EncephalopathyHarsh Gupta, PGY4

O The term “delirium” means “a going off the ploughed track, a madness”.

O Acute or subacute syndrome characterized by disturbance of consciousness, global cognitive impairment, disorientation, attention deficits, disordered sleep-wake cycle, and fluctuation in presentation.

O Neurologists and Internists prefer the term “encephalopathy”, which literally means “disease of the brain.”

TypesO Mixed form (46%)O Hyperactive (30%)O Hypoactive (24%) – difficult type to

identify.

O Arousal – hyper – or hypovigilance.O Sleep-wake cycle.O Attention.O Orientation.

DELIRIUM O AgeO Baseline cognitionO Use of IV lines, restraints, and

bladder catheter. O Is CNS involved itself?

O Drugs – Always ask!!!O MetabolicO EndocrineO Withdrawal stateO InfectionsO Nutritional

O A 36-year-old real estate agent was in the first trimester of her first pregnancy when she awoke with diplopia. She had not been well for several days, feeling lethargic, off-balance and slightly disoriented; symptoms that she attributed to severe morning sickness during the previous eight weeks. She was not taking any medications and had been previously healthy. Exam revealed bilateral ptosis, limitation of gaze in all directions, slow upward saccades, upbeat nystagmus and mild ataxia.

O Wernicke’s encephalopathy – triad of ophthalmoplegia, ataxia, and confusion.

O Triad – minority of cases. O Ocular findings – earliest and most

constant. O About 30% have isolated or

predominant mental status changes ranging from confusion to frank coma.

O Sometimes – sudden onset.

O Persistent vomiting of any cause.O Chronic alcoholism. O Malignancy.O Prolonged IV alimentation. O Bariatric surgery. O Chronic renal dialysis. O Leukemia.

O Low serum erythrocyte transketolase – days to obtain.

O Treat on suspicion. O MRI – specific (93%) but sensitivity is

low.

O A 21-year-old primigravida with gestation age of 33weeks whose first and second trimester gestation was uneventful with no history of hypertension and epilepsy before and during pregnancy. She developed sudden onset of headache, giddiness, vomiting, and convulsions. Her blood pressure was 142/94 mmHg. Next day, the patient was taken into C section for fetal distress. On 2nd day of post-caesarean section she developed loss of vision, headache, and vomiting. Her blood pressure was 140/114 mmHg.

PRESO Posterior Reversible Encephalopathy

Syndrome.O Variety of symptoms – headache,

altered mental status, visual disturbances, and seizures.

O Hypertension, Pre-eclampsia/eclampsia, immunosuppression, sepsis, chemotherapy, collagen vascular disease, and renal failure.

Hepatic Encephalopathy

O Syndrome of neuropsychiatric dysfunction. O Mental status changes ranging from subtle

psychologic abnormalities to profound coma. O Clinical manifestations range from Stage I

(mild) to Stage IV (coma). O Asterixis – flapping tremor – stage II

(includes personality change and inappropriate behavior).

O Posturing can be seen in stage IV. O Focal signs and seizures – rare. O EEG, Ammonia, and Imaging.

O Precipitating factors.O Lactulose – enema v/s oralO Oral antibiotics O Protein restrictionO Sometimes there is cerebral edema

in hepatic encephalopathy.

MYOCLONUSO Fastest and briefest. O Sudden muscle contractions –

positive myoclonus. O Muscle tone lapses – negative

myoclonus. O Almost always around a joint. O Physiologic – hypnic jerks and

hiccups.

O Distribution?O Rest?O Action?O Provoked? Tactile? Or Auditory?O Rapid onset: Renal failure, DDS, and

Serotonin syndrome. O Dementia/Neurodegenerative

diseases.

O A 52-year-old woman presented with low-grade fever, headache, disorientation, amnesia, bad response to communication, numbness in the right hand, blurred vision in the right eye and tonic-clonic seizures in the previous two weeks. Her previous neurological history was unremarkable. Physical examination revealed horizontal nystagmus, bilateral Babinski signs, 4/5 of limb power, and poor cooperation in mental status examination.

Hashimoto’s Encephalopathy

O Steroid responsive acute or subacute encephalopathy associated with anti-thyroid antibodies.

O Presenting features vary widely. O Psychiatric symptoms around 60%.O TPO and Thyroglobulin. O TSH should be high but patients may

be euthyroid or hypothyroid.

O Myxedema coma – acute or subacute and precipitated by stress.

O Hypothermic, Hypo ventilate, and “suspended animation.”

Hyper- and Hypoglycemia

O HyperosmolalityO Diabetic ketoacidosis – pH doesn’t

correlate well with level of consciousness.

O Diabetic lactic acidosis. O Sudden lowering of serum osmolality

– cerebral edema – can be fatal. O Head trauma and Stroke patients –

Glucose control.

HypoglycemiaO Stroke like illness.O Delirium.O Coma. O Seizure.

HyperglycemiaO SeizuresO HemianopiaO Hemichorea/Hemiballismus

Hypoglycemic Brain Injury

O Range from reversible focal deficits and transient encephalopathy to irreversible coma.

O Mean blood glucose was around 30mg/dl.

O White matter – more sensitive to ischemia than previously thought.

O The duration of hypoglycemia may be difficult to determine in many cases.

O A 26-year-old woman presented to the emergency department with severe pleuritic chest pain and dyspnea. While waiting for a computed tomographic scan in the radiology department, she had an asystolic cardiac arrest. The resuscitation lasted 20 minutes, after which she was found to have reactive pupils. Three days later the family is considering withdrawing care because she is still comatose. On examination, her pupils are now unreactive and she has no motor response or brainstem reflexes. The nurse reports that the patient had myoclonus 12 hours ago.

O Brainstem reflexes – important to check.

O Sedatives/Paralytics?O Myoclonus?

O Pupillary reaction absent at Day 3 after cardiac arrest – poor outcome.

O Caution – motor response especially if hypothermia protocol was used.

O Corneal reflex. O Cold caloric testing.O Myoclonic status epilepticus – likely

poor outcome.

InvestigationsO Neuroimaging – MRI is preferred. O EEG – looking for reactivity. O SSEP

Chronic post-hypoxic myoclonus

O Lance-Adams syndrome. O Action myoclonus associated with

ataxia, postural imbalance, and very mild intellectual deficit.

O Asthma attack – typically.O Post-hypoxic or Post-hypercapnic.

O http://journals.lww.com/continuum/Pages/videogallery.aspx?videoId=81&autoPlay=true

UREMIC ENCEPHALOPATHY

O The level of BUN can vary widely. O Tremor, Asterixis, and Delirium. O May have hemiparesis. O Some patients free of cerebral symptoms

with values of BUN over 200 mg/dl. O Uremic patients – deficient in Thiamine. O Neurologic recovery does not immediately

follow effective dialysis.O Uremia and hypertensive encephalopathy

– difficult to diagnose.

DIALYSIS ENCEPHALOPATHY SYNDROME

O DementiaO Speech impairmentO MyoclonusO SeizuresO High aluminum content

O http://journals.lww.com/continuum/Pages/videogallery.aspx?videoId=72&autoPlay=true

DDSO HeadacheO NauseaO Muscle crampsO Delirium O SeizuresO 3-4 hours after dialysis may be 24 hours

laterO Self limited – within daysO First hemodialysis, severe uremia,

metabolic acidosis etc.

PULMONARY DISEASEO The degree of carbon di-oxide

retention correlates the most. O Duration of the condition. O Headache, confusion, and

somnolence. O PCo2 should be corrected gradually.

SEROTONIN SYNDROME

O Mental status changesO Autonomic hyperactivityO Neuromuscular abnormalities

(tremor, rigidity, myoclonus, hyper-reflexia, clonus, and babinski)

O Onset within 6-24 hoursO Hunter criteriaO We have to perform the work up!!!

NMSO Mental status changeO RigidityO FeverO DysautonomiaO Single dose or many yearsO Usually within first two weeks of

therapy

TAKE HOME MESSAGE

O ElectrolytesO InfectionO Metabolic disordersO ShockO Post-operative stateO Drugs – always!! Always!!O Withdrawal stateO Thiamine – high dose IV!!!!O Is the CNS involved itself??

O Always Imaging (CT v/s MRI) before LP. O EEG O Correction of underlying factors. O Remove Foley, IV lines etc. O Sleep wake cycle. O Anticholinergics!!!O GABAergic agents!!!O Opioids!!!O Hyper-active or Hypo-active delirium – Anti-

dopaminergic agents. O Others: Ondansetron, Rivastigmine, and

Dexmedetominidine.

O Questions???O Page @ 405-5033.