Metabolic toxic paraneoplastic neuropathic disorders affecting ns
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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.


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

Myotatic reflex – old, but useful…

11


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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


Metabolic toxic paraneoplastic neuropathic disorders affecting ns

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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