Vision Loss in Subacute Sclerosing Panencephalitis: A Systematic Review of Case Reports and Case Series.

Vision loss is a presenting complaint in many patients with subacute sclerosing panencephalitis (SSPE). Data related to vision loss in SSPE is available only in the form of case reports. In this systematic review, we evaluated characteristics of vision loss, affected anatomic site, and patient course and outcome. We extracted data from four databases: PubMed, Embase, Scopus, and Google Scholar. The last search was done on October 26, 2022. We adhered to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The protocol was registered with PROSPERO (CRD42022362652). Dyken's criteria were used for the diagnosis of SSPE. The data were recorded in an Excel sheet. The Joanna Briggs Institute Critical Appraisal tool was used to assess the quality of data. The mean age of patients with SSPE was 17.9 years. Males outnumbered females (60:34). In 73 patients (76%), duration of illness/onset of vision loss was less than 6 months. In 76% patients (n = 73), visual manifestations appeared before encephalopathy. Involvement of the retina (58 of 96, 60.4%), optic nerve (9 of 96, 9.3%), or cerebral cortex (29 of 96, 30.2%) was responsible for vision loss. T2/fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) parieto-occipital hyperintensities were the most frequent (71.4%) neuroimaging abnormality. Retinal biopsy revealed similar findings revealed by brain histopathology. All patients died and became akinetic mute during the follow-up period, which ranged from a few weeks to a few years. In conclusion, retinal involvement was the most common cause of vision loss. Vision loss often precedes encephalopathy. Cortical vision loss was associated invariably with T2/FLAIR MRI hyperintensities in the parieto-occipital region.

Tubercular Harada disease - An unreported uveitic entity.

Introduction: & IMPORTANCE: Ocular tuberculosis and Vogt Koyanagi Harada disease (VKHD) both are the important cause of panuveitis. In tubercular endemic region like Nepal, latent tuberculosis (TB) may be accompanied with the features of VKHD. Hence, the aim of our publication is the use the term Tubercular Harada disease (THD) for such panuveitis with mixed features. Case presentation: We aim to report two cases of panuveitis from Nepal with the simultaneous features of tuberculous uveitis and Harada disease managed with combined antitubercular agents and antimetabolites. Clinical discussion: Two cases presented with bilateral decreased vision with no systemic associations. They had bilateral panuveitis and sunset glow. Ultrasonography showed the choroidal thickening, optical coherence tomography confirmed macular edema with retinal nerve fibre layer edema. Electroretinogram of both eyes showed reduced P1 wave amplitude. All the systemic investigations were normal except the positive tuberculin skin test and TB QuantiFERON Gold test.Both of them were managed with intravenous/oral corticosteroid (1mg/kg) along with CAT- I ATT regimen (2HRZE+7HR) for 9 months and oral antimetabolites (azathioprine or methotrexate). Long term follow-up showed normal visual acuity with no evidence of recurrence of uveitis. Conclusion: Mycobacterium could have triggered the onset of Harada disease in TB endemic country like Nepal leading to simultaneous presentation of Tubercular Harada Disease.

Foreign Body causing Displacement of Immature Fractured Apical Root Fragment: An Unusual Case Report.

Trauma is a common cause of fractured teeth with exposed canals in growing children. These children use foreign bodies like stapler pin, lead pencil, nail, etc., to explore the canal of fractured tooth. Sometimes, these foreign objects may get stuck in the canal, which the children do not reveal to their parents because of fear. These foreign objects may act as a potential source of infection. We herewith present a case of a 12-year-old boy who presented with a stick lodged in the root canal of maxillary right lateral incisor along with the displaced fractured tooth segment at the apex and the associated management. How to cite this article: Moda A, Singla R, Agrawal PM. Foreign Body causing Displacement of Immature Fractured Apical Root Fragment: An Unusual Case Report. Int J Clin Pediatr Dent 2018;11(3):247-249.

Absolute Lymphocyte Count as a Surrogate Marker of CD4 Count in Monitoring HIV Infected Individuals: A Prospective Study.

INTRODUCTION: CD4 cell count has been proposed to be substituted by Absolute lymphocyte count in monitoring HIV infected individuals as methods of CD4 cell count and plasma viral estimation require expensive, specialized equipments and highly trained personnel. AIM: To assess the clinical utility of the Absolute Lymphocyte Count (ALC) to serve as a surrogate marker for predicting a CD4 count < 200 cells/µl in patients with HIV infection in resource poor countries. MATERIALS AND METHODS: A prospective study of 61 patients with HIV/AIDS was conducted. Sensitivity, specificity, Positive Predictive Value (PPV), Negative Predictive Value (NPV) of various ALC cut-offs were computed for CD4 cell count < 200 cells/µl for age < 30 or age ≥ 30 years. Pearson correlation, Linear regression and Receiver Operating Characteristics (ROC), were used. RESULTS: For patients aged ≥ 30 years, sensitivity, specificity, positive and negative predictive value of ALC <1200 cells/µl to predict CD4 cell count < 200 cells/µl were 34.48%, 67.5%, 43.48%, 58.69% respectively. For subjects aged < 30 years, these values were 27.27%, 67.5%, 18.75%, 77.14%, respectively. A ALC < 1643 was found to have maximal sensitivity for predicting a CD4 cell count <200/ µl. CONCLUSION: Our data revealed good correlation between ALC and CD4 cell counts but ALC cut-off of 1200 was not a surrogate marker for CD4 cell count < 200 cells/µl. As we increase the cut-off to <1643/ µl it could be the cost-effective surrogate marker for CD4 cell counts < 200 cells/µl in resource limited settings.

Thoracic intramedullary chordoma without bone involvement: a rare clinical entity.

An 8-year-old boy presented with a 1-year history of low backache, followed by paraparesis and urinary incontinence. MRI of the thoracic spine revealed an intramedullary, intensely contrast-enhancing lesion extending from T11 to L1 vertebral level, consistent with astrocytoma, ependymoma or haemangioblastoma. A diagnosis of intramedullary chordoma was made on tissue biopsy and immunohistochemical study. This is the second report of an intramedullary chordoma without bone involvement in English literature. After 6 months of follow-up, patient showed good clinical outcome in terms of improvement in power in lower limbs and backache.

Histopathological study of cutaneous manifestations in HIV and AIDS patients.

OBJECTIVES: Most human immunodeficiency virus (HIV)-infected patients develop various skin diseases. These skin manifestations not only act as markers but also reflect the patient's underlying immune status. Investigating CD4 counts is costly and not always possible. Thus, the potential value to be gained by using skin manifestations as predictors of low CD4 counts and disease progression should be explored. The present study attempted to correlate the association of various cutaneous disorders found in HIV patients with CD4 and CD8 counts, the CD4 : CD8 ratio and stage of HIV infection. METHODS: This was a prospective study involving 61 patients who were HIV-positive and demonstrated skin lesions. Punch biopsies of skin were taken for histopathological diagnosis. CD4 and CD8 T cell counts were performed. RESULTS: The study sample included a majority of male patients, most of whom were aged 21-40 years. Pruritic papular dermatitis was the most common skin manifestation, followed by molluscum contagiosum, eosinophilic folliculitis, and Hansen's disease. Most of the lesions were associated with CD4 counts of <220/µl (n = 38). All skin lesions associated with HIV or acquired immune deficiency syndrome (AIDS) showed a CD4 : CD8 ratio of <0.50. CONCLUSIONS: The study findings demonstrate an inverse relationship between CD4 counts and the occurrence of skin lesions. The majority of lesions were associated with stage 3 or stage 4 infection. Thus, specific cutaneous manifestations can be considered as good clinical indicators for predicting underlying immune status in resource-poor countries.

Rumination syndrome: an emerging case scenario.

This article describes the case of a 19-year-old woman presenting with repetitive episodes of effortless vomiting, which started within 3 weeks of her naval boot camp training. She underwent a battery of costly investigations before the diagnosis of rumination syndrome could be made. One of the reasons for her delayed diagnosis is that many physicians are unaware of, or are reluctant to make the diagnosis of rumination syndrome. The purpose of this article is to make the general physician aware of the possibility of rumination syndrome in adolescents and adults of normal intelligence, even though it was initially considered only in infants and mentally retarded individuals. The key to diagnosis is a thorough patient history. Reassurance and behavioral therapy is the mainstay of treatment, with a reported success of >80%.

Enoxaparin-induced skin necrosis: a fatal outcome.

Enoxaparin, a low-molecular-weight heparin used to treat and prevent deep venous thrombosis, has been evaluated in several clinical trials. Thrombosis induced by enoxaparin with no evidence of heparin-induced thrombocytopenia (HIT) is seldom described. We report a rare case in which an 89-year-old African-American female developed large, multiple, painful lesions induced by enoxaparin administration. Laboratory investigations for HIT, disseminated intravascular coagulation, protein C, protein S, factor V, factor VIII, antithrombin III, and homocysteine deficiency were negative. Unfortunately, despite aggressive management for 2 weeks, the patient developed severe sepsis and died.