Department of Neurology UPJŠ LF Košice 16.5.2018 Neurological complications of alcoholism collagenous disorders, electrolyte disturbances and endocrinopathies Department of Neurology UPJŠ LF Košice 16.5.2018

Neurological complications of acute alcohol intoxication and alcoholism

Alcohol and nervous system Acute intoxication Abstinence and withdrawal syndrome Alcohol and dementia, Wernicke´s encefalopathy

Alcohol and nervous system Acute intoxication Changes in behavior, agressivity, incoordination of movement and gait, slurred speach Drowsiness, stupor, coma, problems with breathing

Alcohol and nervous system Abstinence and withdrawal syndrome Tremulousness Hallucinations Epileptic seizures Confusion Psychomotor and autonomic overactivity, which develops several hours or days after an addictive drinker abstains from alcohol

Alcohol and nervous system Delirium tremens In 10% starts with seizures Profound confusion, gross tremor, hallucinations, autonomic overactivity – fever, tachycardia, dilated pupils 48-96 hours after cessation of drinking

Alcohol and nervous system Alcohol and dementia Chronic abuse of alcohol – cognitive deficit (loss of neurons, brain atrophy) Wernicke´s encefalopathy (hemorhagic) Korsakov´s syndrom

Alcohol and nervous system Wernicke´s encefalopathy Confusion, eye movement problems, ataxia – dysarthria, ataxia of body and extremities, residual signs Loss of neurons, axons, myelin, small hemorrhagies – corpora mamillaria, around aqueductus, III. and IV. ventricles Th.: Thiamin 100 mg/day i.m. or i.v. , B-vitamin complex

Alcohol and nervous system Korsakov´s syndrom - amnestic syndrom with amnesia, confabulations Marchiafava-Bignami syndrom - demyelinisation of corpus callosum (MRI - hyperintense lesions Confusion, seizures, dementia http://www.scielo.br/scielo.php

Alcohol and nervous system Alcoholic degeneration of cerebellum- cerebellar atrophy Alcoholic polyneuropathy Alcoholic myopathy: Acute necrotisans myopathy Chronic myopathy

Neurological complications of liver diseases

Liver encefalopathy Increased ammonium in blood Apathy, cognitive disturbances Pyramidal signs Apraxia Unconsciousness Brain MRI- hyperintensive signals

Neurological complications of endocrinopathies

Endocrine disturbances Hypotyreosis Polyneuropathy – axonal type Tinnitus, loss of hearing, dizziness Ptosis Myalgia Hypo- arreflexia (tendon reflexes) – 75% Apathy, concentration problems Problems with memory Depression Seizures

Endocrinne disturbances Hypertyreosis Tyreotoxic myopathy – weakness of proximal muscles Dif.dg. : myasthenia gravis, myopathy Epileptic seizures

Neurological complications of diabetes mellitus

Diabetes mellitus - neurological complications Acute metabolic decompensation: 1. Diabetic ketoacidosis, DM 1. type 2. Hyperosmolar encephalopathy - non-ketotic “coma” , DM 2. type, extreme hyperglycaemia (40–80 mmol/l) with hypernatraemia, hyperosmolality, change of consciousness, stupor or coma, seizures, dystonic movements; these neurological features resolve completely when the metabolic state has returned to normal 3. Cerebral edema - potentially fatal, diabetic ketoacidosis Etiology: electrolyte exchanges in and out of cells with a net influx of sodium into the cells 4. Hypoglycaemia - glucose less than 3 mmol/l; autonomic stimulation, cognitive dysfunction, restless,violent behaviour, unconsciousness, seizures, dysarthria, rarely reversible hemiplegia Chronic diabetic complications: 1. Hyperglycaemic neuropathy 2. Generalised neuropathies 3. Focal and multifocal neuropathies 4. Superimposed chronic inflammatory demyelinating polyneuropathy (CIDP) 5. Cerebrovascular disease, ischaemic stroke 6. Diabetic myopathy

Diabetes mellitus Hypoglycemia General signs, headache Neurological signs – seizures, coma, acute hemiparesis Autonomic symptoms – sweating, nausea, blushing, heart pounding, chest pressure, headache Cerebral symptoms - epi seizures, disturbances of consciousness, focal neurological symptoms - acute hemiparesis Recurrent hypoglycaemia - reduction of intellectual and cognitive functions, dementia

Diabetic polyneuropathy After 5-10 years of DM, 10 % - earlier than DM Pathogenesis: metabolic and ischemic changes of vasa nervorum (hyalinisation) Metabolic changes of peripheral nerves = earlier problems with sensitivity, myelin fibers are resistant against ischemia 1. Hyperglycaemic neuropathy 2. Generalised neuropathies: - Sensorimotor polyneuropathy - Acute painful sensory neuropathy   - Autonomic neuropathy 3. Focal and multifocal neuropathies: Cranial neuropathies, Thoracolumbar radiculoneuropathy, Focal limb neuropathies (including compression and entrapment neuropathies), Proximal diabetic neuropathy 4. Superimposed chronic inflammatory demyelinating polyneuropathy (CIDP)

Diabetic polyneuropathy Symmetric diabetic polyneuropathy Mainly distal localization (LL) Lower extremities – more often affected Loss of proprioception - leads to ataxia Neuropatic pain

Diabetic polyneuropathy Proximal asymmetric diabetic PNP Rare Starts with night pain Proximal (ischias) localization Muscle atrophy Symmetric proximal weakness Mononeuropathy Cranial nerves lesions ANS – impotence, impairment of bladder and bowel function

Neurological complications kidney disorders (uraemia, dialysis) and electrolyte disturbances

Uraemic syndrome and dialysis Uraemia: 1. Encephalopathy 2. Neuropathy - affect cranial, peripheral and autonomic nerves 3. Uraemic proximal myopathy – caused by secondary hyperparathyroidism Etiology: retention of uraemic toxins – main cause of uraemic encephalopathy and neuropathy Chronic dialysis: 1. Dialysis disequilibrium syndrome - can occur in any patient who receives hemodialysis; neurologic deterioration that is seen in patients who undergo hemodialysis during or immediately after their first treatment (abnormal osmotic gradient) Th: starting dialysis with a low dose and progressively increasing the dialysis dose in subsequent dialysis sessions Brain edema symptoms , increased intracranial pressure or acute hyponatremia Seizures , restlessness, headache, mental confussion, increased nervous irritability, spasms, coma 2. Dialysis dementia - results from aluminium overloading 3. Nerve entrapment - carpal tunnel syndrome - caused by beta2-microglobulin amyloidosis

Kidney - Uremia Uremic encephalopathy Concentration problems Cognitive impairment Motor problems Polyeuropathy Seizures, multifocal myoclonus Delirium Coma

Electrolytes disturbances Metabolic encephalopathies Etiology: rapid change of blood osmolality value - over 310 mmol/l or - less than 270 mmol/l

Electrolytes disturbances Hyponatremia a hypoosmolality Water – to CSF and brain cells = brainedema – decreased K+ concentration – decreased action potential at membrane – incerased neuronal irritability Decreased brain blood flow Headache Problems with concentration Halucinations Seizures Coma

Electrolytes disturbances Hypernatremia a hyperosmolality Reason: lack of water Water from brain cells a CSF to a body = decreased brain function Cognitive impairment Focal signs Coma Trombosis of capillaries

Central pontinne myelinolysis Pontinne demyelinisation (Osmotic demyelinisation syndrome) Rapid change of the electrolytes level Pontinne syndrome: Dysphagia, dysarthria, tetraparesis Oculomotor disturbances Occurance: often in alcoholics after rapid hyponatriemy correction

Neurological complications of collagenous disorders

Neurological complications of collagenous (rheumatic) disorders Central and peripheral NS is affected The spectrum of neurological complications is very broad and variable - Vascular disease (microangiopathy, macroangiopathy, vasculitis) of the brain, spinal cord, and peripheral nerves   Compressive neuropathy (peripheral nerves) Rheumatoid arthritis /RA/- myelitis SLE - neuropsychiatric disorders http://imagebank.asrs.org/file/3465/lupus-vasculitis

Systemic lupus erythematodes SLE, multiorgan disease: rheumatologic, vascular, skin, heart, pulmonary, renal and neuro-psychological symptoms Neurological complications - 25-75 % (central and peripheral NS), in 3 % - the first manifestation SLE Headache Mood problems – depression, anxiety Confussion, halucinations, psychosis, mental and behavioral problems Hemiparesis, paraparesis, aphasia Myelopathy- transversal spinal cord lesions Mononeuritis (e.g. optic neuritis), Polyneuritis (polyneuropathy) Polymyositis, myalgie Stroke - brain infarctions Cognitive deficit – attention problems, dementia (15 – 66 %) Uncousciousness, epileptic seizures Cranial nerves lesions (optic nerve) Dyskinesis (chorea)

Systemic lupus erythematodes Causes of brain infarctions Lupoid-like anticoagulans antiphospholipid antibodies (LA, aCLA) Cardiogenous embolisation to the brain Vasculitis CSF – pleocytosis, OCB positive EEG – diffuse abnormalities Brain MRI – multiple subcortical white matter lesions

Rheumatoid arthritis (RA) Neurological complications - due to vasculitis, inflammatory cells infiltration, or rheumatic nodes Symptoms: Sensitive problems, paresthesias, paresis, epileptic seizures, cranial nerve lesions Spinal cord and spinal root compression –myelopathy, radiculopathy Atlanto-axial arthritis a subluxation - loosening the transverse tendon leads to cervical spine compression Kompressive neuropathies - peripheral nerve compression at anatomically predilectional sites and proliferative changes in joints (paraesthesia, pain, sensory and motor deficit in the innervation zone of the damaged nerve), most often n. median in the carpal tunnel Vasculitis may affect n. peroneus and n. ulnaris, or polyneuropathy

RA MRI- cervical spinal cord: Dnes axis dorsal deviation and shift Basilar impression due to atlanto-axial arthritis in a patient with RA Dens axis compresses anterior part of medulla oblongata and cervical spinal cord

Sjögren syndrome (SS) Visceral disorders: pulmonary, cardial, renal, lymphadenopathy, hepatosplenomegaly, central and peripheral nervous system Senzitive polyneuropathy or Senzitive-motoric polyneuropathy Senzitive ganglionitis - pain, ataxia Trigeminal nerve neuropathy and  CIDP – rarely Stroke Transverzal myelitis – truncal sense problems, autonomic disturbance, urinary retention Pupiler abnormalities Dermatomyozitis and polymyositis – rarely

NeuroBehçet disease Nervous system complications in 5 – 10 % Symptoms: Aseptic meningitis Encephalitis Intracranial venous and sinus thrombosis Peripheral neuropathy - rarely

Sclerodermia (Progressive systemic sclerosis) Polyneuropathy Trigeminal nerve neuropathy Compressive neuropathies Focal cerebral lesions, stroke, demyelinations Epileptic seizures Encephalopathy

Temporal arteritis (Horton´s arteritis) Older patients Vasculitis , large vessels, a. temporalis and a. carotis externa branches Symptoms: severe headache of temporal area, diplopia, blurred vision, or amaurosis fugax Visual problems may lead to blindness (40 %) due to optic nerve ischemia Lab. tests: inflamm.markers (FW, CRP), vessel biopsy – vasculitic changes (infiltration of vessel wall with mononuclear cells, gigantic cells) Th: high doses of corticoids (Prednison 40 – 80 mg/d)

Sarcoidosis The nervous system is affected in about 5% of cases, it may be the first manifestation of sarcoidosis Cranial nerves - mononeuropathy n. abducens or n. facialis Polyneuropathy, Guillain-Barre Syndrome, Plexopathy Bazilar meningitis with multiple cranial nerve lesions Diencephalic syndrome (hypersomnia, diabetes insipidus) Chronic granulomatous pachymeningitis Rarely leads granuloma to hydrocephalus Polyneuropathy Painless granulomatous polymyositis Myelopathy Optic nerve papila edema CSF: increased ACE MRI: thickening of meninges, with postcontrast enhancement