Metabolic, toxic, paraneoplastic, neuropathic

disorders affecting NS

M. BojarM. BojarCharles University Prague, Charles University Prague,

2nd Medical School, Dpt. of 2nd Medical School, Dpt. of Neurology, FN MotolNeurology, FN Motol

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.

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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

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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)

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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

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What is typical for polyneuropathies?

1. PNP – typical features:

2. affect mainly long nn – LE, but UE, too. Entreppement sy – „narrow channels“- carpal tunnel sy, elbow tunnel sy

3. manifest mainly distally.

4. start typically and „silently“ in rest, in night, sleep…

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Subjec. problems, complaints - impaired sensitivity and vegetative system

Sensitive neuropathic symptoms – start typically

when resting, in the night. Rarely during the day.1. Irritative, positive : neuropathic pain, itching-

paresthesias, hot, dysesthesias, oversensitivity. Restless legs sy, usually night + „fire“ feeling. Sy canalis carpi, entrempement sy…

2. Failure, negative : strange, cold, icy, numb, „wooden“ LE

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Subjective complaints, troubles – motor, movement functions

Motor neuropathic symptoms1. Irritative, positive : spasms- crampi,

fasciculations.2. Failure, negative : fatigue, heaviness,

weakend UE,LE, palsy, unstable gait

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Neuritis vs. neuropathy ...

Neuritis – inflammatory impairment of

peripheral nerves Sensu lato

even neurons.

Neuropathy – impairment of neurons

and axons. Only non-inflammat.

origin– metabol., toxic,

mechanical... But…

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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

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Myotatic reflex – old, but useful…

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Neurologic finding, neurostatus

Impaired function of muscles, movements, trophic functions

1. Hyporeflexy or areflexy L5/S2, later areflexia L2/4

2. Hypotonia and atrophy of distal muscles, mainly LE, espec. msc. interossei

3. Diminished msc. strenght- LE ( gate), later UE( PET bottles , locks, zips )

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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.

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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…

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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.

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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.

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