Arq Neuropsiquiatr 2005;63(3-A):666-669 CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY Two cases with cervical spinal cord compression Marcos R.G. de Freitas1, Osvaldo J.M. Nascimento1, Cristiane N. Soares2, Adriana Rocha Brito2, Romeu Cortes Domingues3 ABSTRACT - Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a peripheral nerve dis- order probably due to an immunological disturb. It evolves either in a steadily progressive or in a relaps- ing and fluctuating course. Weakness is mainly in the lower limbs proximally and distally. The electro m y o- graphy is demyelinating. The cerebral spinal fluid protein is most of times elevated. Sometimes enlarged nerves are found. There are few cases described with spinal cord compression due to hypertrophic spinal n e rve roots. Two patients (females, 66 and 67 years old) with diagnosis of a long standing CIDP are described. In the first one, the evolution was characterized by remission and relapsing course. The second patient had a chronic and pro g ressive course. These patients presented after a long evolution a cervical spinal cord c o m p ression syndrome due to hypert rophic cervical roots. Neurologists must be aware of the possibility of development of spinal cord compression by enlarged spinal roots in patients with a long standing CIDP. KEY WORDS: chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), spinal root hypert ro- phy, spinal cord compression. P o l i rr a d i c u l o n e u ropatia desmielinizante inflamatória crônica: dois casos com síndrome de com- pressão medular RESUMO - A polirr a d i c u l o n e u ropatia desmielinizante inflamatória crônica (PDIC) é uma afecção dos ner- vos periféricos de natureza autoimune, com evolução por surtos de exacerbação e remissão ou de evolver p ro g ressivo. O acometimento motor é predominante, com fraqueza proximal e distal nos membros infe- riores. A eletroneuromiografia é do tipo desmielinizante com bloqueio de condução nervosa em dois ou mais nervos. Há aumento de proteínas do líquor. Com a evolução da doença pode haver espessamento dos n e rvos distal e/ou proximalmente. Excepcionalmente ocorre compressão da medula espinhal em qualquer segmento por raízes próximas hipert rofiadas. Foram estudadas duas mulheres de 66 e 67 anos re s p e c t i v a- mente com quadro de PDIC de longa evolução. A primeira tinha evolução por surtos e na segunda o evolver era pro g ressivo. Nos dois casos o espessamento proximal dos nervos provocou síndrome de compre s s ã o medular alta. Esta complicação deve ser pensada em casos de PDIC de longa duração. PA L AV R A S - C H AVE: polineuropatia inflamatória desmielinizante crônica (PIDC),hipert rofia de raízes ner- vosas, compressão medular. C h ronic inflammatory demyelinating polyradi- b rospinal fluid (CSF) demonstrates albuminouscy- culoneuropathy (CIDP) usually presents as a more tologic dissociation and nerve conduction studies or less symmetric sensorimotor polyradiculoneuro- reveals multifocal conduction slowing, conduction pathy with chronic relapsing or remitting or pro- block and temporal dispersion. The nerve biopsy g ressive course. There is no clear estimate of its f re- shows primary segmental demyelination and axon- quency but it may re p resent as many as 10%-30% al degeneration with or without inflammatory of previously undiagnosed cases of polyneuropa- infiltration and onion bulbs2 , 3. The therapeutic may t h y 1. Usually there is predominance of weakness be considered a confirm a t o ry diagnostic criterion with diffuse hyporeflexia or areflexia. The cere- and consists of immune modulating agents includ- N e u romuscular Disease Unit, Department of Neuro l o g y, Hospital Universitário Antonio Pedro da Universidade Federal Fluminense, Niterói RJ, Brazil (UFF): 1Full Professor; 2Master Student; 3Radiologist. Received 4 November 2004, received in final form 2 February 2005. Accepted 1 April 2005. Dr. Marcos R.G. de Freitas - Rua Gastão Ruch 16 / 1402 - 24220-100 Niterói RJ - Brasil. E-mail: [email protected] Arq Neuropsiquiatr 2005;63(3-A) 667 i n g c o r t i c o s t e r o i d s , p l a s m a e x c h a n g e a n d i n t r a - couldnot walk. The tonus was increased in lower limbs venous immune globulin4. Occasionally the re p e t- and a sensitive cervical level to painful-touch sensation itive demyelination and remyelination with onion could be found. The tendon reflexes were abolished bulb formation results in gross enlargement of spi- and there were withdraw reflexes with bilateral Babinski nal nerves end roots. CIDP is one of the main cau- s ign. A ce rv ic a l ma gne ti c res onanc e ima ge (MRI ) re v e a l e d h y p e rt rophy of cervical spinal roots, with spinal com- s e s o f t h e h y p e r t rop h i c n e u r o p a t h y 5. Thickened p ression, enhanced with gadolinium (Fig 1). Stero i d s , peripheral nerves were seen in 11% in one larg e imunoglobulin and plasma exchange were given with s e r i e s 3. Although exceedingly rare, there have been no improvement. re c o rdedcases of CIDP presenting with spinal cord 5-13 compression due hypertrophic spinal roots . Case 2 – A 67-year-old woman presented in 1981 a We re p o rttwo patients with CIDP of long evo- c e rvical pain irradiating to the left arm. She was sub- lution with cervical spinal compression due to mitted to a myelography and a cervical spine surg e ry hypertrophic roots. showed hypert rophic cervical roots. In 1983 she had a low back pain radiating to posterior surface of right CASES thigh and weakness of this limb. A laminectomy was Case 1 – A 66-year-old black woman had re c u rre n t performed with some relief of the pain. There is no ref- p a resthesias and weakness in hands and feet was first e rence of the neurological examination in this period. seen in 1986. At that time she had distal tetrapare s i s Five years after she complained of pain and asymmet- with abnormal gait, reduced tendon reflexes, proprio- ric weakness of all limbs, and stopped walking. No sim- ceptive ataxia and superficial hypoesthesia in her legs. ilar cases in the family are re p o rted. There were distal The peripheral nerves were not thickened. Tonus, coor- amyotrophy in all 4 limbs with slight deformity of the dination and cranial nerves were normal. An electro d i- left hand, distal and proximal tetraparesis more severe agnostic evaluation showed a sensorimotor demyeli- in the lower limbs. Deep tendon reflexes were absent nating polyneuropathy features: absence of sensitive in lower limbs. There were superficial hypoesthesia responses, prolonged distal motor latencies and con- below the knees and loss of vibratory sense in lower duction block in bilateral median and ulnar nerves, seve- limbs and in the inner aspects of the left fore a rm and re slowing of motor conduction velocity and abolished hand. The ulnar and posterior auricular nerves were uni- F waves. Needle electromyography (EMG) showed active f o rmly palpable and thick. The CSF showed 1 cell/mm3 d e n e rvation in distal limbs. CSF examination re v e a l e d and 48 mg/dl of protein. The tonus was increased in the albuminous-cytologic dissociation and a sural biopsy lower limbs. There was bilateral Babinski sign. The left showed demyelination and remyelination feature s , biceps reflex was present and the other tendon reflex- axonal regeneration and presence of some onion bulbs. es were absent. A nerve conduction study revealed a T h e re was no duplication in the PMP22 gene. The pati- generalized slowing of motor conduction and a con- ent was treated successfully with prednisone; however, duction block in ulnar and median nerves, the sensory t h e re were subsequent relapsing courses. Over a peri- n e rve action potentials were abolished in the sural, ulnar od of 11 years she had been maintained in alternating and median nerves and the F waves were prolonged in treatment with steroids and plasma exchange. In 2002 most nerves. The EMG was not performed. Sural nerve her symptoms worsened. She became tetraparetic and biopsy demonstrated a great loss of fibers, some inflam- Fig 1. Case 1. Axial and coronal T2 weighted cervical spinal MRI showing roots hypert ro p h y. 668 Arq Neuropsiquiatr 2005;63(3-A) Fig 2. Case 2. Sural nerve biopsy. A) Presence of perivascular inflammatory infiltration (hematoxylin-eosin stain, X400). B) Semi-thin sections showing onion bulbs (toluidine blue stain, X400). m a t o ry infiltrates, fibrosis, clusters of regenerated axons beginning of the disease, first in cervical level and as well as thinly myelinated axons and many onion bulbs two years after in lumbar level. The most striking (Fig 2). Ultrathin sections showed similar features. The f e a t u re was the diffuse, marked enlargement of patient was put on 60 mg prednisone per day, and 30 peripheral roots, demonstrated in one case by MRI days after she could walk without support with gre a t and the other by necropsy studies. They had an i m p rovement of the strength and sensation. She had unusual clinical picture of cervical spinal cord com- been maintained with prednisone and intravenous metil- pression determined by CIDP. p rednisolone for 10 years. In 1999 there was worsening of her neurological examination, showing paraplegia Spine MRI is a valuable addition to the diagnos- with increased muscle tonus, bilateral Babinski sign and tic armamentarium in CIDP.