities, used inductive participant-observer involvement to identify operating attributes characteristic of these organizations. An objective description of each facility is provided. While implementing a turnaround intervention, fifteen behaviors or outcomes were found to be consistent across all six entities. This information is used to posit factors associated with or accounting for identified performance weaknesses.Conclusions: It is conceptualization that observed organizational behaviors can be explained as remnants of an agrarian ideology. Such a mindset is focused on preserving the status quo despite challenges that would require strategic positioning of the organization. In addition, emerging studies on community types indicates that follow-up research is needed that assesses the impact of community attributes on rural hospital performance. Also, this study shows that a theory of the rural hospital firm based on neo-classical economics has no explanatory value. Thus, a theory of the firm can be developed that includes behavioral economic principles.

scholarly journals Spontaneous Uterine Rupture in a Preterm Pregnancy following Myomectomy

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Claire Sutton ◽  
Prue Standen ◽  
Jade Acton ◽  
Christopher Griffin
Keyword(s):  
Abdominal Pain ◽  
Caesarean Section ◽  
Uterine Rupture ◽  
Good Condition ◽  
Acute Onset ◽  
Uterine Wall ◽  
Unusual Presentation ◽  
Postoperative Course ◽  
Spontaneous Uterine Rupture ◽  
History Of

A 44-year-old nulliparous woman was transferred to a tertiary obstetric hospital for investigation of acute onset abdominal pain. She was at gestation of 32 weeks and 2 days with a history of previous laparoscopic fundal myomectomy. An initial bedside ultrasound demonstrated oligohydramnios. Following an episode of increased pain early the following morning, a formal ultrasound diagnosed a uterine rupture with the fetal arm extending through a uterine rent. An uncomplicated classical caesarean section was performed and the neonate was delivered in good condition but with a bruised and oedematous right arm. The neonate was transferred to the Special Care Nursery for neonatal care. The patient had an uncomplicated postoperative course and was discharged home three days following delivery. This is an unusual presentation of uterine rupture following myomectomy where the fetal arm had protruded through the uterine wall.

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