Behaviourial manifestation in neurologic disease

Topic : Neurological Disorder With Behavioral Manifestation

Dr Saurav Deka is an MBBS ,MD clinical pharmacologist having good clinical experience in neurologic disease while he was working in clinical field . He has been working as medical advisor in some reputed pharmaceutical company of India from 2008.The topic discussed is a complex one which is edited by Dr Deka .

He has complied lot of information scattered in literature about this topic. You can contact him

for any other topic : [email protected]

Neurological Disorder With Behavioral

Manifestation

Contents

• Introduction

• Clinical manifestations

• Pathogenesis and pathophysiology

• Prognosis

• Management

Introduction

• All human behavior is generated by the brain

• Unlike some neurologic signs like hemiparesis,aphasia most behavioral signs do not localize to specific brain sites

• Behavioral disturbances occur to majority of patients with neurologic disease

Introduction

• Yet those behavioral signs and symptoms may go unnoticed

• Most of time these these manifestion of neurologic disease is ascribed to pre existing phychiatric diorder

• Misunderstood as “Phychogenic” in origin

IntroductionBehavioral disturbances due to primary neurologic conditions

can be distinguished from idiopathic psychiatric disease by applying principles of brain-behavior relationships.

NEUROLOGIC DISEASE & CLINICAL MANIFESTATIONS

Neurologic Disease • Alzheimer disease

• Parkinson disease

• Stroke

• Epilepsy

• Brain Tumour

• Trauma brain

• Huntington disease

• Multiple sclerosis• Wilson Disease

Clinical Manifestations

• Disturbances in emotional expression

• Disturbances in motor function

• Perceptual disturbances

• Delusions and abnormal thought content

• Cognitive dysfunction

• Personality and personality change

Disturbances In Emotional Expression

• Disturbances in emotional expression are 2 to 3 times more common in patients with neurologic, especially among the elderly .(Coffey and Coffey 2011)

• Distinguishing these states from idiopathic psychiatric illness is difficult when clinicians use descriptors like “depression” and “anxiety

• Emotional expressions of pathologic states such as melancholia, apathy, and mania are subjectively and objectively distinct.

Melancholia Melancholia is a pathologic emotional state strongly

associated with mortality from suicide and with positive responses to specific treatments (Taylor and Fink 2006),

Making it the most important emotional disturbance for clinicians to distinguish from demoralization, depression associated with personality deviation, and apathy from frontal circuitry disease.

Unlike these other conditions, melancholia is often accompanied by psychosis and catatonia.

Patients may also appear reversible “pseudodementia.

Mania is rarely seen in neurologic practice, but chronic hypomania is important to distinguish from frontal disinhibition syndromes.

Features of Melancholia and Syndromes Mistaken for Clinical Depression

Melancholia

Apathy Demoralization

Pathologic mood of unremitting gloom or apprehension

Psychomotor retardation or agitation

Vegetative signs reflecting an exaggerated stress response (eg, anorexia and weight loss, slowed bowel motility, insomnia, loss of libido, altered circadian rhythms)

Cognitive impairment in concentration and working memory

Diminished goal-directed overt behavior (eg, lack of productivity, effort, initiative)

Diminished goal-directed cognition (eg, lack of interests in hobbies or new experiences, lack of concern for one's health)

Diminished emotional concomitants of goal-directed behavior (eg, unchanging affect, absence of excitement or emotional intensity)

Not attributable to intellectual impairment, emotional distress, or diminished arousal

Nonpathologic, sad, or dysphoric mood in response to illness

Emotional responsivity to positive events

Feelings of understandable helplessness and hopelessness

Feelings of subjective

incompetence (eg, “I can't do it”)

Pessimistic thinking with demanding or indifferent behavior

Hypomania Mistaken for Bipolar DisorderHypomania • Infectious, exaggerated happiness or modest, easy irritability when frustrated

• Emotional lability, transient with depressive features• Hyperactivity and pressured speech; increased productivity unless mania emerges• Decreased sleep need• Increased appetitive behaviors (eg, food, sex, drugs)• Psychosis uncommon; some psychosensory features may occur

Disinhibition • Behavioral impulsivity and coarsening of personality; loss of social graces

• Distractibility and modest hyperactivity, but with reduced productivity• Witzelsucht, but no sustained expansive mood state• No psychosis• No change in sleep need

Delirium (hyperactive type) • Acute onset, fluctuating course

• Altered level of arousal and alertness (EEG abnormal)• Impulsivity, distractibility, fearfulness, irritability• Multisensory hallucinations and delusions• General medical condition (eg, infection) or intoxication (eg, medication overdose)• Signs of general medical illness (eg, fever)

Pathologic laughing and crying (PLC) • Paroxysmal and brief

• Emotional expression disconnected from emotional experience (emotions are excessive or socially inappropriate)• Denial of feeling the emotion expressed

Emotional Expression in Neurologic Disorders Disease Apathy Melancholia Demoralisation laughing & crying

Alzheimer disease •45-90%•Early onset

•15-50% ,•Early onset,•Congnitive complain

Less common •30-40%•Mixed laugh & cry

Parkinson disease •30-50%•Precede onset of motor symptoms

•30-50%•Precede onset of motor symptoms

Prevalence is as high as 36% in multiple systems atrophy.

Stroke Anergia Bradykinesisa

3-6 month after stroke Catastrophic Immediate

10-25%

Epilepsy Most common

Brain Tumour Tumor histology doesnot predict symptoms .frontal lobe tumor associated with frontal lobe syndrome temporal tumors produce emotional disturbance ,right sided lesion more associated with disinhibition and elevated mood .

Trauma brain 40-50%Early onset

rare Not known 5-11%

Huntington disease Associate with degeneration of caudate nucleus

rare Stress of fatal disease

Multiple sclerosis 50%Lesion in left frontal region ,cortical atrophy

Non Melancholic depression 10-20%

Wilson disease Disinhibition (50% to 70%): Irritability, aggression, and “incongruous behavior”. The 10-fold increase in prevalence of bipolar disorder among persons with Wilson disease likely reflects the prevalence of disinhibition rather than primary manic-depression.








Disturbances in motor function

• Changes in motor functioning can be simple Simple : eg like hemiparesis

Complex : eg parkinsonism

• Changes can be subtle and nonspecific

eg : restlessness suggesting anxiety or

• Changes can be dramatic and diagnostic

eg postures or stereotypy suggesting catatonia.

Catatonia• Catatonia can be missed, first, from the mistaken idea that

patients with catatonia must be frozen in an odd posture.

• Many conditions elicit Catatonia Postictal states

Nonconvulsive status

Complex partial seizures

Basal ganglia disease

Stroke (ischemic and hemorrhagic)

Encephalitis

Postencephalitic states

Temporal lobe infarction

Thalamic lesions

Demyelinating disease

Tuberous sclerosis

Narcolepsy








Perceptual disturbancesPerceptual disturbances occur in all sensory modalities Hyperesthesia and hypoesthesia Distortions of stimulus intensity (eg, a dim light appears as glaring)

Synesthesia Stimulation of one sensory modality eliciting a perception in a different sensory modality (eg, “seeing a sound”)

Dysmegalopsia Distortions of the size and shape of objectsColor spectrum distortions Changes in color perception

Illusions False perceptions or misinterpretations of environmental stimuli

Hypnagogic and hypnopompic hallucinations Visual or auditory perceptions, not vivid but distinct from dreams, occurring while falling asleep or on waking

Extracampine hallucinations False perception outside the limits of the normal sensory field (eg, hearing plotters in another country)

Peduncular hallucinations Visual perceptions of cartoon-like people or animals that are non-threatening

Autoscopic hallucinations Perceptions of one's own image, often sensed as vague or slightly to one side

Lilliputian (or Brobdingnagian) hallucinations Visual perceptions of small (or gigantic) objects or creatures

Tactile hallucinations Perceptions experienced as emanating from inside the body or from the skin

Olfactory (or gustatory) hallucinations Sudden, intense perceptions of smells (or tastes) that are often unpleasant

Phoneme (voices) hallucinations Hallucinated voices, varying from vague or muffled whispers to sustained or clear voices, perceived as originating from a source external to oneself

Musical hallucinations Perceptions of vivid, often familiar tunes or lyrics








Delusions and abnormal thoughtPhenomena Description Differential Diagnosis

Delusions of persecution • Isolated and simple

“The nurse is trying to kill me”

Neurodegenerative conditions

• Isolated and elaborate Eg, a man believed his wife and the government had teamed up in a complicated plot to “render him impotent”

Encephalopathy, temporal lobe dysfunction

• Non-isolated The patient demonstrates other psychopathology

Mood disorders, schizophrenia

Delusions of misidentification • Capgras syndrome

A relative or familiar person is believed to be replaced by a similar-looking impostor

More often right-hemisphere lesions from a stroke or seizure focus, traumatic brain injury, neurodegenerative conditions

• Fregoli syndrome Unfamiliar persons are thought to be well known to the patient and often said to be celebrities

Phenomena Description Differential Diagnosis

Doppelganger The belief that one has a double who carries out independent actions

Reduplicative paramnesia A familiar person, place, or object is believed to be duplicated

Delusions of passivity Experience of being controlled by an outside force or having another person's thoughts

Schizophrenia, manic-depression, right-hemisphere lesions

Delusions of poverty Beliefs of becoming bankrupt or losing one's home and belongings

Melancholia

Nihilistic delusions(Cotard syndrome)

Beliefs of being dead or that one's body or body parts are deteriorating

Manic-depression (90%), brain tumor, migraine

Delusions of grandiosity Inflated self-importance and beliefs of superior accomplishments, bodily perfections, and attention from others

Manic-depression, conditions involving frontal lobe circuitry dysfunction

Erotomania Delusional belief that one's love for another person (sometimes a celebrity) is reciprocated

Neurologic disease (25%; degenerative conditions, HIV.,seizure disorder, hemorrhage, traumatic brain injury), schizophrenia (35%), mood disorders (22%)

Delusional memories(paramnesia)

False memories derived from illusions in association with intense emotion (eg, the depressed person “remembers” past sins)

Mood disorders, schizophrenia








Cognitive dysfunction

Most behavioral disorders have associated cognitive impairments.

An increasingly common clinical challenge is faced when a person over 65 years of age presents with a significant decline in behavioral functioning

. Between ages 65 and 75 years, melancholia is several-fold more likely. So it is a this diagnostic dilemma

Depressive PseudodementiaVs From Alzheimer Disease

Clinical Feature Depressive Pseudodementia

Alzheimer dementia

Mood Melancholic

Apathetic, avolitional

Cognition Bradyphrenia, executive dysfunction, recall deficits, false negative errors, some benefit from serial presentation, less behavioral consistency

Visuospatial deficits, executive dysfunction, recall deficits, false negative errors, no benefit from serial presentation, more behavioral consistency

Insight Exaggerates problems

Minimizes problems

Course Episodic; more discrete onset Progressive; insidious onset with mild cognitive impairment

Personal history Mood disorder Late-life non-melancholic depression

Family history Mood disorder Dementia

Functional imaging Frontal or diffuse hypometabolism Biparietal and temporal hypometabolism

Structural imaging Normal or with mild atrophy without progression

Temporal atrophy and ventricular enlargement








Personality Changes Seen in Neurologic DiseaseDescriptor Behavioral Traits

Differential Diagnosis

“Epileptic” Adhesive and viscous, stubborn and perseverative, humorless sobriety, pedantic and circumstantial speech, hypergraphic, pseudo-profundity, hyposexual

Epilepsy (up to 60%), chronic manic-depression

“Paranoid” Moody and irritable, suspicious and defensive, quarrelsome and litigious, “neighborhood crank”

Chronic limbic system disease, alcoholism

“Emotional” Deep emotions, intense expressions, easily tearful, Witzelsucht, hyperreligious

Epilepsy (less common)

“Frontal lobe” Lateral orbital prefrontal: irritability and emotional lability, episodic dyscontrol and unplanned violence, suspiciousness, restlessness and impulsivity, self-destructive and lacking insight, childishly self-centered and insensitive, overly talkative Dorsolateral prefrontal: lack of spontaneity and initiative, loss of drive or ambition, loss of interests, sluggish, socially isolative, dysphoric

Stroke (particularly large or multiple lesions in anterior areas), tumor (left frontal areas associated with apathy, right frontal areas with disinhibition), traumatic brain injury (40% lateral orbital prefrontal, 10% to 30% dorsolateral prefrontal, 30% to 50% mixed), neurodegenerative conditions

“Rigid” Inflexible, stoic, frugal, slow-tempered, orderly, muted emotional expression, vulnerability to depression

Parkinson disease

PATHOGENESIS AND

PATHOPHYSIOLOGY

Functional brain Systems and Their Signature Psychopathology

Functional brain system Psychopathologic features

Frontal lobe circuits Catatonia, basal ganglia signs, apathy and disinhibition (“frontal lobe syndromes”)

Cerebellar-pons Apathy, mutism, disinhibition

Dominant cerebral cortex • Frontal • Temporal • Parietal

Apathy, disinhibition Psychosensory phenomena Gerstmann syndrome (dysgraphia, dyscalculia, finger agnosia, and left-right disorientation)

Non-dominant cerebral cortex • Frontal • Temporal parietal

Motor aprosodia Misidentification delusions; passivity delusions; receptive aprosodia; psychosensory phenomena

Stress response system Melancholia, anxiety disorders

Hedonistic reward system Substance abuse

Arousal system Stupor, delirium, sleep disorders

Prognosis

Behavioral disturbances are substantial factors determining quality of life for persons with neurologic disease.

Compared to the general population, suicide risk is increased among patients with

I. Huntington disease (12-fold),

II. epilepsy (11-fold),

III. traumatic brain injury (8-fold),

IV.multiple sclerosis (7-fold),

V. stroke (2-fold),

VI.migraine with aura (3-fold)

Ref : Arciniegas DB, Anderson CA. Suicide in neurologic illness. Curr Treat Options Neurol 2002;4:457-68

Management

Treating the underlying condition When behavioral disturbances persist,

treatments are similar to those for patients with primary psychiatric disorders

Refer to Coffey CE, McAllister TW, Silver JM. Guide to Neuropsychiatric Therapeutics. Philadelphia, PA: Lippincott Williams & Wilkins, 2007

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Behaviourial manifestation in neurologic disease