scholarly journals Bilateral Optic Atrophy with Thickened Meninges (Idiopathic Hypertrophic Pachymeningitis) - A Rare Entity

JMS SKIMS ◽  
2011 ◽  
Vol 14 (1) ◽  
pp. 25-27
Author(s):  
Ravouf Asmi ◽  
Feroze Ahmad ◽  
Rangreze Imran ◽  
Zarka Amin Masoodi ◽  
Mushtaq A Wani
Keyword(s):  
Infectious Diseases ◽  
Optic Atrophy ◽  
Visual Loss ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Rare Entity ◽  
Hypertrophic Pachymeningitis ◽  
Open Biopsy

Idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic nonspecific and granulomatous inflammatory disorder of the dura with unknown etiology. The diagnosis can be established by an open biopsy and exclusion of all other specific granulomatous and infectious diseases. We report a case of Idiopathic hypertrophic pachymeningitis who manifested primarily by headache and visual loss with optic atrophy. The patient was thoroughly investigated and showed no evidence of underlying cause. The diagnosis was established by an open biopsy. JMS 2011;14(1):25-27

Idiopathic hypertrophic spinal pachymeningitis

2011 ◽  
Vol 15 (2) ◽  
pp. 195-201 ◽  
Author(s):  
Moksha G. Ranasinghe ◽  
Omar Zalatimo ◽  
Elias Rizk ◽  
Charles S. Specht ◽  
G. Timothy Reiter ◽  
...  
Keyword(s):  
Conservative Therapy ◽  
Surgical Intervention ◽  
Clinical Course ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Review Of The Literature ◽  
Neurological Damage ◽  
Hypertrophic Pachymeningitis ◽  
Pathological Confirmation ◽  
Prompt Diagnosis

Spinal idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic, nonspecific, granulomatous inflammatory disorder of the dura with unknown etiology. It can cause a localized or diffuse thickening of the dura mater with compression of the spinal canal and possible myelopathic symptoms. The authors report 3 consecutive cases of spinal IHP with a review of the literature. The diagnosis of spinal IHP was based on biopsy and pathological confirmation. Typical MR imaging findings suggestive of spinal IHP were noted in all cases. The clinical course may be marked by deterioration despite conservative therapy and may require surgical intervention to prevent irreversible neurological damage. Therefore, prompt diagnosis and institution of proper treatment is critical.

scholarly journals Hypertrophic pachymeningitis: case report

2003 ◽  
Vol 61 (1) ◽  
pp. 107-111 ◽  
Author(s):  
Leonardo de Deus-Silva ◽  
Luciano de Souza Queiroz ◽  
Verônica de Araújo Zanardi ◽  
Enrico Ghizoni ◽  
Hoyama da Costa Pereira ◽  
...  
Keyword(s):  
Case Report ◽  
Optic Atrophy ◽  
Nasal Septum ◽  
Visual Loss ◽  
Inflammatory Process ◽  
Inflammatory Diseases ◽  
Early Stage ◽  
Underlying Disease ◽  
Extensive Investigation ◽  
Hypertrophic Pachymeningitis

Hypertrophic pachymeningits is an unusual cause of neurological symptoms and is often secondary to infections, carcinomatosis or inflammatory diseases. It may also be idiopathic. We report a case of pachymeningitis which was manifested primarily by psychosis and visual loss with optic atrophy and destruction of nasal septum. The patient, a 45 year old woman was submitted to extensive investigation without evidence of any underlying disease. A meningeal biopsy was performed and showed a mostly unspecific inflammatory process with extensive fibrosis of the dura and few early stage granulomas. These findings suggest either neurosarcoidosis or idiopathic hypertrophic pachymeningitis.

P01-228-Behcet's syndrome comorbid with bipolar disorder: A case report

2011 ◽  
Vol 26 (S2) ◽  
pp. 229-229
Author(s):  
F. Maner ◽  
Ö. Şahmelikoğlu ◽  
Ö. Hısım ◽  
H. Özhan ◽  
H. Sarıahmetoğlu ◽  
...  
Keyword(s):  
Bipolar Disorder ◽  
Manic Episode ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Behçet’S Syndrome ◽  
Psychomotor Activity ◽  
Chronic Inflammatory Disorder ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

IntroductionBehcet's Syndrome is a chronic inflammatory disorder of unknown etiology, characterized by aphthous lesions and recurrent ulceration of the mouth, genitals and uveitis.ObjectivesThe central nervous system is involved in about 20% of cases.AimsOnly few reports deal with affective symptoms associated with Behcet's syndrome.MethodsWe report a case of a 43 year old male with Neuro-Behcet's Syndrome that presents with a psychotic manic attack. He developed Behcet's Syndrome at the age of 23, with recurrent uveitis and aphthous lesions in the mouth, painful ulcers in the genitalia and erythema nodosum. HLA-B 5 was positive.ResultsHe was treated with azothioprine 150 mg/day for 13 years and prednole 100 mg/day during uveitis attacts for a week. At the age of 37 a sudden occurrence of right hemiparesia due to cerebrovascular accidence salicylic acid 100mg/day, siclosporine 150 mg/day, piracetame 1600mg/day were administered. He presented to psychiatry clinic in manic episode with euphoric mood, psychomotor agitation, talkativeness, decreased need for sleep, excessive buying and he had an unrealistical thought that he was a player of a famous soccer team. He was diagnosed as bipolar I disorder, according to DSM-IV. This was the patient's first admission and the symptoms which were continuing for 6 years exaggerated during uveitis attacks.Psychiatric examination releaved that increaced psychomotor activity, hypomaniac affect, amount and affect speed of speech affect, increased associations, grandiose delusions.ConclusionThere are a few reports dealing with bipolar disorder as an entity related to Behcet's syndrome.

scholarly journals Zero prevalence of hepatitis B and hepatitis C infections in clinicopathologically proven lichen planus cases: a cross sectional study at a tertiary care centre in south India

2017 ◽  
Vol 3 (3) ◽  
pp. 351 ◽  
Author(s):  
Lekshmi Sajini ◽  
Gopikrishnan Anjaneyan ◽  
Soumya Jagadeesan ◽  
Vinitha Varghese Panicker ◽  
Sreedevan V. ◽  
...  
Keyword(s):  
Hepatitis C ◽  
Hepatitis B ◽  
Lichen Planus ◽  
Tertiary Care ◽  
Unknown Etiology ◽  
Study Group ◽  
Inflammatory Disorder ◽  
Hepatitis C Infection ◽  
Cell Degeneration ◽  
The World

<p class="abstract"><strong>Background:</strong> Lichen planus (LP) is a common chronic mucocutaneous inflammatory disorder of unknown etiology. Several editorials, chapters, studies have suggested an association between hepatitis C virus and hepatitis B virus (to a lesser extent) in LP. This association was not seen in various parts of the world including India.</p><p class="abstract"><strong>Methods:</strong> Patients attending the dermatology outpatient department with lichen planus confirmed by histopathologic examination were included in the study and along with clinic-epidemiological data, all the patients were tested for hepatitis B and hepatitis C infection.<strong></strong></p><p class="abstract"><strong>Results:</strong> A total of 84 patients of clinico-pathologically proven lichen planus were included in the study out of which 31 were males and 53 were females. The most common age group of lichen planus in our study group was 40-50 years. Major histopathological findings were basal cell degeneration (92.7%), hyperkeratosis (89%), pigment incontinence (73%) and lymphohistiocytic infiltration (70%). All patients in this study group tested negative for HBsAg and anti-HCV antibodies.</p><p><strong>Conclusions:</strong> Although numerous studies around the world have suggested an association between HCV and HBV infections with lichen planus- multiple subsequent studies including the present study shows contrasting results especially in this part of the world. The authors believe routine screening of lichen planus patients for presence of hepatitis B and hepatitis C infection is not warranted, particularly in Indian population. </p>

scholarly journals A Rare Case of Kimura Disease in the Wrist

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4881-4881
Author(s):  
Parastou Tizro ◽  
Taraneh Hashemi Zonouz ◽  
Fawaz Almutairi ◽  
Donald Karcher
Keyword(s):  
Soft Tissue ◽  
Soft Tissues ◽  
Conflicts Of Interest ◽  
Soft Tissue Mass ◽  
Germinal Centers ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Kimura Disease ◽  
High Endothelial Venules ◽  
Tissue Mass

Kimura disease is a rare benign chronic inflammatory disorder of unknown etiology that typically involves lymph nodes and soft tissues. This disease is most common in middle-aged Asian men. Although the disorder most frequently involves the head and neck region, it has also been reported to involve the extremities. Lesions typically show follicular lymphocytic hyperplasia with prominent interfollicular venules and a mixed inflammatory infiltrate, including numerous eosinophils. We describe a case of Kimura disease in a 29-year-old female presenting with a slowly enlarging, painless soft tissue mass in her left wrist for five months. The lesion was non-tender, mobile, and compressible. Lab results were significant for peripheral blood eosinophilia and elevated IgE. Microscopically, the mass was circumscribed, with apparent fibrous capsule or pseudocapsule, prominent high-endothelial venules, and inflammatory cells consisting of lymphocytes and eosinophils. There were prominent secondary B-lymphocytic follicles with germinal centers containing many penetrating small blood vessels, and some follicles exhibiting follicle lysis and a small amount of eosinophilic extracellular material. Polykaryocytes were noted in many germinal centers. Angiolymphoid hyperplasia with eosinophilia, a main differential diagnosis of Kimura disease, was considered; however, the combined clinical and morphologic findings appeared to be more consistent with Kimura disease, particularly in the context of eosinophilia and elevated IgE level. Kimura disease may present as a rare cause of a soft tissue mass and a diagnostic challenge for clinicians and pathologists, especially in less common sites such as the wrist. Disclosures No relevant conflicts of interest to declare.

scholarly journals Immunopathogenesis of Ocular Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Un Chul Park ◽  
Tae Wan Kim ◽  
Hyeong Gon Yu
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Inflammatory Disorder ◽  
General Hypothesis ◽  
Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

scholarly journals Harding’s disease: an important MS mimic

2019 ◽  
Vol 12 (3) ◽  
pp. e228337
Author(s):  
Stuti Joshi ◽  
Allan G Kermode
Keyword(s):  
Multiple Sclerosis ◽  
Mitochondrial Dna ◽  
Optic Neuropathy ◽  
Movement Disorders ◽  
Optic Atrophy ◽  
Cardiac Arrhythmias ◽  
Visual Loss ◽  
Mtdna Mutation ◽  
Individual Family ◽  
Hereditary Optic Neuropathy

Leber’s hereditary optic neuropathy (LHON) is a mitochondrially inherited disorder characterised by bilateral, painless visual loss which leads to severe optic atrophy. It can be associated with other conditions including multiple sclerosis (MS), movement disorders, epilepsy and cardiac arrhythmias. The association of LHON with an MS-like illness is often referred to as Harding’s disease (or Harding’s syndrome). We report two siblings, who both harbour the 11 778 mitochondrial DNA (mtDNA) mutation, but who manifest markedly different clinical phenotypes; a male with classical LHON and a female with an MS-like illness. LHON affects males four to five times more often than females. By contrast, Harding’s disease is seen predominantly in females, in a pattern comparable to that seen in MS. The pathogenic basis behind the variation in penetrance and phenotype between genders and individual family members remains unclear.

scholarly journals Inhibition of autophagy curtails visual loss in a model of autosomal dominant optic atrophy

2020 ◽  
Vol 11 (1) ◽  
Author(s):  
Marta Zaninello ◽  
Konstantinos Palikaras ◽  
Deborah Naon ◽  
Keiko Iwata ◽  
Stephanie Herkenne ◽  
...  
Keyword(s):  
Optic Atrophy ◽  
Visual Loss ◽  
Autosomal Dominant ◽  
Autosomal Dominant Optic Atrophy ◽  
Dominant Optic Atrophy

scholarly journals Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan’s Syndrome

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Ahad Azami ◽  
Nasrollah Maleki ◽  
Mohammadreza Kalantar Hormozi ◽  
Zahra Tavosi
Keyword(s):  
Hearing Loss ◽  
Systemic Vasculitis ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Conjunctival Hyperemia ◽  
Cogan’S Syndrome ◽  
Auditory Brain Stem Response ◽  
Chronic Inflammatory Disorder ◽  
Cogan's Syndrome ◽  
Interstitial Keratitis

Cogan’s syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan’s syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement.

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