Session 3: Cognitive Problems

1. Session 3: Cognitive Problems

2. Definitions • Dementia: clinical state characterized by loss of function in multiple cognitive domains; diagnostic features include : memory impairment and at least one of the following: aphasia, apraxia, agnosia, disturbances in executive functioning. Cognitive impairments must be severe enough to cause impairment in social and occupational functioning and there must be a decline from from a previously higher level of functioning. • Acute confusional state: impairment of cognitive function that is not progressive, but is reversible. The impairment of consciousness varies, often being worse at night. It may be described as a transient organic brain syndrome characterized by concurrent disorders of attention, perception, thinking, memory, psychomotor behaviour and the sleep-waking cycle. • Delirium: acute cognitive and behavioral change with attentional problems (analogous to above) • Encephalopathy: diffuse brain dysfunction (includes acute confusional state and delirium) • Amnestic syndrome: Partial or total loss of memory, usually resulting from shock, psychological disturbance, brain injury, or illness. (cf Bourne Identity) • Mental retardation: a disability characterized by significant limitations both in intellectual functioning and in adaptive behavior as expressed in conceptual, social, and practical adaptive skills beginning before age 18. • Schizophrenia: any of several psychotic disorders characterized by distortions of reality and disturbances of thought and language and withdrawal from social contact

3. Schizophrenia Two (or more) of the following, each present for a significant portion of time during a 1-month period (or less if successfully treated): • delusions • hallucinations • disorganized speech (e.g., frequent derailment or incoherence) • grossly disorganized or catatonic behavior • negative symptoms, i.e., affective flattening, alogia, or avolition. Note: Only one symptom is required if delusions are bizarre or hallucinations consist of a voice keeping up a running commentary on the person's behavior or thoughts, or two or more voices conversing with each other.

4. Man found down • BP: 116/68; 104 HR; 99.5 F; 14 RR • Opens eyes to voice; grimaces to pain; unable to follow commands; blinks to threat bilaterally • Normal oculocephalics; symm reactive pupils; facial symmetry • Reduced tone with withdrawal of all extremities to pain

5. Laboratory Findings • Na 152, K 4.1, BUN 76, Cr 2.1; Glc 116 • AST/ALT: 23/47; INR 1.9 • Urine tox neg; • serum alc 0 • Head CT: bifrontal hygromas without mass effect; old parietal encephalomalacia; basal ganglia calcification • CXR: old granulomas • EEG: diffuse triphasic waves

6. What is needed to work up confusion? • Structural imaging: • Brain CT • Brain MRI • Infection/hemorrhage/tumor evaluation: • Spinal tap • Seizures/Brain death/psychogenic/other: • EEG Herpes Encephalitis Focal status epilepticus Other

7. Confusion in the Nursing Home • Dementia with superimposed conditions • Infection: UTI, pneumonia • Medication errors/overdose • End-stage medical diseases: CHF, renal • Poorly managed diabetes • Stroke • Encephalitis/meningitis • Seizure/post-ictal state • Other

9. 38 year old man • Talking crazy/staggering around • No recent ETHO though has a history of chronic liver disease, coagulopathy, hypertension, seizures, pancreatitis and head trauma • Medications: ? Phenytoin and nadolol • Exam: disheveled; 96 F; 179/100 BP; HR 112; disoriented to place, season and is confabulating with poor attention and recall; gaze-evoked nystagmus and incomplete right eye abduction on right gaze; absent reflexes and wide-based ataxic gait.

10. Issues • Cognitive syndrome: encephalopathy • Diagnosis • Treatment • Where is the pathology

11. Subtle bilateral abnormal hyperintense signal in the paraventricular region of the medial thalami seen on diffusion, flair and T2. Possible subtle abnormal signal of periaqueductal gray matter seen on flair and T2. 50 yo with mental status changes and abnormal eye movements.

12. Findings Subtle bilateral abnormal hyperintense signal in the paraventricular region of the medial thalami seen on diffusion, flair and T2. Possible subtle abnormal signal of periaqueductal gray matter seen on flair and T2. Further history revealed alcohol abuse. Diagnosis Wernicke's encephalopathy Discussion MRI of the brain with contrast: MRI demonstrates acute lesions of Wernicke-Korsakoff syndrome in medial thalamic and periaqueductal regions. This can be a useful diagnostic procedure in patients presenting with suggestive history and stupor or coma, where ataxia and ophthalmoplegia are not detectable. Alcohol abuse is the most common etiology. Prompt Thiamine administration is essential and actually was given to the patient prior the this MRI. Wernicke encephalopathy is a medical emergency. Prompt recognition of the symptom complex and a high index of suspicion are crucial to ensure early treatment. Early treatment can rapidly reverse the ophthalmoplegia and improve ataxia/dysequilibrium and early mental confusion, as well as prevent development of the amnestic state. In advanced cases, where severe prolonged deficiency has led to permanent structural damage, permanent deficits most often are manifested as the amnestic state and severe ataxia. Reference: emedicine. Contributor: Sanders

13. Acute Alcohol Intoxication

14. Alcohol Withdrawal Withdrawal seizures Delirium tremens Alcohol hallucinosis Headache/hangover

15. Chronic Alcohol Effects Cerebellar degeneration Vascular risks ICH SDH Thrombotic Embolic Seizures Cognitive Spinal cord: B12 def Neuropathy Muscular atrophy Heavy drinkers compared with light or non drinkers are: twice as likely to die of heart disease twice as likely to die of cancer twelve times as likely to die of cirrhosis of the liver three times more likely to die in a car accident six times more likely to commit suicide

16. 60 year old man • Making mistakes; forgetful; unable to complete his report; no longer interested • Irritable and defensive; lost his way home • Guarded/suspicious • Inattentive with digit span of 5; ¼ recall & confabulates 2 others • Occasional paraphasias • Difficulty with 3 step command; problem with 3 D cube drawing

17. Cognitive Syndrome • Differential diagnoses • Work-up • Blood: thyroid/B12/RBC folate +/- VDRL • Imaging?: CT/MRI • Management • Behavioral • Pharmacological • Acetylcholinesterase inhibitors • Glutamate modulators • Prognosis

18. This 80-year-old man presented with a gradual decline in functioning. Examination revealed a marked aphasia and poor visual-spatial ability with an MMSE score of 18/30. These T1-weighted axial MR images reveal diffuse cortical atrophy with prominent sulci and enlarged lateral ventricles.

26. Cognitive Syndrome in the Young • Differential diagnoses • Infection: HIV • Tumor • Drugs • Tests

27. Vignette • 75 year old with • Dementia • Hallucinations • Episodic alterations in consciousness • Bradykinesia • Differential diagnoses

28. Click here to view movie

29. Initial Symptoms Years Later AD DLBD PDD Dementia Parkinsonism Dementia Dementia Parkinsonism Dementia Parkinsonism

30. Vignette 56 year-old with 6 month history of rapidly progressive dementia, myoclonus and increased tone

31. SPORADIC CJD There are three investigations which might provide support for a diagnosis of sporadic CJD.  These are: The EEG The CSF 14-3-3 estimation The MR scan Transverse FLAIR MRI showing bilateral anterior basal ganglia high signal

32. This is an EEG tracing showing the characteristic periodic complexes.

33. 78 year old woman • Confusion; started “talking crazy” and was stubborn • Speaks with “meaningless words” and cannot answer yes/no questions accurately • Can mime but cannot follow commands, name or repeat • Unable to cooperate with most of exam

34. Questions • What has happened to this woman? • The nature of her deficit • What mechanism? • Is she aware of her deficits? • In what settings is anosognosia seen? • Would she be able to read aloud, write or comprehension related to reading? • Visual fields would show? • Discuss the Wernicke-Geschwind model of language and the anatomical localization

36. SP = Superior Parietal Lobule EC = Exner’s Writing Center A = Angular Gyrus B = Broca’s Area T= Pars Triangularis W = Wernicke’s Area H = Henschen’s Music Center

37. Definitions • Aphasia: loss of the ability to use or understand language due to a brain lesion • Mutism: the condition of being unable or unwilling to speak • Fluency:"Production and/or perception of verbal elements of communication that adhere to the sequence, rhythm, and timing patterns approriate for the communicative context and expectations of the speaker and/or listener" (Cross, 1998). • Paraphasia: A person with aphasia might use an incorrect word or unrecognizable word in place of the target word. This is a paraphasia. Paraphasias can be classified in 3 types. Phonemic or literal paraphasias are word errors that sound very close to the intended word (e.g., coke for coat). A verbal or semantic paraphasia occurs when a word that is related in meaning to the target word is substituted (e.g., plum for peach). The third type of paraphasia is a neologism - an invented word that is not recognizable as a word in the speaker's language. • Dysarthria: impaired articulation due to impairment in peripheral nerves or in speech musculature • Dysprosody: loss of or deficit in the comprehension or production of nonverbal aspects of language that convey attitudinal, emotional, and similar information to the listener. • Apraxia: loss of the ability to produce purposeful, skilled movements as the result of brain damage

38. Aphasias Fluency Comprehension Naming Repetition Global - - - - Broca - + - - Wernicke + - - - Conduction + - - - Transcortical-M - + +/- + Transcortical-S + - + + Anomic + + - +

39. 73 year old woman • Sudden onset headache, dizziness with vomiting; unsteadiness of gait and poor coordination of the right arm • What neurological conditions cause sudden, severe headache? • What is the localizing value of dizziness, gait instability, and difficulty controlling the RUE?

40. Time Passes • Patient is no long able to speak clearly; can open eyes and grunt Then in ER: • BP 185/105; afebrile; no nuchal rigidity • Extensor posturing with stimulation • No response to voice and no spontaneous limb movements • Pupils reactive • Eyes deviate to left with cold water in left ear without nystagmus; no response when done to the right ear • 2 calls • Test • Specialist

41. Questions • What other parts of the exam is needed • Eye movements? • Caloric testing results in • Normal awake patient • Comatose patient with intact brainstem • Brain-dead patient • Characterize and localize patient’s limb movements • What is the diagnosis • What phone calls were made • What is the prognosis

42. Definitions & Underlying Structures • Coma • Persistent vegetative state • Locked-in syndrome • Brain death

46. Arousal • ARAS

47. Differentiate causes of Coma • Diffuse processes • Findings • Causes • Structural • Supratentorial • Infratentorial

50. Coma Exam Findings Diencephalic Midbrain Pons Medulla Pupils: size/light response Calorics Corneals Motor response Respiration