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 ◽  
...  

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.

2019 ◽  
Vol 11 (01) ◽  
pp. 175-177
Author(s):  
Abdulla Alsulaiman

AbstractIdiopathic hypertrophic pachymeningitis (IHP) can resemble other disorders associated with spinal compression. It is a rare inflammatory fibrosing disease of the dura of unidentified etiology and is considered a diagnosis of exclusion. We present a case of idiopathic hypertrophic spinal pachymeningitis occupying a long segment of cervical dura. This is a case of 38-year-old female patient, who suffered progressive neck pain for 2-year duration. Examination revealed spasticity in all four limbs, plus three symmetric reflexes all over, and the sensory level at T4. Magnetic resonance imaging showed spinal cord compression by a thickened anterior and posterior dura adjacent to the cord from C2 to C7. The diagnosis of spinal IHP was confirmed through biopsy. The patient improved after treatment with corticosteroids. Early surgical intervention with postoperative corticosteroid therapy is a known treatment for this disease, as a way to prevent irreversible neurological damage.


JMS SKIMS ◽  
2011 ◽  
Vol 14 (1) ◽  
pp. 25-27
Author(s):  
Ravouf Asmi ◽  
Feroze Ahmad ◽  
Rangreze Imran ◽  
Zarka Amin Masoodi ◽  
Mushtaq A Wani

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


2010 ◽  
Vol 76 (11) ◽  
pp. 1282-1286 ◽  
Author(s):  
David M Margolius ◽  
Thomas E. Cataldo

The adverse effects of nonsteroidal anti-inflammatory drugs (NSAIDs) on the colon have been reported as a wide spectrum of symptoms, signs, and endoscopic findings. Despite the extensive use of NSAIDs, and the potential seriousness of NSAID colopathy, this condition often goes unrecognized or misdiagnosed. We report three cases of NSAID colopathy in which the diagnosis of malignancy was incorrectly made based on endoscopic findings. Before any surgical intervention, we entertained the diagnosis of NSAID colopathy based on clinical presentation. Ultimately, in two of the three cases, surgery was avoided and the lesions resolved. We present their clinical course and a review of the pertinent literature reviewing theories of the pathophysiology, the range of clinical presentations, and the pathological findings of this entity.


2018 ◽  
Vol 32 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Shahine Goulam-Houssein ◽  
Jeffrey L Grenville ◽  
Katerina Mastrocostas ◽  
David G Munoz ◽  
Amy Lin ◽  
...  

IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG4 pachymeningitis mimicking a sphenoid wing meningioma, which is to our knowledge the largest mass-forming pachymeningitis published in the literature. Our second case depicts another presentation of extensive IgG4 pachymeningitis involving both cavernous sinuses and surrounding Meckel’s caves. The third case describes a patient with presumed lymphocytic hypophysitis, which was later determined to be IgG4-related hypophysitis with concomitant pachymeningitis and perineural spread along the optic nerves. The delayed diagnoses in our cases illustrates the diagnostic challenge that clinicians face in differentiating intracranial IgG4-RD from other infiltrative diseases such as sarcoidosis, granulomatous disease, tuberculosis and lymphoma. Earlier consideration of IgG4-related hypophysitis and hypertrophic pachymeningitis in the differential diagnosis can prevent significant morbidity including unnecessary surgical intervention and organ failure secondary to extensive fibrosis.


2020 ◽  
Vol 12 (3) ◽  
pp. 231-235
Author(s):  
Carl Maximilian Thielmann ◽  
Wiebke Sondermann

Erythromelanosis follicularis faciei et colli, a rare condition of unknown etiology, was first described by Kitamura et al. from Japan in 1960. It is characterized by a triad consisting of well-demarcated erythema, hyperpigmentation, and follicular papules. We report the case of a 50-year-old Caucasian male, who had asymptomatic symmetrical facial lesions since the age of 42. His family history was unremarkable. Published erythromelanosis follicularis faciei et colli cases of the last 10 years are summarized in this report to demonstrate the variability and differences in the clinical presentation of this uncommon diagnosis.


2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Hyma Polimera ◽  
Prashanth Moku ◽  
Shady Piedra Abusharar ◽  
Monali Vasekar ◽  
Jayakrishna Chintanaboina

Ewing sarcoma (ES) is a highly aggressive malignant bone cancer. ES is part of the Ewing sarcoma family of tumors (ESFT), which express characteristic t(11;22) translocation as well as higher levels of CD99. Given that metastasis and tumor burden are significant prognostic factors in patient’s response to treatment, prompt diagnosis is needed to effectively treat ESFT patients. However, the challenges in classifying and characterizing ESFT complicate effective management and treatment of ES. In this report, we present a rare case of ES metastasis to the pancreas. Upon review of the literature, we found 39 cases of ESFT involving the pancreas, but only 3 were metastatic to the pancreas while the remaining cases of ESFT primarily originated from the pancreas. Given the rarity of such metastasis, the positive outcome in our patient’s case may explain the importance of prompt diagnosis in order to initiate appropriate treatment.


2011 ◽  
Vol 26 (S2) ◽  
pp. 229-229
Author(s):  
F. Maner ◽  
Ö. Şahmelikoğlu ◽  
Ö. Hısım ◽  
H. Özhan ◽  
H. Sarıahmetoğlu ◽  
...  

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.


2004 ◽  
Vol 128 (2) ◽  
pp. 210-213 ◽  
Author(s):  
Peter M. Voorhees ◽  
Kathryn A. Carder ◽  
Scott V. Smith ◽  
Lanier H. Ayscue ◽  
Kathleen W. Rao ◽  
...  

Abstract Follicular lymphoma is an indolent lymphoma characterized by the (14;18) translocation, which leads to aberrant expression of Bcl-2. Translocations involving 8q24 are most commonly associated with Burkitt lymphoma and result in c-Myc overexpression. We report a case of follicular lymphoma of predominant small cleaved-cell type (grade 1) associated with both a t(14;18)(q32;q21) and a t(8;22)(q24;q11). The 8q24 translocation predicted an aggressive clinical course, as the lymphoma transformed into acute lymphoblastic leukemia within a year of initial diagnosis. Routine cytogenetic analysis is recommended at initial diagnosis of follicular lymphoma to better identify abnormalities that may predict prognosis and influence therapy.


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