Idiopathic Chronic Hypertrophic Craniocervical Pachymeningitis

Botella, Carlos; Orozco, Manuel Alejandro Meza; Navarro, Jos??; Riesgo, Pedro

doi:10.1097/00006123-199412000-00020

Cited by 21 publications

(29 citation statements)

References 32 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…She was treated with steroid (solumedrol) pulse therapy and followed by maintenance treatment with oral predniso- found intracranially 4) . The spinal form of IHP is rarer and first described by Charcot and Joffroy with its first name as "Pachymeningitis hypertrophica cervicalis" in 1869 1,7,8,[10][11][12] . Charcot and Joffroy described three stages; in the first stage, patients experience local and radicular pain.…”

Section: Casementioning

confidence: 99%

“…This pattern represents more intense enhancement in a peripheral zone of active inflammation than in a central zone of fibrosis 3,4,12) . Linear dural enhancement pattern was known to have better response to steroid therapy than those with nodular pattern because of its less fibrosis and more vascularity 1,3,8,10,12,13) . The typical pathologic features are a thickening of the dura, with nonnecrotizing chronic inflammatory infiltrate of lymphocytes, plasma cell, and, occasionally with histiocytes, giant cells, polymorphonuclear cells or eosinophils 2,3,5,9,14) .…”

Section: Casementioning

confidence: 99%

See 1 more Smart Citation

Idiopathic Hypertrophic Spinal Pachymeningitis : Report of Two Cases and Review of the Literature

Kim

Park

Chin

2011

J Korean Neurosurg Soc

View full text Add to dashboard Cite

392ed 7 months ago and worsened gradually. Her family and past histories were noncontributory. There was no trauma history. On physical examination, motor and sensory function were intact. Her deep tendon reflexes and ankle clonus were checked as normal. There was no documented fever or leukocytosis [White blood cell (WBC) count 6,280/uL]. However, erythrocyte sedimentation rate (ESR) was elevated to 47 mm/hr (normal : 0-20 mm/hr), and C-reactive protein (CRP) level also was elevated to 37.9 mg/L (normal : 0.1-6.0 mg/L). Other laboratory data were within normal ranges and various culture studies revealed no growth. Initial magnetic resonance imaging (MRI) showed an epidural lesion involving from C6 vertebral body level to mid-thoracic spine area with low signal intensity on T1 (Fig. 1A) and T2 (Fig. 1B) weighted images.The patient was treated initially with dexamethasone 5 mg every 6 hours for 7 days, but she had no improvement. Seven days later, follow-up MRI with gadolinium enhancement revealed diffuse homogeneous enhancement (Fig. 2) which was suspected as acute or early subacute stage of epidural hematoma, surgical exploration was done. Total laminectomies of T6 and T7, lesional biopsies were performed immediately. No extradural abnormality was visible during the operation. After INTRODUCTIONIdiopathic hypertrophic spinal pachymeningitis (IHSP) is a comparatively rare disorder characterized by marked inflammatory hypertrophy of the dura mater, with subsequent neurological deficits resulting from the compression of adjacent structures 3) . We present two cases of IHSP with description of etiologies, diagnoses, managements and clinical outcomes. CASE REPORT Case 1A 55-year-old woman visited to our hospital complaining of back pain, progressive paraparesis, both leg numbness, and voiding difficulty in July, 2009. These symptoms had been start- Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disease characterized by hypertrophic inflammation of the dura mater and various clinical courses that are from myelopathy. Although many associated diseases have been suggested, the etiology of IHSP is not well understood. The ideal treatment is controversial. In the first case, a 55-year-old woman presented back pain, progressive paraparesis, both leg numbness, and voiding difficulty. Initial magnetic resonance imaging (MRI) demonstrated an anterior epidural mass lesion involving from C6 to mid-thoracic spine area with low signal intensity on T1 and T2 weighted images. We performed decompressive laminectomy and lesional biopsy. After operation, she was subsequently treated with steroid and could walk unaided. 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. Initial MRI showed anterior and posterior epidural mass lesion from foramen magnum to C4 level. She underwent decompressive laminectomy and durotomy followed by steroid therapy. However, her conditions deteriorate...

show abstract

Section: Casementioning

confidence: 99%

Section: Casementioning

confidence: 99%

Idiopathic Hypertrophic Spinal Pachymeningitis : Report of Two Cases and Review of the Literature

Kim

Park

Chin

2011

J Korean Neurosurg Soc

View full text Add to dashboard Cite

show abstract

“…Males are affected more frequently than females (3:2). It commonly involves the cervical and thoracic dura [1,5,7,12] and presents initially as progressive radicular symptoms, with muscle weakness and atrophy as the second stage, and paraplegia, loss of bladder function, bowel disturbance, and respiratory distress caused by intercostal and diaphragmatic denervation as the third stage [2].…”

Section: Discussionmentioning

confidence: 99%

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

et al. 2011

View full text Add to dashboard Cite

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. We report a case of IHSP followed up for 20 years in a 46-year-old man. Expansive laminoplasty was performed in 1991, and this case has been previously reported by a co-author. After 17 years, the patient's gait disturbance returned. Physical examination and imaging confirmed IHSP that had developed into syringomyelia at the T2-L1 conus level. This case was diagnosed as adhesive spinal arachnoiditis due to pachymeningitis caused by syringomyelia. T1-T4 laminectomy, a syringo-subarachnoid shunt (S-S shunt), and L2-L3 laminectomy were performed. The patient again developed dysesthesia and gait disturbance 3 years after the second operation. Most reports of IHSP have limited their focus to short-term follow-up after initial treatment with no long-term results. At present, there are only five reports referring to long-term results of greater than 5 years. All but one case needed additional surgery. To the best of our knowledge, this is the first case in which syringomyelia occurred in a patient with IHSP. It is important to note that syringomyelia may be a cause of symptom recrudescence during long-term follow-up in IHSP patients.

show abstract

“…A PHIC acomete ambos os sexos e é mais freqüente na 6º década de vida 7 . Os sintomas mais c omuns são cefaléia, decorrente da inflamação da dura-mater ou do aumento da pressão intracraniana, e neuropatia craniana isolada ou múltipla 4 , 8 .…”

Section: Discussionunclassified

Paquimeningite hipertrófica idiopática craniana associada a pseudotumor orbitário: relato de caso

Moura

Pereira

Gonçalves

et al. 2005

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

RESUMO -Paquimeningite hipertrófica se caracteriza por espessamento das meninges, podendo ser dec o rrente de infecção, infiltração tumoral, doença inflamatória ou idiopática. Relatamos sobre um homem de, 40 anos, com queixa de cefaléia de longa data e perda progressiva da visão em ambos os olhos acompanhadas de proptose bilateral. A imagem por ressonância magnética de crânio e órbitas revelou espessamento dural difuso e lesão orbitária bilateral. Extensa investigação não revelou qualquer afecção sistêmica. Estudo anatomopatológico realizado após biópsias de meninges e da massa orbitária evidenciou pro c e s s o inflamatório crônico compatível com paquimeningite hipertrófica idiopática (PHI) e com pseudotumor orbitário respectivamente. Este caso evidencia que o acometimento orbitário pode ocorrer na PHI e que a sua identificação precoce é de fundamental importância para o prognóstico visual.PA L AV R A S -C H AVE: paquimeningite hipertrófica, paquimeningite hipertrófica idiopática craniana, pseudotumor orbitário, neuropatia óptica.Cranial idiopathic hypert rophic pachymeningitis associated with orbital pseudotumor: case re p o rt ABSTRACT -Hypert rophic pachymeningitis is a rare disorder characterized by meningeal thickness, that can be caused by infection, tumoral infiltration, inflammatory disorders or idiopathic. We re p o rt the case of a 40 year-old man that presented with longstanding headache and pro g ressive bilateral visual loss and p roptosis. Cranial and orbital magnetic resonance imaging revealed diffuse dural thickness and bilateral extraconal orbital lesion. Extensive investigation did not reveal any systemic condition. Histopathological study after meningeal and orbital biopsy disclosed a chronic inflammatory process compatible respectively with idiopathic hypert rophic pachymeningitis (IHP) and orbital pseudotumor. This case emphasizes that orbital involvement can occur in IHP and that its early identification is of great importance in order to impro v e the visual prognosis of this condition.KEY WORDS: hypert rophic pachymeningitis, cranial idiopathic hypert rophic pachymeningitis, orbital pseudotumor, optic neuropathy.

show abstract

Idiopathic Chronic Hypertrophic Craniocervical Pachymeningitis

Cited by 21 publications

References 32 publications

Idiopathic Hypertrophic Spinal Pachymeningitis : Report of Two Cases and Review of the Literature

Idiopathic Hypertrophic Spinal Pachymeningitis : Report of Two Cases and Review of the Literature

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

Paquimeningite hipertrófica idiopática craniana associada a pseudotumor orbitário: relato de caso

Contact Info

Product

Resources

About