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Metabolic, toxic, paraneoplastic, neuropathic disorders affecting NS

Metabolic, toxic, paraneoplastic, neuropathic disorders affecting NS. M. Bojar Charles University Prague, 2nd Medical School, Dpt. of Neurology, FN Motol. I. Metabolic, toxic, paraneoplastic, disorders of the CNS a PNS.

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Metabolic, toxic, paraneoplastic, neuropathic disorders affecting NS

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  1. Metabolic, toxic, paraneoplastic, neuropathic disorders affecting NS M. Bojar Charles University Prague, 2nd Medical School, Dpt. of Neurology, FN Motol

  2. I. Metabolic, toxic, paraneoplastic, disorders of the CNS a PNS • Incidence, prevalence -high, rising due to many factors • Etiology – diabetes, thyroid gland • DM neuropathy 10% in the manifestation, 50% of px after 25 l.,thg endocrinopathy. Inter. disorders- liver, kidney. • Abuse + spirits – alcohol, drugs 15%? Inflammatory + infections. Compression + vibrations (P.C., musicians). Medicaments + env. toxins and noxae. • Heredity - HSMN, SCA, inherited metabolic encephalopx • PA – axonopathy (demyel., mixed), neuropathy, neuronopathy, (angiopathy). Encefalo/myelopathy - atrophy, leukoencefalopathy.

  3. Metabolic, toxic, paraneoplastic, disorders of the CNS a PNS • Metabolic - inherited - acquired • Toxic ( industry, environment, drugs, food and water) • Paraneoplastic ( autoimmunity, therapy …) • Hereditary – various inherit.disorders of NS • Combined with hereditary disposition and infectious +parainfectious disorders 3

  4. Diabetic polyneuropathy. 2006 – Treated diabetic pts in ČR n748 528. • DM I.type : 18-29 yr - 18%, > 30 yr 58% • DM II.type :at time of dg. 8.3%, after 10 yr 32 % • Cca 50% diabetes pts have DN, out of them 18% px are symptomatic. • ČR cca 67 400 diabetes px with symptomatic DN • (Pelikánová,Bartoš:Diabetes mellitus minimum pro praxi, Perušičová:Trendy • soudobé diabetologie, Rušavý:Diabetická noha) 4

  5. II. Metabolic, toxic, paraneoplastic, disorders of the CNS a PNS • Clin. symptoms – • Sensitive • Motor • Combined • Distribution • Cr.nn, radiculo/neuropathic. sy - spinal roots, peripher.nn. • Encefalo/myelopathy • Combined 5 5

  6. What is typical for polyneuropathies? PNP – typical features: affect mainly long nn – LE, but UE, too. Entreppement sy – „narrow channels“- carpal tunnel sy, elbow tunnel sy manifest mainly distally. start typically and „silently“in rest, in night, sleep… 6

  7. Subjec. problems, complaints - impaired sensitivity and vegetative system Sensitive neuropathic symptoms – start typically when resting, in the night. Rarely during the day. Irritative, positive : neuropathic pain, itching- paresthesias, hot, dysesthesias, oversensitivity. Restless legs sy, usually night + „fire“ feeling. Sy canalis carpi, entrempement sy… Failure, negative : strange, cold, icy, numb, „wooden“ LE 7

  8. Subjective complaints, troubles – motor, movement functions Motor neuropathic symptoms Irritative, positive : spasms- crampi, fasciculations. Failure, negative : fatigue, heaviness, weakend UE,LE, palsy, unstable gait 8

  9. Neuritis vs. neuropathy ... • Neuropathy – impairment of neurons and axons. • Only non-inflammat. origin– • metabol., toxic, mechanical... • But… • Neuritis – inflammatory impairment of peripheral nerves Sensu lato even neurons. 9

  10. III. Metabolic, toxic, paraneoplastic, disorders of the CNS a PNS • Diagnostics • Illness hist.- Fam H, Epi H, Proff H, ToxH, Travel H. • Biochemistry, CSF. Immunology. Serology, virology • X- rays + NIM - MRI,CaT, US. • EF - EMG, EP - VEP, BAEP, MEP.EEG. ENG. Stabilometry • Biopsy. • Clinical examination, neurostatus 10

  11. Myotatic reflex – old, but useful… 11

  12. Neurologic finding, neurostatus Impaired function of muscles, movements, trophic functions Hyporeflexy or areflexy L5/S2, later areflexia L2/4 Hypotonia and atrophy of distal muscles, mainly LE, espec. msc. interossei Diminished msc. strenght- LE ( gate), later UE( PET bottles , locks, zips ) 12

  13. Paraneoplastic impairment of the NS • Reactivity of the immune system against Tumor tissue/disease and its treatment. Anticancer „surveillance“. • Autoimmune reactions – pre/post tumor manifestation and therapy modifying effx. • Combined reactions – drug-induced, opportune - superinfections, actinotherapy, metabolic disorders, hypovitaminosis, hypo/dyssimunity. 13

  14. Impairment of the NS of toxic-metabolic origin • Toxic – addiction : alcoholism, nicotinism, drugs+ medicaments Exogennous – toxic substances – environmental, industry, agriculture - organofosfates, intoxications - org.sbst. Iatrogennous – cytostatics, antibiotics, immunomodulans, neuropharma drugs Metabol. & organ impairment – liver, renal, amyloidosis… 14

  15. Renal, uremic polyneuropathy • Chronic renal insuficiency – late stage • Distal symmetrical sensitive and motor form – slowly progressive. Koincidence with DM, myeloma, vasculitis • Mononeuropathy affecting dialysed pts. Mainly n.medianus, n.ulnaris + n.peroneus. 15

  16. Hepatal polyneuropathy • Acute – viral hepatitis B, C. Guillain Barré syndrom-like polyradiculoneuritis. • Chronic – hepatopathy, postinfectious, toxic-metabolic, combined. • Mononeuropathy multiplex. • Th. hepatoprotectives, INFa,vitamins (B1,6,12). Abstinency, diet. Rhb., physiotherapy, balneotherapy. 16

  17. Metabolic, toxic, paraneoplastic, infectious impairment of CNS a PNS • Therapy • Causative - against metab. dysfunction, agent, noxa, toxin. Neuroprotection.Vasoactive. SSRI. NMD. AED • Symptomatic- circulation,nutrition. Diet, psychotherapy. • Physiotherapy. Balneotherapy. Spa therapy. • Prognosis – >> chronic-progressive, > deficit. Letality - toxic encefalomyelopathy, infectious, limbic - paraneoplast.encx

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