Neurological syndromes Neurological syndromes in systemic diseases and in systemic diseases and
general medicine general medicine
Marta Lipowska 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
Ischemia Hypoxia Metabolic abnormalites Brain dysfunction
headache,
disorder of consciousness:
(drowsinessconfusion, 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
• Different
abnormalities
• Electrocerebral silence
Worse ence- phalo- pathy
Peripheral aquired neuropathies 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 Signs and symptoms of
neuropathy neuropathy
• Motor deficit- flaccid weakness
• Sensory deficit- pain, paresthesia, sensory loss, ataxia
• Autonomic involvement
Distribution of neuropathy Distribution of neuropathy
• Polyneuropathy- symetric, diffuse lesions of Polyneuropathy- symetric, diffuse lesions of peripheral nerves, distal
peripheral nerves, distal
• Mononeuropathy- disorder of a single nerveMononeuropathy- disorder of a single nerve
• Mononeuropathy multiplex – focal involvement Mononeuropathy multiplex – focal involvement of two or more nerves
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
Lung diseases
• Pneumonia
– 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
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
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
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
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 Neurogenic diabetic foot- pathogenesis
pain and temperature sensory loss – susceptibility to injurespain and temperature sensory loss – susceptibility to injures
autonomic nerves involvement –dry skin, vascular autoregulation disturbencesautonomic nerves involvement –dry skin, vascular autoregulation disturbences
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)
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-
