Mielita Cervicală – doctor Mihaela Antoce

Definitie
Mielita ( gr. myelón = “maduva spinarii” si sufixul –itis=inflamatie) reprezinta o afectiune neurologica caracterizata prin inflamatie localizata strict la nivelul maduvei spinarii.[1]

Istoric
In 1882 au fost descrise cateva cazuri de mielita , cauzate de leziuni vasculare sau de inflamatie acuta.
In 1892 Osler descrie un caz de polinevrita acuta post-infectioasa, iar in 1922, savanti din Anglia si Olanda descriu un caz de encefalomielita post vaccinare anti-poliomielita. In 1928 apare ipoteza conform careia mielita ar fi o boala post infectioasa, explicata prin observatia ca simptomele apar dupa disparitia febrei sau a eruptiilor cutanate. A fost propusa ideea conform careia mielita acuta ar fi un raspuns alergic la o agresiune virala. In 1948 , termenul de mielita acuta transversa a fost utilizat pentru a descrie un caz de mielopatie inflamatorie acuta fulminanta, complicatie a unei pneumonii. [3]

Epidemiologie
Boala este rara , inregistrandu se 1-4 cazuri/1 milion locuitori, cu distributie preponderenta intre 10-19 ani si 30-39 ani. Exista o predominanta feminina.
Mecanism de producere
Cauzele exacte ale bolii nu sunt bine cunoscute. Cercetatorii considera ca , in urma unei agresiuni asupra organismului, sistemul imun va ataca propriile lui structuri ducand la inflamatia specifica mielitei. Procesul are la baza o asemanarea existenta intre agentul infectios si structura maduvei spinarii (mimicrie moleculara): de exemplu exista o reactie incrucisata intre gangliozide din structura sistemului nervos periferic si zaharide din strcutura Campylobacter jejuni. [4]
Campylobacter jejuni-induced acute transverse myelitis
I Baar1, B C Jacobs2, N Govers1, P G Jorens3, P M Parizel4 and P Cras1
‘Campylobacter jejuni infection is related to various syndromes in which the peripheral nervous system is involved. An immune response is triggered through molecular mimicry between gangliosides of the peripheral nervous system and lipo-oligosaccharides of C. jejuni. We report a case of a previously healthy 17-year-old girl, who developed clinical manifestations of acute transverse myelitis (ATM) 7 days after a culture-proven C. jejuni enteritis. High titres of serum IgG antibodies to the ganglioside GM1 were found in the acute phase of disease, which decreased with clinical recovery. These antibodies cross-reacted with C. jejuni lipo-oligosaccharides, indicating that C. jejuni infections may induce ATM.”

Cauze

a) mielite virotice determinate de virusul gripal, virusul Epstein-Barr sau citomegalovirus, post afectiuni eruptive : rujeola, varicela, rubeola, oreion sau asociate cu hepatita A.

b) mielite acute microbiene: cauzate de stafilococi, streptococci, gonococ, punctul de plecare putand fi un abces, furuncul; se poate asocia si cu tuberculoza, sifilis sau alti paraziti sau protozoare.

c) mielite alergice care apar post vaccine si seroterapie(in special antirabica)

d) mielite asociate cu vasculitele: lupus eritematos sistemic, sindrom Sjogren, panarterita nodoasa.

e)mielite asociate unor boli demielinizante: encefalomielita diseminata, neuromielita optica

f) mielite idiopatice la care etiologie nu este cunoscuta .[2]

Anatomia regiunii cervicale:
Maduva spinarii este o extensie a sistemului nervos central care realizeaza legatura intre creier si restul structurilor corpului prin fibre nervoase. Este o structura cilindrica la nivelul careia se transporta informatie in ambele sensuri:
-descendent: comenzile de la nivelul cortexului pentru miscarile voluntare ale membrelor, trunchiului si gatului
-ascendent: impulsurile pentru senzatiile generale, precum cea tactila sau dureroasa sunt conduse spre cortex , unde vor fi integrate unor procese complexe.
Maduva spinarii de la nivel cervical este protejata de un inel osos format de cele 7 vertebre cervicale( primele doua vertebre avand nume proprii:C1- atlas, C2-axis). La nivelul C4 exista umflatura brahiala, locul corespunzand plexului brahial, principal sursa a nervilor pentru membrele superioare. De asemenea la C4 isi are originea si nervul frenic, care asigura inervatia principalului muschi implicat in respiratie-diafragmul.
Exista 8 segmente cervicale de la nivelul carora ies nervii spinali corespunzatori. Deoarece exista numai 7 vertebre cervicale, nervul spinal 8 iese intre C7-T1.
In interior, maduva prezinta la interior substanta cenusie continand neuroni specializati impreuna cu tracturile ascendente sau descendente , iar la exterior substanta alba continand prelungirile neuronilor din substanta cenusie. Substanta cenusie are un corn posterior-partea senzoriala si un corn anterior-partea motorie.[1]

Simptome:
Polimorfismul clinic al mielitelor este determinat pe de o parte de formele clasice evolutive :acute, subacute si cornice si pe de alta parte , de tipul fundamental si repartizarea leziunilor, putandu se realiza tablouri clinice de sectiune fiziologica complete sau incomplete (sindromul Brown-Sequard).[4]
Simptomatologia poate debuta acut (in cateva ore sau zile) sau subacut (intre 1-4 saptamani).[5]
E dominata de rahialgii, febra, parestezii ,tulburari de sensibilitate cu nivel, tetraplegia flasca sub nivelul leziunii , uneori cu grave tulburari respiratorii (lezarea frenicului C4). In cazul in care afecteaza si maduva toracala in cadrul unei mielite cervico-toracale, bolnavul poate prezenta si un sindrom Claude–Bernard-Horner(leziunea centrului cilio-spinal C8-T2).[4].
Durerea reprezinta principalul simptom de adresare catre medic si apare la aproximativ 1/3 din pacienti. E puternica, cu character penetrant si iradiaza in brate sau torace.
80% din pacienti prezinta zone de hipersensibilitate la atingere si presiune (alodinie)[5]
Simptomatologia poate cuprinde si alte etaje medulare, supraadaugand tulburari sfincteriene.

 

Diagnostic
1) Examen neurologic: durere, tulburari de sensibilitate cu nivel , deficite motorii .
2) Examen biologic: VSH ,fibrinogen , proteina C reactiva si hemoleucograma modificate.
3) Examen lichid cefalorahidian:pleiocitoza discrete sau moderata (pana la cateva elemente/mmc cu granulocitoza); o pleiocitoza marcata se realizeaza cand inflamatia cuprinde si memingele, albuminorahie crescuta pana la 3-4 g cu discociatie albumin-citologica.
4) Imagistic: prezenta la examenul RMN a diverse zone de hipersemnal care capteaza gadolinium reprezentand focare inflamatorii cu localizare cervicala; daca nu se poate realiza RMN (prezenta de peacemaker cardiac) se efectueaza CT coloana cervicala.[6]

De asemenea se incearca eliminarea unor posibile cauze tratabile ale mielitei cervicale:tumori, abcese, hemoragii, utilizand examene imagistice.
Se dozeaza marker pentru patologii precum:lupus eritematos sistemic, infectie HIV, deficit de vitamin B-12.

In caz ca se exclude prezenta unei afectiuni specifice, pacientul va fi incadrat cu diagnosticul de mielita cervicala idiopatica. [5]

Criterii de diagnostic ale mielitei cervicale idiopatice[7]

Evolutie
Evolutia depinde de intensitatea leziunilor.
In cazul unei leziuni ce realizeaza o sectiune incompleta a maduvei , dupa starea de soc medular (explicabila prin fenomene de edem tranzitorii), apar fenomenele de automatism medular cu regresiunea partiala a tulburarilor motorii si de sensibilitate aflate sub nivelul leziunii. Se instaleaza reflexe de aparare, hipertonie piramidala, reflexe osteo-tendinoase vii, reflex Babinski present bilateral, constituindu se o plegie spastica in final.
In cazul unei leziuni cu sectiune definitive a maduvei, dupa starea de soc medular, se instaleaza oarecare fenomene de automatism medular, reflexe osteotendinoase vii, dar motilitatea si sensibilitatea raman compromise total. Aceasta stare sfarseste de obicei letal, prin casexie, in aproximativ 2 ani.
Intr un numar redus de cazuri, simptomele medulare instalate progreseaza ascendent cuprinzand structuri din trunchiul cerebral care controleaza functii respiratorii si cardiac, cu evolutie spre exitus=paralizia ascendenta de tip Landry. Uneori poate inregistra si un mers favorabil, fenomenele clinice retrocedand.[4]

 

Tratament
Tratamentul este simptomatic, urmarind sa reduca inflamatia si intensitatea simptomatologiei, ajutand la vindecarea naturala.
Exista mai multe directii pe care acest tratament le urmareste:
1) Tratament igieno-dietetic: se impun masuri de supraveghere a tegumentelor si mucoaselor, de prevenire si tratament al escarelor, supraveghere a functiilor vitale.
2) Tratamentul etiologic: este posibil doar in mielitele microbiene, in mielita din lupusul eritematos sistemic se incearca pulsterapie cu metilprednisolon/3 zile si ciclofosfamida.[6]
3) Tratamentul patogenic: tinteste sa combata pe de o parte procesul inflamator, edematos si alergic, iar pe de alta parte procesul de degenerescenta nervoasa. Desi studiile clinice nu au evidentiat un beneficiu sigur al corticoterapiei, aceasta e utilizata frecvent pentru a reduce activitatea sistemului imun. Se utilizeaza metilprednisolon administrat intravenous sau dexametazona timp de 5 zile, continuand la domiciliu cu terapie cortizonica orala. Pentru cazurile care nu raspund la aceasta terapie se utilizeaza schimbul plasmatic.[2] Vitaminele din grupul B intervin in metabolismul glucidic si proteic si contribuie la refacerea structurilor nervoase lezate. Se mai utilizeaza si tratamentul cu imunoglobulina umana intravenous cu rezultate discutabile.
4) Tratament simptomatic: tulburarile respiratorii care pun viata pacientului in pericol, oblige la terapia de reanimare.Febra nu trebuie tratata decat la valori foarte ridicate, caci pare sa defavorizeze infectia virala
5) Tratamentul recuperator: dupa trecerea perioade acute este necesara mobilizarea pasiva si masajul segmentelor paralizate pentru evitarea retractiilor tendinoase si a atitudinilor vicioase. In faza spastica se administreaza miorelaxante( Mydocalm, Cloroxazona),kinesiterapia activa si pasiva, gimnastica medicala in servicii de recuperare neuromotorie.[4]
Terapia de recuperare joaca un rol crucial in existenta ulterioara a individului, permitandu i sa invete sa supravietuiasca cu noile probleme dobandite sau sa I asigure un grad variabil de independenta.
O buna terapie de recuperare ar trebuie sa urmareasca si dimensiunea emotionala a problemei, pacientii experimentand depresia ca reactie de adaptare la noua situatie.
Pacientii vor fi ajutati in centre specializate sa si imbunatateasca forta musculara si rezistenta la efort, coordonarea si sa si reduca spasticitatea si gradul de atrofie musculara, precum si sa si imbunatateasca controlul sfincterian.[3]
Prognostic
Recuperarea incepe cam in a doua saptamana de la debutul bolii si poate dura pana la 2 ani. De obicei , daca nu exista o imbunatatire a simptomatologiei intre 3-6 luni, recuperarea semnificativa are o probabilitate mica. 1/3 din pacienti se recupereaza aproape total, 1/3 raman cu diverse deficite de tipul:mers spastic, parestezii, durere cronica, restul ajung in scaunul cu rotile si dependenti de apartinatori pentru efectuarea sarcinilor zilnice.
Un debut rapid al simptomatologiei se asociaza de regula cu un prognostic prost. De regula pacientii experimenteaza un singur episod de mielita, cazurile de recurenta fiind rare si necesitand investigarea unei patologii autoimmune asociate si administrarea de imunosupresive.

 

Studii de caz

1) – persoana de sex feminin care dezvolta paralizie flasca ascendenta post infectie respiratorie
- diagnosticul a fost de mielita cervicala cu herpes simplex
Cervical myelitis from herpes simplex virus type 1.
Mewasingh LD, Christiaens FJ, Dachy B, Christophe C, Dan B.
Department of Paediatric Neurology, Hôpital Universitaire des Enfants Reine Fabiola, Université Libre de Bruxelles, Brussels, Belgium.
Pediatr Neurol. 2004 Sep;31(3):234.
‘ Although subacute ascending paralysis without sensory involvement is typically evocative of Guillain-Barré syndrome, it can alternatively be due to infection or inflammation of the spinal cord. We describe a 16-month-old female who presented with ascending flaccid paresis after an upper respiratory tract infection. She then developed signs of upper motor neuron involvement of the lower limbs associated with upper motor neuron involvement of the upper limbs. Motor nerve conduction and electromyographic studies of upper limbs demonstrated anterior horn cell involvement. Neuroimaging was consistent with cervical myelitis, and cerebrospinal fluid polymerase chain reaction was positive for herpesvirus-1. Although association with the primary infection of the respiratory tract may be fortuitous, possible neurotropic or hematogenous spread of herpesvirus-1 to the cervical spinal cord cannot be excluded. She then developed signs of upper motor neuron involvement of the lower limbs associatred with lower motor neuron involvement of the upper limbs.’

2) – barbat de 55 de ani prezinta parestezii la nivelul membrului inferior stang
- e diagnosticat cu mielita cervicala cu Toxocara
Eosinophilic Myelitis in the Cervical Cord Mimicking Intramedullary Cord Tumor
Cheon Wook Park, M.D., Woo Jin Choe, M.D., Ph.D., and Young Il Chun, M.D.
Published online 2012 October 22
A 55-year-old man was referred from local hospital due to tingling sensation in the left upper extremities and having radiological abnormalities. The original impression was intramedullary astrocytoma. The patient’s motor power was intact, and pathological reflexes were not found. The outside MRI showed HSI lesion with the cord swelling at the level of 4-6th cervical spine and a focal nodular enhancement confined to dorsal segment at C5 (Fig. 3). The SEP study described increased latency and wave
attenuation, whereas the MEP was normal. CSF findings were within the normal range, and no evidence of autoimmune disease was found. Similar to case 1, total IgE was elevated and the response to D. farinae was active in the serum study. IgG for Toxocariasis antigen was also positive. With the impression of eosinophilic myelitis by laboratory, radiological and clinical findings, steroid and albendazole were administered. Three months later, the cord HSI lesion was markedly improved in the follow-up MRI (Fig. 3). The clinical symptom was improved and stable for 8 months.

3) – Pacienta in varsta de 71 de ani cu mielite cervicale recurente tratate cu imunoglobulina intravenous si cu evolutie buna sub tratament
Recurrent myelitis in common variable immunodeficiency successfully managed with high-dose subcutaneous immunoglobulin.
August 2012
Danieli MG, Pettinari L, Marinangeli L, Logullo F.
Scienze Cliniche e Molecolari, Clinica Medica, Torrette di Ancona, Italy.
Acute myelitis is an aetiologically heterogeneous inflammatory disorder of the spinal cord. We report on a 71-year-old woman with a recurrent cervical and thoracic myelitis who presented with a new relapse of the disease. Neuromyelitis optica was ruled out such as other possible causes of acute and/or recurrent myelopathy. Serum immunoglobulin levels and specific antibody responses were consistent with the diagnosis of common variable immunodeficiency (CVID). She was treated with high-dose methylprednisolone and intravenous immunoglobulin. As a remission-maintaining drug, we decided to treat her with subcutaneous immunoglobulin (CSL Behring) at 0.2 g/kg/week at doses higher than usually employed in replacement therapy in CVID. At 3-year follow-up, the response to treatment was good. No relapses occurred. Our case suggests the effectiveness and safety of subcutaneous immunoglobulin in maintaining remission and in sparing prednisone in a woman with recurrent myelitis associated with CVID.

 

4) - pacient in varsta de 17 ani care prezinta imposibilitatea mersului si tulburari de sensibilitate
-e diagnosticat cu mielita cervicala cu virus varicelo-zosterian
-simptomele au disparut fara nici un tratament
Cervical transverse myelitis after chickenpox in an immunocompetent patient.
Rasoolinejad M, Abdi Layali Z, Shojaei E, Kalantari S.
2010 Nov-Dec
Department of Infectious Diseases, Tehran University of Medical Sciences, Tehran, Iran.
Varicella-zoster viruses complications involving the CNS are estimated to occur rarely, transverse myelitis after Varicella-zoster virus in most patients is characterized by an abrupt onset of progressive weakness and sensory disturbance in the lower extremities , like other viruses. We describe the case of 17 year-old boy who experienced cervical transverse myelitis after chickenpox with inability to walk and with urinary retention. He was not treated with any medication but complete recovery has been occurred.

 

5) – un studiu care arata asocierea intre mielita cervicala si lupus eritematos systemic
[Acute transverse myelitis in seven patients with systemic lupus erythematosus.
Gómez-Argüelles JM, Martín-Doimeadios P, Sebastián-De la Cruz F, Romero-Ganuza FJ, Rodríguez-Gómez J, Florensa J, Oliviero A.
2008 Aug
Unidad de Neurología Funcional, Hospital Nacional de Parapléjicos, 45071 Toledo, España.
Abstract
INTRODUCTION:
Although the association between transverse myelitis and systemic lupus erythematosus is rather infrequent, it is important to take this form of clinical presentation into account because it is a serious complication, which can potentially be treated but even when dealt with in the early stages does not always have a good prognosis.
PATIENTS AND METHODS:
We conducted a retrospective review over the past 13 years of the cases that have been admitted to our specific centre for the treatment of spinal cord injuries that were diagnosed as suffering from myelitis associated with disseminated lupus erythematosus. Demographic and clinical data, together with data about acute phase and maintenance treatments, as well as the patients’ progress and sequelae are described.
RESULTS:
The case reports of seven patients, all of whom were young females, are studied. In two cases, myelitis was the initial presentation of lupus. The main disorder was at the dorsal, followed by the cervical, levels. Only two patients had a favourable long-term progression from the neurological point of view (both managed to walk) despite acute treatment with high doses of intravenous corticoids, and regardless of the fact that cyclophosphamide was later used.
CONCLUSIONS:
Myelitis associated to lupus is a rare manifestation but, owing to its important functional repercussions, it must be taken into account when faced with an acute clinical picture involving the spinal cord; this is particularly the case when it occurs in young females, with or without a previous diagnosis of autoimmune disease.

Bibliografie:
1)myelitis.org
2) http://www.ninds.nih.gov/index.htm
3) http://en.wikipedia.org/wiki/Transverse_myelitis
4)Neurologie clinica-Prof .Dr. Felicia Stefanache
5) http://www.ninds.nih.gov/index.htm
6)Neurologie integrala-A.Hufschmidt, C.H.Lucking
7) http://www.slideshare.net/yassermetwally/topic-of-the-monthtransverse-myelitis-presentation

Articol scris de – Doctor Mihaela Antoce