Progresívna nekrotizujúca myelopati a – kazuistika

Progressive Necrotizing Myelopathy – a Case Report

Progressive necrotizing myelopathy is an extremely rare disease, even considered among non-compressive myelopathies. Spinal cord symptoms are characteristic of it, with the peripheral motor neuron severely affected. A typical cerebrospinal fluid finding is increased total protein without oligoclonal bands, sometimes with mild pleocytosis. A specific MRI criterion is the involvement of at least three or more neighbouring vertebral spinal cord segments with hypersignal in the TW2 picture and gadolinium enhancement, evolving over months into atrophy, which corresponds to spinal cord necrosis confirmed on bioptic examination. The range of differential diagnosis is wide. Apart from other more common diseases (multiple sclerosis, inflammatory, neoplastic, systemic, vascular and metabolic causes of myelopathy), rare paraneoplastic spinal cord involvement must also be excluded. Recently, autoimmune pathogenetic mechanisms related to neuromyelitis optica have been considered, based on the occurrence of specific NMO IgG antibodies against aquaporin 4. The disease has a progressive course, although immunomodulatory treatment may possibly prove therapeutic. The authors present the case report of a 67-year-old man with noticeably atopic terrain revealed on immunological examination, fulfilling clinical and MRI criteria for progressive necrotizing myelopathy. Treatment with corticoids had no effect and was therefore followed by intravenous immunoglobulins, which resulted in a temporary improvement in the clinical condition and an improvement in the serious motor deficiency. Two years after the first manifestation of neurological symptoms, a conventional type of carcinoma was diagnosed in the patient from gr. II WHO kidney cells. As a consequence, in spite of repeated negative findings for antineuronal antibodies, we have to consider a possible paraneoplastic origin for the progressive necrotizing myelopathy.

Key words:
progressive necrotizing myelopathy – neuromyelitis optica – paraneoplastic syndrome – intravenous immunoglobulins

Autori: I. Lisá ¹; V. Belan ²; D. Podhoranská ¹
Pôsobisko autorov: II. Neurologická klinika LF UK a FNsP Bratislava, 2Radiodiagnostická klinika SZU a FNsP Bratislava ¹
Vyšlo v časopise: Cesk Slov Neurol N 2009; 72/105(3): 265-269
Kategória: Case Report

Súhrn

Progresívna nekrotizujúca myelopati a je v rámci nekompresívnych myelopati í veľmi zri edkavé ochoreni e. Charakteristickým klinickým nálezom je mi echová symptomatológi a s dominujúcim postihnutím periférneho motone urónu. V likvore je typický nález so zvýšenými celkovými bi elkovinami a neprítomnými oligoklonálnymi pásmi, ni ekedy i s mi erno u plei ocytózo u. Špecifickým MR kritéri om je rozšíreni e aspoň troch a vi acerých susedných vertebrálnych segmentov mi echy s hypersignálom v TW2 obraze a s enhancementom po kontrastnej látke v pri ebehu mesi acov vystri edané atrofi o u, čo zodpovedá nekróze mi echy potvrdenej pri bi optickom vyšetrení. Diferenci álna di agnostika je široká. Treba vylúčiť okrem častejších ochorení (SM, zápalové, ne oplastické, systémové, ci evne a metabolické príčiny myelopati e) aj zri edkavé parane oplastické postihnuti e mi echy. V najnovších prácach sa zvažuje a uto imunitný patomechanizmus so vzťahom ku ne uromyelitis optica na základe výskytu špecifických IgG protilátok proti aqu aporínu 4. Ochoreni e má progresívny charakter s možným terape utickým efektom imunomodulačnej li ečby. Autori prezentujú kazuistiku 67- ročného paci enta s výrazne atopickým terénom pri imunologickom vyšetrení, spĺňajúceho klinické aj MR kritéri á progresívnej nekrotizujúcej myelopati e. Li ečba kortiko idmi bola bez efektu, preto nasadená li ečba intravenóznymi imunoglobulínmi, ktorá vi edla ku prechodnému zlepšeni u klinického stavu so zlepšením ťažkého motorického deficitu. Dva roky po zači atku manifestáci e ne urologickej symptomatológi e bol u paci enta di agnostikovaný konvenčný typ karcinómu z buni ek obličky gr II WHO, prečo i napri ek opakovane negatívnym nálezom antine uronálnych protilátok musíme zvažovať možný parane oplastický pôvod progresívnej nekrotizujúcej myelopati e.

Kľúčové slová:
progresívna nekrotizujúca myelopatia – neuromyelitis optica – paraneoplastický syndrom – intravenózne imunoglobulíny

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MUDr. Iveta Lisá, PhD.
II. Neurologická klinika LF UK a FNsP Bratislava
Limbová 5
833 05 Bratislava
Slovenská republika
e-mail: l.lisy@hotmail.com

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