Naslov
Chronic inflammatory demyelinating polyneuropathy associated with central nervous system demyelinization

Autori
Škarpa-Prpić, Ingrid ; Rudež, Josip ; Antonelli, Licia ; Milohanić, Severin ; Sepčić, Juraj

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
European Journal of Neurology Volume 3, Supplement 5, Octobe 1996 / Russell Fionnula - London : Delhi : Rapid Science Publishers, 1996, 208-208

Skup
Second Congress of the European Federation of Neurological Sciences

Mjesto i datum
Rim, Italija, 30.10.1996. - 03.11.1996

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
chronic inflammatory demyelinating polyneuropathy; central nervous system; demyelinization

Sažetak
We presented a patient, 36-year-old white man, with chronic inflammatory demyelinating polyneuropathy with central nervous system (CNS) demyelinization. The disease started in adolescence with diplopia, laesio n. abducentis, intentional tremor, paraesthesia and numbness of all four limbs. The disease had a chronic relapsing-remitting character, the clinical features of which resembled multiple sclerosis. During the follow up period (20 years) the patient gradually developed typical clinical, neurophysiological and pathophysiological features of chronic inflammatory demyelinating polyneuropathy (CIDP). At the present he complains of unsteady gait, paraesthesia and plain of the limbs, specially distal parts and night pollution. A neurologic examination revealed ataxic gait, total areflexia and altered sensory with symmetrical polyneuropathy of all limbs with distal amyotrophic syndrome of upper limbs. Nerve truncus were hypertrophied, especially superficial cervical bundle. EMNG displayed reduction in motor velocity for the ulnaris nerve (10 m/s) and a complete block for the peroneal nerve. VEP and BAER were normal, but SSEP of the medianus nerve demonstrated dysfunction in conduction of somatosensory stimulus at the level of peripheral nerve and cervical medulla. Nerve biopsy (n. auricularis magnus) showed extremely perineurial and endoneurial edema with mononuclear infiltration (immunohystochemistry - T and B lymphocytes) and numerous "Onion-bulb" formation. Various degrees of demyelinization were present between fascicles. Cerebrospinal fluid (CSF) studies showed elevated protein (5g/l), IgG quotient 47.4, IgG intratecal synthesis of 0.74 and present IgG oligoclonal bands. Cell count was 6 mm3 and VDRL negative. CT and MR imaging demonstrated CNS demyelinization. Further studies excluded collagenosis, paraneoplastic syndrome, HIV infection, monoclonal and biclonal gammapathy, toxic, metabolic and familiar polyneuropathy. The course of the disease is chronic with remission and relapses usually followed by stress situation. Corticosteroids and ACTH were effective in treatment of objective symptoms. Our patients confirms the existence of central-peripheral demyelinization syndrome. Inflammatory infiltration of mononuclear cell's cloud represents a common pathopsyhiological ground for both, peripheral and central nervous system demyelization process.

Izvorni jezik
Engleski

Znanstvena područja
Javno zdravstvo i zdravstvena zaštita

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