Presentation
A diagnosis of HSP was subsequently shown to be the initial presentation of ANCA-related systemic vasculitis. [ncbi.nlm.nih.gov]
We present 2 cases of this condition with a description of MR imaging findings. [ajnr.org]
Entire Body System
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Shivering
The shiverings interest arise as to subsidiary lesions and appearances, and before admission to the infirmary marked, no doubt, the If we speak beginning of suppuration. [kundoc.com]
Gastrointestinal
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Upper Abdominal Pain
RESULTS: We report a 66-year-old male with a history of renal cell carcinoma and bladder cancer who presented with upper abdominal pain, thoracic myelopathy (progressive weakness and numbness of the lower extremities, and gait disturbances), and weight [ncbi.nlm.nih.gov]
Skin
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Flushing
The patient was thin and anaemic; his face wore an ex- of the brain the puncta cruenta were large and, numerous, pression of suffering; his cheeks were flushed; he com. blood oozing from them. [kundoc.com]
Urogenital
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Microscopic Hematuria
Additionally, simple microscopic hematuria was found in 1 case suggestive of renal involvement and the other 1 complicated with interstitial lung disease. Then, a diagnosis of HSP secondary to AASV was made. [ncbi.nlm.nih.gov]
Neurologic
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Cranial Nerve Involvement
We present a case which is interesting not only by virtue of the rarity of this disease but also due to the extent of cranial nerves involved and the drastic response to therapy initiated once suspected on MRI. [omicsonline.org]
nerve involvement and excluded diabetic neuropathy. [jcor.in]
Lower cranial nerve involvement, especially VIII and X, was most commonly observed. [neurologyindia.com]
Cranial nerve involvement with Lyme borreliosis demonstrated by magnetic resonance imaging. Neurology 1992;42: 671-3. [44] Lee JH, Na DG, Choi KH, et al. [em-consulte.com]
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Papilledema
Clinical features include headache, cranial nerve impairment, paraparesis or tetraparesis, ataxia, hydrocephalus, papilledema, hemorrhage or secondary vascular occlusions (5). [path.upmc.edu]
Other neurologic symptoms of IHP include cranial nerve palsies (cranial nerves IV–VIII), cerebellar ataxia, seizures, and neuroophthalmic symptoms such as visual field loss, complete blindness, optic neuropathy, and increased intracranial pressure with papilledema [pubs.rsna.org]
Her left optic disk was atrophic and there was papilledema on the right eye. Another MRI was performed and showed the same pattern of meningeal enhancement seen on the first one. [scielo.br]
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Unable to Walk
ABSTRACT We report a case of idiopathic hypertrophic spinal pachymeningitis in a 56-year-old woman who complained of a tingling sensation and progressive weakenss of both legs since 6 months prior, and then was unable to walk for 1 week. [jpatholtm.org]
On presentation, he was unable to walk. He had a history of hypertension, diabetes mellitus, and hyperlipidaemia. [yumpu.com]
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Absent Deep Tendon Reflex
Diagnostic criteria for GBS * Brighton Case Definitions Findings in the Present Case Bilateral & flaccid weakness of limbs Decreased or absent deep tendon reflexes Monophasic course & time btwn onset & nadir of 12 hrs–28 days CSF white blood cell count [doi.org]
Workup
Case details (continued) The further workup revealed a HIV virus load of 198,322 and CD4 count of 281 cells/μL (normal range: 490-1740 cells/μL). Tuberculin skin test reading was positive 8 mm. [jpgmonline.com]
[…] fibrosis. [14] Chronic periaortitis had been recognized as a common manifestation of IgG4RD, and it commonly affects abdominal aorta; it often coexists with autoimmune pancreatitis, another prototype manifestation of this disease. [15] In the diagnostic workup [indianjrheumatol.com]
Urine
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Albuminuria
[…] leucocythaemia a pathognômonic sign of any one patho- were very loose and very light in colour; the urine was logical process, seems to me as unscientific as if we were scanty, with excess of uric acid, but without albumen; still to use the terms " jaundice" or "albuminuria [kundoc.com]
Serum
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Albumin Increased
Summary of tests performed Test Comment IgG subtyping Normal IgG4 Serum protein electrophoresis Low albumin & increasing α1-globulin; no evidence of paraproteinemia; normal IgG & IgA; IgM was increased to 3.4 g/L (ref range 0.5–2.0 g/L) Factor XII Factor [doi.org]
Treatment
Spinal canal decompression and autogenous bone grafting are accepted treatments in the young patient. Careful observation of the patient to watch for local progression is necessary. [ncbi.nlm.nih.gov]
Recent decades have also witnessed fundamental progress in understanding of the pathophysiology of spinal cord diseases, treatment options, neurosurgical procedures, and endovascular treatments. [books.google.de]
Prognosis
The prognosis of idiopathic HSP with positive inflammatory signs may be worse; the authors stress the significance of early surgical treatment. [ncbi.nlm.nih.gov]
According to a review of the English and Japanese literature on idiopathic HSP, the prognosis of patients with positive inflammatory signs is worse than that of those with no inflammatory signs. [journals.lww.com]
Keywords Idiopathic hypertrophic spinal pachymeningitis Radiculalgia CSF Therapy Prognosis This is a preview of subscription content, log in to check access. [link.springer.com]
Etiology
Neurosurgery, Penn State Hershey Medical Center, Hershey, Pennsylvania, USA. [email protected]u Abstract Spinal idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic, nonspecific, granulomatous inflammatory disorder of the dura with unknown etiology [ncbi.nlm.nih.gov]
Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. [londonspine.com]
HP has diverse etiologies like infections, chronic inflammatory processes, collagen vascular diseases and malignancy. [ijpmonline.org]
Epidemiology
In epidemiology, it may be used to analyze a closely grouped series of events or cases of disease or other health-related phenomenon with well-defined distribution patterns in relation to time or place or both. [bioportfolio.com]
The epidemiology and pathogenesis of IgG4-RHP are poorly un-derstood. [docslide.com.br]
Epidemiology Pachymeningitis is most often found in infants and in middle-aged men. [iliveok.com]
Epidemiology of sarcoidosis in the Isle of Man: evidence for space time clustering. Thorax 1987;42:427-430. [ Links ] 27. Kern DG, Neil MA, Wrenn DS, Varone JC. Investigation of an unique time-space cluster of sarcoidosis in firefighters. [scielo.br]
[An unsolved epidemiological problem: cerebrospinal meningitis in Africa. Apropos of a recent epidemic in Niger]. Med Trop (Mars) 1961 Sep-Oct; 21 :491–525. [ PubMed ] [ Google Scholar ] LAPEYSSONNIE L, LOUIS J, BONNARDOT R, LEFEVRE M. [ncbi.nlm.nih.gov]
Pathophysiology
Recent decades have also witnessed fundamental progress in understanding of the pathophysiology of spinal cord diseases, treatment options, neurosurgical procedures, and endovascular treatments. [books.google.de]
Abstract In pathophysiology and clinical practice, the intracranial pressure (ICP) profiles in the supratentorial and infratentorial compartments are unclear. [ncbi.nlm.nih.gov]
Prevention
Idiopathic HSP is a clinical emergency and early surgical intervention is essential to prevent irreversible damage to the nervous system. [Indexed for MEDLINE] Free full text [ncbi.nlm.nih.gov]
Idiopathic HSP is a clinical emergency and early surgical intervention is essential to prevent irreversible damage to the nervous system. [jstage.jst.go.jp]
Early institution and long-term maintenance of steroid therapy prevents neurologic sequelae. Occurrence of abdominal inflammatory pseudotumor in a patient of HP possibly as part of multifocal fibrosclerosis has not been described earlier. [annalsofian.org]