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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

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
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.
slide3

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

slide4

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

slide5

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

slide6

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

slide7

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

slide8

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

slide9

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

slide10

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

slide12

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

slide13

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

slide14

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

slide15

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

slide16

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

slide17

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