4.

Neurology

Lawrence 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 with residua, 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, unconsciousness 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 presentation

Glioblastoma 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 successful 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

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