scholarly journals Tuberous sclerosis complex associated neuropsychiatric disorder in children: experience sharing from a tertiary care hospital

2021 ◽  
Vol 8 (6) ◽  
pp. 961
Author(s):  
Kanij Fatema ◽  
Mizanur Rahman ◽  
Shaheen Akhter ◽  
Roushan Jahan

Background: Tuberous sclerosis complex (TSC) is a genetic disorder where there is multisystem involvement. Most important manifestations are neurological and psychiatric disorders. These disorders should be detected timely and addressed adequately. The common psychiatric disorders are autism spectrum disorder (ASD), attention deficit hyperactivity disorder (ADHD), intellectual disability (ID), learning disorder etc. This study has been done to describe the pattern of psychiatric disorders in children with TSC.Methods: This is an observational study taken place in a tertiary care hospital taken place on Children of 0-18 years of age. The study subjects were 84 patients with TSC. Detail history and physical examination had been done along with neuroimaging, EEG and target organs screening. Different psychometric tool was used for psychiatric evaluation.  Results: Total 84 patients were included in this study, mean age was 7+3.96 years, 54% were female. Physical finding were as follows: ash leaf spot, shagreen patch, adenoma sebaceum, cafe au lait spot, rhabdomyoma, renal cyst and angiomyolipoma etc.  Seventy patients had epilepsy, most common being focal epilepsy (45.7%), 17.1% had epileptic spasm. Fifty percent patients had developmental delay. Regarding psychiatric disorders, most common disorder was ADHD in 27.38%, ASD in 23.81% and both in 10.71%. ID was found in 20.24% study subjects. Early onset of seizure was associated with more psychiatric disorders.Conclusions: Neuropsychiatric manifestations of TSC are diverse and often are poorly addressed. The most commonly found disorders in this study were ADHD, ASD and ID. Early onset of seizure was associated with the psychiatric disorders in TSC.

2013 ◽  
Vol 2 (1) ◽  
pp. 10-13 ◽  
Author(s):  
Ajay Risal ◽  
Pushpa Prasad Sharma ◽  
Rajkumar Karki

Background and Aims- Self-poisoning is the commonest mode of suicide in our part of the world. Patients attempting suicide by self-poisoning usually land up in the Emergency. They are admitted for management of medical complications and subsequently referred to Psychiatry for evaluation of possible Psychiatric illnesses. The aim of this study was to explore the patterns of psychiatric illnesses in the patients admitted for self-poisoning in a tertiary care center in Nepal. Methods- The study population included those patients who were admitted and being managed for self-poisoning and brought for psychiatric evaluation during the period of one year (1st June 2011- 30th June 2012) at Dhulikhel Hospital, Nepal. Each patient underwent a detailed psychiatric evaluation by a consultant psychiatrist once they were medically stable. Details including sociodemographic data, psychiatric diagnosis, and treatment offered and outcome was tabulated and analyzed using SPSS-16. Results- Among the total patients (N=100), 43 were in the age group 21-40 years, median age being 27.5. There was almost equal gender distribution. Majority was of Mongolian ethnicity, homemaker by occupation and married. More than 90% were single-attempters, suicidal attempt using organophosphorus compounds. Almost 50% had depression; family dispute (19%) and marital disharmony (17%) were the most common psychosocial precipitant. Conclusion- Patients with history of self-poisoning are commonly brought to the Emergency Department of any tertiary care hospital. It is widely prevalent on younger age group. It is usually by the use of Organophosphorus compound in our setting and most commonly associated with depression. Hence, psychiatric care is essential for these patients. Journal of Advances in Internal Medicine 2013;02(01):10-13 DOI: http://dx.doi.org/10.3126/jaim.v2i1.7630


2019 ◽  
Vol 30 (4) ◽  
pp. 2199-2214
Author(s):  
Benoit Scherrer ◽  
Anna K Prohl ◽  
Maxime Taquet ◽  
Kush Kapur ◽  
Jurriaan M Peters ◽  
...  

Abstract Tuberous sclerosis complex (TSC) is a rare genetic disorder characterized by benign tumors throughout the body; it is generally diagnosed early in life and has a high prevalence of autism spectrum disorder (ASD), making it uniquely valuable in studying the early development of autism, before neuropsychiatric symptoms become apparent. One well-documented deficit in ASD is an impairment in face processing. In this work, we assessed whether anatomical connectivity patterns of the fusiform gyrus, a central structure in face processing, capture the risk of developing autism early in life. We longitudinally imaged TSC patients at 1, 2, and 3 years of age with diffusion compartment imaging. We evaluated whether the anatomical connectivity fingerprint of the fusiform gyrus was associated with the risk of developing autism measured by the Autism Observation Scale for Infants (AOSI). Our findings suggest that the fusiform gyrus connectivity captures the risk of developing autism as early as 1 year of age and provides evidence that abnormal fusiform gyrus connectivity increases with age. Moreover, the identified connections that best capture the risk of developing autism involved the fusiform gyrus and limbic and paralimbic regions that were consistent with the ASD phenotype, involving an increased number of left-lateralized structures with increasing age.


2019 ◽  
Vol 43 (1) ◽  
pp. 15-20 ◽  
Author(s):  
Gopen Kumar Kundu ◽  
Sanjida Ahmed ◽  
Shaheen Akhter ◽  
Md Nasir Hossain ◽  
Rana Kumar Biswas

Background: Neurocutaneous syndromes (NCS) represent a group of central nervous system disorders associated with lesion in the skin, eye and possibly other visceral organs. Various cutaneous manifestations usually appear early in life, progress with time but neurological features generally present at a later age. The objective of this study is to observe the frequency of pattern and presentation of various forms of neurocutaneous syndrome. Methodology: A descriptive cross sectional study was conducted at department of Paediatric Neurology of Bangabandhu Sheikh Mujib Medical University(BSMMU), Dhaka, Bangladesh from March, 2015 to February, 2016. Children (3month to 8 years) with NCS, diagnosed on the basis of standard diagnostic criteria for different NCS were included and compared. Results: Among of 27 children,16 (59.26%) boys, 09(40.74%) girls with neurocutaneous syndrome, mean age of presentation was 38.67±18.57 (range 3 month to 84 months). The various forms of NCS observed were Tuberous sclerosis complex (48.14%), Ataxia telangiectasia (29.62%), Sturge Weber Syndrome (14.81%), Neurofibromatosis1(3.7%) and Linear nevus syndrome (3.7%). In Tuberous sclerosis complex , most common feature was facial angiofibroma ( 92.30%), Hypomelanotic macule (76.90%), Shagreen patch (76.90%). In Ataxia telangiectasia, commonest presentation was ataxia 8(100%) followed by ocular telangiectasia (62.50%). In Sturge weber syndrome, commonest presentation was facial capillary malformation (100%). Neurofibromatosis patient presented with café-au-lait spot (100%). Common systemic manifestations of NCS were found in TSC and those were multicystic kidney disease (30.76%), autism spectrum disorder (15.38%) & attention deficit hyperactivity disorder (7.69%). Conclusion: In our study, the commonest neuro-cutaneous syndrome is tuberous sclerosis complex and it’s cutaneous manifestation is facial angiofibroma Bangladesh J Child Health 2019; VOL 43 (1) :15-20


Author(s):  
Kanchan Srivastava ◽  
Jyoti Bajpai ◽  
Apoorva Narain ◽  
Adarsh Tripathi ◽  
Surya Kant

KYAMC Journal ◽  
2013 ◽  
Vol 2 (1) ◽  
pp. 128-131
Author(s):  
Muhammad Abdul Kayum Shaikh ◽  
Md Zulfikar Ali ◽  
Md Saiful Islam ◽  
AKM Mokhlesuzzaman

Health includes both physical and psychological well being of an individual. There is strong chance that every physical illness has psychological component and in many cases psychological disorders present with somatic symptoms; so psychiatric referral is very important to the proper management of a patient. The aim of this study was to find out the pattern of referral from different outpatient departments in a tertiary level hospital in rural setting of Bangladesh. In this descriptive study total 237 patients were evaluated in 7 months period and most of the diagnoses were anxiety disorders (40%), major depressive disorder (35%) and somatoform disorders (17%). Departments of medicine (cardiology 34%, internal medicine 28% and neurology 26%) referred most of the patients than the surgical or gynecological departments.DOI: http://dx.doi.org/10.3329/kyamcj.v2i1.13517 KYAMC Journal Vol.2(1) 2011 pp.128-131


2020 ◽  
Vol 7 (4) ◽  
Author(s):  
Mudasir Maqbool ◽  
Ginenus Fekadu ◽  
Dinka Dugassa ◽  
Firomsa Bekele ◽  
Ebisa Turi ◽  
...  

Background: Substance-use related disorders (SUD) are a major public health concern worldwide, especially in developing nations. Currently, it is characterized by high rates of mortality and morbidity. Moreover, through increased utilization of healthcare services, it causes both direct and indirect significant medical expenditures. Objectives: The current study aimed to evaluate the pattern of Substance abuse in the department of psychiatry of a Tertiary Care Hospital, Srinagar, Jammu, and Kashmir, India. Methods: This is a descriptive, cross-sectional, and open study conducted in the psychiatry inpatient department of a tertiary care hospital, Srinagar, Jammu, and Kashmir, India, for 7 months (January to May 2020). Results: Over a period of seven months, a total of 135 participants were recruited (105 males and 30 females). Most of them were Muslim (96.29%), married (68.14%), and living as a nuclear family (75.55%). Tobacco (Nicotine) was the most commonly used substance by those admitted to the psychiatry ward (62.96%), followed by cannabis (11.11%) and opioids (11.11%). The most comorbidity associated with substance abuse was psychiatric disorders (36.84%), followed by neurological disorders (21.05%), and gastrointestinal disorders (7.89%). Among those with psychiatric disorders, 14 (10.37%) had mood (bipolar) disorders. A total of 1129 medicines were prescribed for 135 patients. The mean ± standard error of the mean (SEM) of the prescribed medicines was 8.48 ± 3.3. The average number of drugs per encounter was 8.48%. Conclusions: In this study, cigarette smoking (nicotine) was the most commonly abused substance. It can be justified by the fact that Kashmir is a conflict zone, and many people suffer from the post-traumatic stress disorder. Hence, a collaborated effort is necessary to improve the prescribing pattern and to enhance the rational use of medications for better treatment outcomes.


2012 ◽  
Vol 34 (2) ◽  
pp. 44-50 ◽  
Author(s):  
AKM Moinuddin ◽  
Md Mizanur Rahman ◽  
Shaheen Akhter ◽  
Syeda Tabassum Alam ◽  
CA Kawser

Objective: Identifying the risk factors of poor seizure control in children in a setting of tertiary care hospital. Design: Retrospective study. Setting: Child Development and Neurology Unit in the department of Paediatrics of Bangabandhu Sheikh Mujib Medical University (BSMMU). Study period: January 2004 through December 2005. Subjects: One hundred and twenty epileptic children were studied. They were grouped into controlled group (seizure free for more than six months) and poorly controlled group (having one or more seizure per month over a period of six months or more and who had experienced trials of at least two different antiepileptic drugs at optimum doses alone or in combination with adequate compliance) at the end of intervention and compared. Results: In this study 76 (63.3%) children were male and 44 (36.7%) children were female. Out of 120 cases 79 (65.8%) were in controlled group and 41 (34.2%) cases had poorly controlled epilepsy. Mean age of the controlled group and poorly controlled group of children were 79 months and 40.3 months respectively. Focal epilepsy was found in 30 (68%) cases in controlled and in 14 (31.8%) cases of poorly controlled group and generalized epilepsy was found in 42 (72%) cases in controlled and in 19 (28.8%) cases in poorly controlled group. Idiopathic epilepsy was more common which was 37 (46%) in controlled group against 14 (34%) in poorly controlled group. But symptomatic and cryptogenic cases were more prevalent with poorly controlled group 57.5% than controlled group 53%. In poorly controlled group 48.8% had cerebral palsy in comparison to 22.8% of controlled group. Early onset of seizure before one year was 25.3% in controlled and 78% in poorly controlled group (odds ratio=.2322, p =.0082) and one or more seizure per week 43% in controlled and 92.7% in poorly controlled group (odds ratio=.1218, p=.0032) were found as risk factors of poorly controlled epilepsy. Conclusion: Early onset of seizure before one year, symptomatic epilepsy and one or more seizure per week at diagnosis were found as risk factors of poorly controlled epilepsy in children attending a tertiary care hospital. DOI: http://dx.doi.org/10.3329/bjch.v34i2.10216 BJCH2010; 34(2): 44-50


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