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Spinal Pachymeningitis


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]
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]
Rigor
  • Rigor mortis fairly well-marked. subject of inflammation of the spinal dura mater. Body considerably emaciated.-Head : Vessels of the dura The subject of this case was a labouring lad, aged four- mater full of dark, fluid blood.[kundoc.com]
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]
Upper Limb Weakness
  • Does the presence of cord compression immediately exclude AMSAN, and, if so, how does one explain the upper limb weakness or the areflexia? Diagnostic parsimony in this case could leave the clinician vulnerable and the patient short of a diagnosis.[doi.org]
Urinary Retention
  • The patient suffered from progressive mild thoracic backache followed by truncal and lower extremity weakness, numbness and urinary retention. The diagnosis was confirmed by magnetic resonance (MR) imaging and histopathologic examination.[ncbi.nlm.nih.gov]
  • Results The patient suffered from progressive mild thoracic backache followed by truncal and lower extremity weakness, numbness and urinary retention. The diagnosis was confirmed by magnetic resonance (MR) imaging and histopathologic examination.[springermedizin.de]
  • Case 2 A 68-year-old woman presented with a 2-month history of progressive lower and upper extremity weakness and urinary retention, bilaterally decreased sensation in lower extremities, and a poor rectal tone.[ajnr.org]
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]
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]
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]
Quadriplegia
  • A 67-year-old man presented with progressive quadriplegia. Magnetic resonance imaging (MRI) revealed spinal cord compression by a thickened dura ventral to the cord from C3 to C7.[ncbi.nlm.nih.gov]
  • A neurological examination revealed severe quadriplegia and bilateral positive Babinski signs, numbness and hypoaesthesia below the C5 level. His manual muscle test scores below the level of the deltoid were[yumpu.com]
  • In the second case, a 45-year-old woman presented with fever and quadriplegia after a spine fusion operation due to lumbar spinal stenosis and degenerative herniated lumbar disc.[londonspine.com]
Papilledema
  • Clinical features include headache, cranial nerve impairment, paraparesis or tetraparesis, ataxia, hydrocephalus, papilledema, hemorrhage or secondary vascular occlusions (5).[path.upmc.edu]
  • 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]
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]
Flaccid Paralysis
  • It causes a rapid decrease in the compound muscle action potential and the sensory nerve action potential without evidence of demyelination, leaving patients with an acute severe flaccid paralysis.[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]
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]
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.com]

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. mranasinghe@hmc.psu.edu 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]
Sex distribution
Age distribution

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.com]

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]

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