Neurological syndromes in systemic diseases and general medicine Marta Lipowska

Encephalopathy Brain pathology of varies origins (metabolic, degenerative, genetic, posttraumatic, ischemic, hypoxic…) Generally it affects large parts of the brain instead of leading to identifiable focal changes Often developes when the reason is outside the brain

Encephalopathy Deprivation of oxygen (hypoxia, diffuse ischemia), substrate (hypoglycemia) or metabolic cofactor (thiamine deficiency) Diseases of organs other than the brain: Liver (hepatic coma) Kidney (uremia) Lung (CO2 narcosis) Pancreas Pituitary Thyroid (myxedema, thyrotoxicosis) Adrenal Exogenous poisons Abnormalities of ionic or acid-base environment Disordered temperature regulation (hypothermia, heat stroke) Primary neuronal or glial disorders

disorder of consciousness: Ischemia Hypoxia Metabolic abnormalites Brain dysfunction headache, disorder of consciousness: (drowsinessconfusion, stupor coma) convulsions, involuntary movements (mioclonic) focal neurological syndromes

Glasgow coma scale Estimates the level of consciousness Scores: Eye opening response (1-4 points) Verbal response (1-5 points) Motor response (1-6 points) 3-8 points - coma

Seizures (convulsions) as a part of ecephalopathy Metabolic abnormalities: glucose, Na, Ca, Mg Toxic factors („irritating”) Drugs (neuroleptics) Acute brain disorders (stroke, inflammation, trauma)

a patient with pneumonia (secondary CNS hypoxia) confusion

A patient with encephalopathy- how to diagnose? Blood and urine tests Chest X-ray, other pulmonary investigations Cardiological investigations etc. Sometimes Neurological examinations: CT scan, MRI, CSF

Brain MRI scan in metabolic encephalopathy (renal failure)?

Brain MRI scan in metabolic encephalopathy (renal failure)? EEG reflects level of consciousness (changes in the EEG correlate with the severity of cerebral dysfunction)

EEG Alert drowsy lethargic stuporous comatose brain dead Normal EEG Worse ence-phalo-pathy Alert drowsy lethargic stuporous comatose brain dead Normal EEG Different abnormalities Electrocerebral silence

Peripheral aquired neuropathies metabolic (diabetic, uremic) associated with dietary states Vasculitic (usually associated with systemic diseases) Acromegalic, hypo- hyperthyroid paraneoplastic paraproteinemic

Signs and symptoms of neuropathy Motor deficit- flaccid weakness Sensory deficit- pain, paresthesia, sensory loss, ataxia Autonomic involvement

Distribution of neuropathy Polyneuropathy- symetric, diffuse lesions of peripheral nerves, distal Mononeuropathy- disorder of a single nerve Mononeuropathy multiplex – focal involvement of two or more nerves

Cardiovascular disorders Brain ischemia during: Heart attack Arrhythmia Blood pressure  or  Heart failure Ischemic stroke with embolic material form heart: Artrial fibrillation Aortic or valve mitral disease, specially with bacterial vegetation Bacterial miocarditis Hypertensive encephalopathy

Lung diseases Pneumonia Lung cancer Chronic pulmonary insufficiency hipoxia Risk of bacterial meningitis Lung cancer Paraneoplastic syndromes Metastases Pancoast`s syndrome – superior sulcus lungs tumors – brachial plexus damage Chronic pulmonary insufficiency Noctural headaches

Renal diseases- renal failure Uremic encephalopathy Uremic neuropathy Dialysis dysequilibrium syndrome (usually seen with rapid dialysis at the onset of dialysis program) Headache, nausea, muscle cramps, rarely convulsion, delirium Neurologic complication of renal transplantation CNS lymphoma, unusual infections

Hematologic diseases Leukemias Hemorrhage socondary to thrombocytopenia Infections secondary to low white blood cells counts Tumors of CNS Meningeal invasion (of any acute lymphoma) Complications of chemiotherapy

Hematologic diseases Polycythemia Large nad small vessel cerebral infaction (hyperviscosity) Hemorrhages socondary to thrombocytopenia Platelet disorders (primary or secondary) infarctions and hemorrhages Disorders of coagulation (deficiency of ATIII, factors involved in plasma coagulation) Ischemic stroke in young patiens

POEMS syndrome P-polyneuropathy O-organomegaly E-endocrynopathy M- monoclonal protein S-skin changes (angiomas, hiperpigmentation) In the course of solitary osteosclerotic plasmocytoma

Thyroid – Hypothyroidism Endocrine diseases Thyroid – Hypothyroidism Changes in cognition and level of consciousness  myxedema coma Entrapment neuropathy- carpal tunnel syndrome (attributed to the accumulation of acid mucopolysaccarides in the nerve and surranding tissues)

Thyroid - hyperthyroidism Endocrine diseases Thyroid - hyperthyroidism Ophalmoplegia (Graves disease) Thyreotoxic myopathy – weakness, wasting of the muscles, myasthenic signs Thyreotoxic periodic paralysis Irritation, anxiety

Endocrine diseases Hypoparathyroidism Symptoms of hypocalcemia tetany – muscle spasms, paresthesia Convulsions Irritation, insomia

(the main problem- pain) Diabetes mellitus Diabetic neuropathy- occurs in 80% of patients Distal symetric, predominantly sensory, with or without autonomic manifestations (the main problem- pain) Mononeuropathies Liability to pressure palsies (CTS) Cranial nerve palsies

Neurogenic diabetic foot- pathogenesis pain and temperature sensory loss – susceptibility to injures autonomic nerves involvement –dry skin, vascular autoregulation disturbences other factors

Garland`s syndrome - femoral nerve damage (diabetic focal neuropathy)

Diabetes mellitus stroke risk factor hypo-, hyperglicemia: decrease of consciousness  coma Convulsions Stroke-like episodes (focal syndromes)

Encepalopathy, hepatic coma Liver diseases Chronic liver disease, when portal hypertension induces an excessive portal collateral circulation cerebral intoxication Encepalopathy, hepatic coma

Viral hepatitis type C - extrahepatic manifestations Cryoglobulinemia Peripheral neuropathies

Vitamin B12 (cyjanocobalamin) deficiency Megaloblastic anemia Classic clinical syndrome of subacute degeneration of spinal cord and polineuropathy

Neurologic paraneoplastic syndromes Results from indirect effect of primary malignancy on the nervous system (or other organs) Usually autoimmune cause: The host makes antibodies against antigens in the neoplasm and that these antibodies cross- react with neural antigens (anti- onconeural antibodies, anty –Hu)

Neurologic paraneoplastic syndromes Usually precede primary neoplasm occurence !!! (the most strong immunologic reaction at the beginning) Some patients may have easy controlled neoplasm but die from the neurologic disorder

Neurologic paraneoplastic syndromes May affect any part of peripheral and central nervous system Encephalomyelitis Limbic encephalitis Cerebellar degeneration Opsoclonus Sensory neuropathy Myasthenic Lambert-Eaton syndrome Dermatomyositis and polymyositis

Treatment Treatment of underlying malignancy Immunomodulatory therapy

Collagen-vascular diseases Periarteritis nodosa systemic lupus erythematosus, rheumatoid arthritis, Wegener granulomatosis Churg- Strauss vasculitis Vasculitis syndromes

Vasculitis of central and peripheral vessels of nervous system Encephalopathy- multiple infarcts Vasculitic neuropathy

Alcohol abuse Acute alcohol intoxication – decrease of consciousness (euphoria/dysphoria, slurred speech and ataxic gait, stupor  coma) Ethanol withdrawal syndromes (in dependency) : tremor, hallucinosis, seizures delirium tremens

Chronic alcohol abuse Wernicke-Korsakoff syndrome - Thiamine deficiency : dementia, amnesia, sometimes with confabulation Alcoholic cerebellar degeneration Alcoholic neuropathy, myopathy head trauma Complications of head trauma+withdrawal syndromes- „alcoholic” epilepsy