chapter 4f neurology

8/2/2019 Chapter 4f Neurology

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4.6 NeurologyLawrence Diamond, MD

Introduction to Neurology

Neurological problems- 10% of MD visits in the United States and 15% of hospitalized patients

Aging of population increasing prevalence of many neurological diseases including Parkinsons, dementias,

chronic pain syndromes

Incidence of CVAs (strokes) actually decreasing due to better control of hypertension and appropriate usage

of anticoagulants

Basic Elements of Neurological Exam

Mental status exam

Cranial nerve exam

Motor function

Sensory exam

Cerebellar testing

Reflexes Gait testing

Mental Status

Level of consciousness- Impairment=Delirium

Cognitive function- Impairment=Dementia

Most common causes of impaired mental status- Toxic (including prescription medications), infections,

CVAs, trauma, Alzheimers disease

Motor Function

Dependent on state of muscles, nerves, joints, cognitive function

Strength- Rated on scale of 0 to 5 Tone- Hypertonic = spastic, as in upper motor neuron disease

Remors, tics, athetosis, asterixis, myoclonus, choreiform movements are all examples of abnormal motor

function

Sensory Exam

Dermatomal testing

Superficial pain

Deep pain

Position

Vibration

Two point discrimination

Temperature

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Cerebellar Testing

Coordination of voluntary movements

Finger-to-nose

Heel-to-knee

Adiadochokinesia- Inability to perform rapidly alternating movements

Reflexes

Deep tendon reflexes, i.e.- Knee jerk

Superficial skin reflexes, i.e.- Rooting, snouting

Brainstem reflexes, i.e.- Corneal, gag

Abnormal reflexes, i.e.- Babinski

Gait Testing

Circumduction gait- Hemiplegia

Scissoring or Spastic gait- Cerebral palsy

Ataxic gait- Cerebellar dysfunction, i.e.- Chronic alcohol abuse

Steppage gait- Foot drop

Festinating gait- Parkinsons disease Waddling gait- Muscular dystrophy

Myopathies

Myopathies are any disease in which the primary pathology is in the muscle tissue itself.

Symptoms and Signs

Fatigue- subjective

Weakness- objective, i.e.- Difficultly in performing tasks, droopy eyelids, change in facial expression, difficulty

chewing and swallowing

Hypotonia

Muscle atrophy Twitches

Spasms

Pseudohypertrophy- Muscle tissue replaced by fat- Seen in muscular dystrophy

Pain- Very rarely seen

Muscular Dystrophy

Most common myopathy

Several types- Most common- Duchennes

Sex-linked recessive

Begins at age two to six years

Pelvic girdle muscles affected first Rapidly progressive

All muscles eventually affected including heart, gut

Intellectual impairment at late stages

Usual lifespan- Teens

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Myasthenia Gravis

Disease of young adults

First symptom- Fatigue

Then- Ptosis, double vision

Increasing fatigue on exercise

Recovery on resting Episodic

No sensory disturbance

Autoimmune disease

Decreased number of acetylcholine receptors

Variable course

Treated with cholinesterase inhibitors, immunosuppression, thymectomy, plasmaphoresis

Polymyositis and Dermatomyositis

Onset in young adults

Initially - Joint pain, malaise, fatigue, fever

Then frank muscle weakness

Autoimmune, collagen vascular diseases

Dermatomyositis- Characteristic heliotrope discoloration of face, also other skin abnormalities common

Diagnoses made by muscle biopsy

Associated with various internal malignancies

Treated with immunosuppressive agents

Other Causes of Myopathy

Thyroid disease

Disorders of potassium, calcium, magnesium

Prescription drugs

Drugs of abuse

Major trauma

Radiculopathies

Pure motor, pure sensory, or mixed

Pain and/or sensory loss and/or motor dysfunction

Muscle atrophy

Fasciculation and decreased deep tendon reflexes

Multiple Causes

Muscle spasm- Most common

Degenerative disc disease

Disc herniation

Inflammatory processes

Space occupying lesions



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General Conditions

Cervical and lumbrosacral regions most commonly affected

Thoracic region- very rare

May or may not have obvious causative factor

Sudden or slowly progressive onset of symptoms and signs depending on etiology

Signs, Symptoms Related to Nerve Root Involved

Cervical spinal radiculopathy

Neck, shoulder or arm pain, weakness or numbness

Symptoms usually increased by extension of neck

Conservative treatment usually successful- immobilization of neck, heat, NSAIDs, muscle relaxants, local

anesthetics

Lumbar disc herniation

Can cause sciatica

Most commonly L4-L5 or L5-S1

Most commonly- Unilateral backache radiating down the affected side

Very often confused with acute muscle spasm of the lower back

May result in partial or total paralysis of the affected extremity depending on nerve root(s) involved

Usually treated conservatively with rest, NSAIDs, muscle relaxants, heat, local anesthetics

If above unsuccessful, traction sometimes attempted

Surgery usually reserved for intractable pain, bilateral symptoms, sphincteric disturbances, due to high

failure rate

Diagnoses usually made by CT scan or MRI

Parkinsons Disease

Neurological disease characterized by akinesia, bradykinesia, rigidity, resting tremor, and postural instability

Disease primarily of the elderly, though incidence in young adults increasing

Rigidity most common symptom with stiffness, increase of resting tone, masklike facie, Parkinsonian stare,infrequent blinking

Akinesia- inability to initiate and execute a movement- Both fine motor and gross motor

Micrographia

Absence of associated movements

Tremor- resting, fine, rhythmic

Pill rolling

Festinating gait- shuffling, accelerating, falling forward

Postural instability

Softening of speech

Autonomic disturbances- increased salivation, sweating, skin disturbances

Dementia and severe depression in late stages



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Causes

Idiopathic

Post encephalopathic

Drug induced- phenothiazines and drugs of abuse

Trauma, i.e.- boxers

Heavy metal poisoning Tumors

Etiology- imbalance between acetylcholine and dopamine in the caudate nucleus, putamen, and substancia

nigra- relative deficiency of dopamine

Treatment

Consists of L-dopa/Carbidopa, direct dopamine agonists, anticholinergics

Disease symptoms improve with treatment but disease progression inevitable

Cerebellar Syndromes

Cerebellar syndromes are characterized by imbalance, irregular, asymmetric, involuntary movements.Huntingtons chorea is the most common primary cerebellar syndrome

Autosomal dominant

Characterized by hyperkinesis

Symptoms develop slowly over several years

Usual age of onset- 30 to 50 years of age

Athetoid, snakelike movements

Affects all muscle groups including face, extremities

Associated mental disturbances- apathy, depression, paranoia, dementia

No effective treatment

Prognosis poor- most commonly fatal within 10-15 years of diagnosis



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Diseases of the Spinal Cord

Most common- multiple sclerosis

Multiple sclerosis

Caused by demyelination

Affects people in northern climates

Familial clustering Most commonly presents in young adults

Three types- relapsing, remitting (most common), primary progressive, secondary progressive

Relapsing, remitting type characterized by irregular bouts of mild, moderate, or severe weakness lasting

days or weeko

Optic neuritis common- Often is the initial presentation of the disease

In progressive types, severe disability can occur with visual impairments, intentional tremor, speech

disturbances, swallowing disturbances, spastic paraplegia, sensory abnormalities, seizures, autonomic

dysfunction including incontinence, dementia, depression, death

Etiology unclear- viral?, genetic?, autoimmune?, environmental?

Antiviral treatments show some benefit in the relapsing, remitting type only

Amyotropic lateral sclerosis Combines spinal muscle atrophy with pyramidal signs such as spasticity

Usually presents at age 40-60

Characterized by paralysis of muscles, muscle atrophy, fasciculation, spasms

No sensory loss

Sphincteric function preserved

Eventually, respiratory insufficiency due to paralysis of respiratory muscles

No effective treatment

Prognosis poor- 80% mortality within fiv years of diagnosis



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Diseases of the Cranial Nerves

Cranial Nerve I- Olfactory

Loss of olfaction related to cranial nerve I most commonly related to nasal disorders, trauma, influenza, in

that order

Cranial Nerve II- Optic

Loss of vision related to cranial nerve II caused by CVAs, trauma, optic neuritis, tumors, B12 deficiency,syphilis

Cranial Nerve III, IV, VI

Abnormalities affecting movement of the eyeball caused by trauma, tumors, inflammatory disorders,

arteritis, diabetes mellitus, multiple sclerosis

Cranial Nerve V- Trigeminal

Trigeminal neuralgia, affecting adults, causing severe unilateral pain in area of second or third division of

the nerve, provoked by touch, chewing or talking, possibly with a viral etiology, most commonly treated

with Tegretol

Cranial Nerve VII- Facial

Bells palsy, sudden onset of unilateral facial paralysis, probably caused by a herpes virus, complete or

incomplete, can affect tongue and other cranial nerves, usually results in complete recovery but many withresidua, treatment with steroids, antiviral may be of benefit

Cranial Nerve VIII- Auditory and vestibular

Various disorders of hearing and balance

Menieres disease- disease of young adults characterized by episodic bouts of often incapacitating vertigo,

tinnitis, as well as progressive hearing loss, no effective treatment

Cranial Nerve IX and X- Glossopharyngeal and Vagus

Loss of Cranial Nerve IX function, most common by CVA can cause loss of gag reflex, with risk of

aspiration, loss of taste, hoarseness. Cranial Nerve X rarely affected

Cranial Nerve XI- Accessory

Trauma can cause severing of nerve resulting in loss of use of ipsilateral sternocledomastoid and trapezius

muscles Cranial Nerve XII- Hypoglossal

CVA can cause upper motor neuron paralysis with deviation of the tongue to the paralyzed side

Seizure Disorders

General Characteristics

Occur in attacks

Motor, sensory, or autonomic involvement

Abnormal focus of excitation in brain

Abnormal EEG during attacks

EEG may be normal or abnormal between attacks

Increase regional blood flow in the brain

Multiple etiologies including idiopathic, febrile, infections, trauma, metabolic, drug-induced

Multiple types including grand mal, petite mal, partial motor

Grand Mal

Classic, generalized seizure

Often, but not always, preceded by aura

Tonic contractures, followed by clonic



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May last seconds to hours

Always amnesia for the event

Status epilepticus- unrelenting seizure despite treatment, may result in brain damage or death

Long term control often successful with phenobarbital, dilantin, valproic acid, neurontin

Petite Mal

Disease of young children

Disturbed level of consciousness lasting for several seconds

No motor involvement

Provoked by hyperventilation

May be many daily attacks

Second most common childhood epilepsy after grand mal

Often progresses to grand mal, but may disappear as child ages

Usually treated with valproic acid

Partial Motor

Focal motor or sensory phenomenon

Most commonly affects the hand, then the face Characterized by motor jerks or paresthesias

With or without loss of consciousness

Can progress to grand mal

Usually treated with Tegretol

Trauma

Trauma is characterized by concussion, which is a head injury involving alteration of consciousness, uncon-

sciousness less than 15 minutes, twilight state less than 24 hours, associated with headache, nausea, vomiting,

impaired mental status

Subdural hematoma

May be acute or chronic

Venous tears result in bleeding beneath dura with subsequent compression of brain tissue

CSF always bloody

Acute- after major trauma with rapidly developing neurological deficits

Chronic- symptoms develop over days, weeks, months

Diagnosis easily mad by CT scan or MRI

Treatment is evacuation of bleed

Prognosis very variable depending on degree of irreversible damage to brain tissue



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Epidural hematoma

Signs and symptoms not easily distinguishable from subdural hematoma, initially

Cause is rupture of middle meningeal artery

Symptoms much more rapidly progressive, therefore

Diagnosed by CT scan or MRI

Prognosis poor if not immediately evacuated

Post-traumatic sequelae may occur after any brain trauma, even if minor

Chronic headaches

Seizures

Cognitive deficits

Personality changes

Psychiatric disorders

Stroke

Also known as cerebrovascular accident (CVA)

Latest terminology- Brain attack

Cause by interruption of blood flow to part of the brain (ischemia) causing cellular death

Types

Thrombotic

Embolic

Hemorrhagic

Others

Thrombotic

Risk factors- Age, hypertension, diabetes mellitus, hyperlipidemia, smoking, polycythemia, obesity, family

history

Variable symptoms and signs- can take many forms depending on location of ischemic event

Prognosis varies from quick, complete resolution to sudden death

Transient ischemic attack- All symptoms resolve within 24 hours

Treatment consists of blood pressure control, risk factor reduction, physical, occupational, and speech

therapies

Embolic

Caused by embolus from distal source

Most commonly- from atrial fibrillation causing sludging of blood in the atria particularly in the presence of

mitral stenosis

Anticoagulation indicated in most people with atrial fibrillation, mostly with warfarin, aspirin and other

antiplatelet drugs not as effective in stroke reduction as warfarin

Prognosis tends to be somewhat worse than in thrombotic strokes

Hemorrhagic

Frank bleeding, usually caused by A-V malformation or aneurysm

Most common cause of stroke in young adults

Preventive surgery often indicated

Worst prognosis of the three most common causes of stroke



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Other Causes of Stroke

Hypertensive

Sickle cell anemia

Anoxia

Temporal arteritis

Vasculitis

Meningitis

Most common neurological infection

Inflammation of the meninges caused by infectious agent

Signs and Symptoms

Often precedent upper respiratory infection

Fever

Headache

Nausea and vomiting

Neck pain and stiffness Backache

Confusion

Drowsiness

Coma

Progression of above over hours or days

Causative Organisms

Bacterial-most common

Viral

Mycotuberculin*

Protozoal*

Fungal*

Rickettsial*

* Most common in individual with AIDS or other immunosuppressed individuals.

Treatment

Specific treatment based on causative organism based on analysis of cerebrospinal fluid

Cerebrospinal fluid findings

Causative organism- Gold standard for the diagnosis

White blood cells

Increased protein

Increased CSF pressure

Decreased glucose (in bacterial meningitis)

Prognosis

Variable with anything from quick resolution to rapid death. Worst in immunosuppressed individuals, young

infants, and in the elderly.



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Tumors

Primary vs. metastatic

Most commonly- Metastatic from other primary neoplasms, i.e. lung, breast, colon

Primary brain tumors

Glioblastoma multiforme - most common primary tumor

Astrocytoma Meningioma

Pituitary adenoma

Angioma

Sarcoma

Signs and Symptoms

Personality changes - most common initial presentation

Other changes of mental status

Headache

Seizures- brain tumor must be ruled out in all adults with new onset of seizures

Focal neurologic signs- usually late in presentationGlioblastoma Multiforme

Most common primary brain tumor

Of common types, the only one which is malignant

Highly malignant and aggressive

Usually presents between 40 and 60 years of age

Anatomically- butterfly lesion in corpus collosum

Surgery usually not possible

Radiation can slow progression of disease

Prognosis grave- usually fatal within two years of diagnosis

Other primary brain tumors can usually be treated with surgical resection. Post-operative therapy usually suc-

cessful in restoring full functioning. Brain tumors easily diagnosed with CT scan or MRI.



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Dementia

Characterized by global loss of intellectual function

Clear consciousness

Age, by far, the major risk factor, though less than 50% over age 85 have frank dementia

Symptoms

Memory loss- Most common presenting symptom

Recent memory loss first

Loss of executive function, i.e.- balancing checkbook

Confabulation

Loss of social skills

Loss of inhibitions

Hypersexuality

Progressing to complete disorientation

May eventually become incontinent, unable to perform self-care skills, even unable to eat

Causes

Irreversible

Alzheimers disease- most common form of dementia

Multiinfarct dementia- second most common cause, characterized by focal neurological symptoms in

addition to loss of intellectual function

Parkinsons disease

Multiple sclerosis

Anoxic dementia

Head injuries

Residua of infectious disease such as meningitis

Tumors

Autoimmune disorders

Alcoholic dementia

Others, including AIDS-related dementias

Reversible

Psychiatric disorders- most commonly depression

Drug toxicities or side effects

Nutritional disorders- most commonly B12 deficiency

Hyper- and hypothyroidism

Metabolic disorders, i.e.- hypercalcemia, dehydration

Infectious disease, i.e.-tertiary syphilis

Fecal impaction



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Work-up

Complete history with focus on specific symptoms, functional losses, past illnesses, medications, history of

substance use, family history, occupational history

Complete physical exam with focus on the general physical examination, neurological examination, cognitive

function including mental status assessment and depression screening

Diagnostic studies to include complete blood count, electrolyte and metabolic panels, thyroid function tests,serum B12 level, serological testing for syphilis

HIV testing in high-risk individuals

CT scan or MRI in individuals with rapid progression of symptoms, focal neurological findings, or a history

of head trauma

Treatment

Directed at reversible causes

Supportive therapies

Medications available for Alzheimers slow progression but unlikely to reverse cognitive deficits

Principles of Psychiatric Disease

Anxiety Disorders

Adjustment disorders

Panic attacks- periodic episodes of severe anxiety-palpitations, tremors, shortness of breath, gastrointestinal

complaints

Generalized anxiety disorder- constant level of anxiety interfering with day-to-day living

Above can be treated with benzodiazepines, more recently with SSRIs

Mood Disorders

Most commonly depression

Vastly diagnosed in medical community

Lowered mood varying from mild sadness to intense feelings of guilt, worthlessness, hopelessness Difficulty in thinking, concentration

Loss of interest in work or recreation

Somatic complaints- often multiple

Over or under eating

Sleep disorders

Most extreme cases- suicidal ideation or suicide

Usually unipolar, may also be bipolar with periods of manic behavior

Most commonly treated with SSRIs, tricyclics, lithium, though other treatments may also be effective



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Personality Disorders

Long history of recurrent maladaptive behavior dating back to childhood

Low self-esteem

Lack of confidence

Minimal introspective ability

Major difficulties with interpersonal relationships Various types- paranoid, obsessive-compulsive, narcissistic, passive-aggressive, antisocial, borderline

Treatment very difficult due to lack of introspective ability

Psychotic disorders

Slowly progressive social withdrawal

Loss of ego boundaries

Loose thought associations with derailment

Delusions- Usually grandiose or paranoid

Hallucinations- rare, though usually of an auditory nature

Flat affect

Concrete thinking

Multiple subtypes

Treatment difficult, symptom improvement though with various antipsychotic agents, newer agents with far

fewer side effects


chapter 4f neurology

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