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Neurological Problems

Localized/Limited Nerve Disorders

  • Bell’s palsy
  • Trigeminal neuralgia
  • Herpes zoster (shingles)
  • Temporal arteritis
  • Central Disorders
  • Headache
  • Brain tumor
  • Meningitis
  • Dizziness
  • TIA/CVA
  • Epilepsy/Seizure disorder
  • Transient Global Amnesia
  • Gait/Movement Disorders
  • Gait disorders
  • Normal pressure hydrocephalus
  • Benign Essential (Familial) Tremors
  • Parkinson’s disease
  • Multiple sclerosis
  • Peripheral Disorders
  • Neuropathic pain
  • Peripheral neuropathy
  • Complex Regional Pain Syndrome
  • Carpal tunnel syndrome
  • Guillain-Barre syndrome
  • Restless leg syndrome
  • Muscle Weakness

Neurological Problems

  1. Localized/Limited Nerve Disorders

1.Bell’s Palsy:

Description

Paralysis of CN VII

Most common cause of facial paralysis

Increased incidence in ages 30-40 and >70

  • Etiology: although usually idiopathic, HSV type 1 may play a role

Risk factors; DM, hypothyroidisms, AIDS, Lyme disease, syphilis, sarcoidosis, viral infections, pregnancy (3rd trimester when increased vascular volume can risk facial edema and nerve compression)

Functions of CN VII

  • Controls the muscles of the neck, the forehead and facial expressions
  • Controls eyelids to close shut and the mouth for smiling
  • Controls perceived sound volume
  • Taste sensation anterior 2/3 of tongue
  • Controls secretions in the tear glands and salivary glands

Clinical Presentation

Abrupt onset over hours, with maximal facial weakness at 24-72 hours

Symptoms usually unilateral

Symptoms vary depending on level of lesion that is preventing transmission of nerve impulses down the facial nerve

Etiology of lesion is unclear, but causes anoxia and swelling of the facial nerve

Involves innervation of forehead, eye, mouth

History

Onset, evolution, presentation of symptoms

Effect of symptoms on day-to-day functioning

Control of DM, HTN, hyperlipidemia

Recent herpes zoster infection

May be ear involvement

Ramsey Hunt syndrome (vesicles on TM)

Subjective

Inability to close the eye; dry eye or tearing

  • Inability to wrinkle forehead/grimace

Asymmetrical smile

  • Normal facial sensation

Sound hypersensitivity (hyperacusis) on affected side, ear pain

Loss of taste (anterior 2/3 of the tongue)

  • Headache

Source:

Physical Exam for Bell’s Palsy: What will you include?

Objective

  • At rest: the loss of facial muscle tone causes downward droopiness of the brow, eyelid, nostril, lip, and cheek on the affected (paralyzed) side of the face
  • Functional:
  • loss of the dynamic muscles of the lip and cheek result in abnormal lip and oral function affecting chewing, retaining fluid while drinking, speech patterns and communication skills.
  • Weakness of the circular muscles around the eye causes incomplete eye closure (lagopthalmos), excessive corneal exposure and tearing, and the inability to smile
  • Bell’s phenomenon: upward diversion of the eye on attempted closure of the lid (seen when eye closure is incomplete)

Facial nerve palsy can be graded using the House-Brackmann Scale, which ranges between Grade I (normal function) and VI (no movement)

Grade I– Normal
Normal symmetrical function

Grade II – Mild dysfunction
Slight weakness noticeable only on close inspection
Complete eye closure with minimal effort
Slight asymmetry of smile with maximal effort
Synkinesis (unwanted facial movements) barely noticeable, contracture, or spasm absent

Grade III – Moderate dysfunction
Obvious weakness, but not disfiguring
May not be able to lift eyebrow
Complete eye closure and strong but asymmetrical mouth movement
Obvious, but not disfiguring synkinesis, mass movement or spasm

Grade IV – Moderately severe dysfunction
Obvious disfiguring weakness
Inability to lift brow
Incomplete eye closure and asymmetry of mouth with maximal effort
Severe synkinesis, mass movement, spasm

Grade V – Severe dysfunction
Motion barely perceptible
Incomplete eye closure, slight movement corner mouth
Synkinesis, contracture, and spasm usually absent

Grade VI – Total paralysis
No movement, loss of tone, no synkinesis, contracture, or spasm

Reference: House JW, Brackmann DE. Facial nerve grading system. Otolaryngol. Head Neck Surg 1985; 93: 146–147.

Differential Diagnosis:

Infectious etiologies (OM, herpes zoster, Ramsay Hunt syndrome, Lyme disease, chronic meningitis, bacterial meningitis, osteomyelitis)

Neoplastic lesions (acoustic neuromas, parotid tumors)

Other neuro causes (CVA, Guillain-Barre syndrome, MS, sarcoidosis)

Trauma

Clinical Pearls

(Source: Piercy, J. (June 11, 2005). 10-minute consultation: Bell’s palsy. BMJ, 330: 1374.)

Is it Bell’s palsy or a CVA?

oA lower motor neuron lesion occurs with Bell’s palsy, causing weakness of all the muscles of facial expression (angle of mouth falls, weakness of frontalis occurs, eye closure is weak)

oAn upper motor neuron is associated with a cerebrovascular accident; frontalis is spared, normal furrowing of the brow is preserved, and eye closure and blinking are not affected

Check that no other cranial nerves are involved.

oBell’s palsy affects only CN VII

oCheck for a painful rash over the ear, which indicates Ramsay Hunt syndrome caused by herpes zoster virus

Diagnostic Studies for Bell’s Palsy: Diagnosis of Exclusion

(Usually no testing is necessary unless diagnosis is in question)

CBC with diff; ESR; FBS (Hgb A1c if known to have diabetes)

Lyme titer

Electromyography (EMG)—not routinely done (not very reliable until after 2 weeks, when it may detect denervation and demonstrate nerve regeneration)

Clinical Management

Most will recover either totally or to an acceptable degree cosmetically and functionally

Increasing age is a risk factor for a less-than-complete recovery.

Pharmacologic Measures

To prevent corneal injury: methylcellulose drops (hourly lubrication drops during day)

  • Antivirals
  • Acyclovir 400 mg by mouth 5 times/day X 10 days

Corticosteroids

o60-80 mg/dayfor 5 days, then taper and d/c by 14th day

oUse cautiously in patients with diabetes

oBest results if started within 3-4 days of symptom onset

Nonpharmacologic Measures

Patch or tape down affected eye at night; eye ointment

Controversial:

ofacial nerve decompression

oFacial nerve graft

oNerve anastomosis

Electromyography stimulation (to prevent muscle atrophy) – Need PT referral

Prognosis

Improvement usually begins within 1-2 weeks; total recovery of function usually within 2-4 months

The younger the individual, the most complete the remission

  • Risk factors associated with poor outcome
  • Age > 60 years
  • Complete paralysis
  • Decreased taste or salivary flow on the side of paralysis

90% have complete remission

10% have some permanent paralysis

2.Trigeminal (CN V) Neuralgia (also known as Tic Douloureux)

Etiology: thought to be related to compression of trigeminal nerve, perhaps from vascular loop. Other causes:

benign tumors (meningioma, acoustic neuroma)

plaque formation in multiple sclerosis

Symptoms

Intense, stabbing pain within distribution of trigeminal nerve

oRange: 1-2 attacks/day to 10-20/hour

oMore pronounced during day

Source:

Trigger points:

otouching of the face

owind blowing across the face

ohot or cold liquids

May begin spontaneously or with facial movements such as speaking, chewing

Usually unilateral

Involves 2nd(maxillary) or 3rd(mandibular) division of trigeminal; rarely ophthalmic division

Majority > age 50

Women > men

Diagnosis

oGuidelines from the American Academy of Neurology recommend that all patients with trigeminal neuralgia have MRI or trigeminal reflex testing, since up to 15% of patients have an underlying structural cause such as a tumor. (Source:

Treatment

Pain relief: anticonvulsant meds

ocarbamazepine (Tegretol) – 80% effective

initial dose: 200 mg/day
increase slowly (100-200 mg every 2-3 days)
Check CBC and LFTs periodically
  • oxcarbazepine (Trileptal)

ophenytoin (Dilantin) – 50% effective

  • gabapentin (Neurontin) (if pain refractory to carbamazepine); better tolerated than carbamazepine by elderly patients
  • lamotrigine (Lamictal)

Surgical therapy

opercutaneous trigeminal neurolysis

omicrovascular decompression

3.Herpes Zoster (Alias: Shingles)

Epidemiology

Incidence rises with increasing age

About 20% of general population will experience zoster

Approximately 50% of people who live beyond age 80 can expect to have zoster

Nearly 70% occur in people > age 50

No difference in gender, race, or season

Etiology

Acute varicella infection, usually as child

Latent virus takes up residency in the sensory nerve ganglia

Periodically reverts to infectious state, but held at bay by immune functions (= contained reversion)

As immune function declines, host resistance drops,”contained” reversion eventually breaks through to cause herpes zoster

Clinical Presentation

Usually heralded by dermatomal pain, sometimes accompanied by malaise, fever

Pain precedes vesicular eruption by 2-3 days

oReports of 2 weeks to 100 days before!

Within few days: skin overlying the dermatome reddens and blisters

A few vesicles are usually grouped on an erythematous base (vs. scattered, single vesicles of chickenpox)

Several days later: vesicles become pustular and develop crusts.

Scabs form.

o*At this point, the lesions no longer contain virus

Thoracic dermatomes most often affected

May affect > 1 dermatome

Early Diagnosis Important

Prompt recognition of herpes zoster infection is important because antiviral therapy, when appropriate, is maximally effective only when started during the earliest stages of the eruption

Course of herpes zoster

Depends on patient’s age, immune status

New lesions continue to appear for 2-3 days

Lesions usually crust/scab within 14 days

Pain may linger for several more days

The older the patient, the longer the duration of zoster-associated pain

Elderly: greatest risk of postherpetic neuralgia

Diagnosis

Usually clinical diagnosis with no lab testing needed

Tricky: herpes simplex vs. herpes zoster

oHSV may mimic zoster, esp. when on thighs, buttocks, or face

oFrequent recurrences = hallmark of HSV

Consider this first when an immunocompetent patient reports repeated outbreaks of herpetic lesions

Lesions of sacrum: sacral zoster vs. anal HSV

If unsure: viral antigen assays

Can It Recur?

< 5% of immunocompetent patients who have 1 episode of herpes zoster will have another; if so, usually separated by years

This may change as we live longer

50% of recurrent zoster in immunocompetent adults develops in same dermatome as previous outbreaks

Subsequent episodes may be more painful, possibly because the patient is older

Ramsey Hunt syndrome

Facial paralysis that resembles Bell’s palsy

Zoster skin lesions are not prominent because the facial nerve has little cutaneous distribution

Small herpetic vesicles on external surface of ear, auditory canal, TM, hard palate

Ophthalmic involvement

15% have involvement in the ophthalmic distribution of the trigeminal nerve

Hutchinson’s sign: lesion on the tip of the nose

oTip-off to corneal involvement and associated keratitis

Can have eye involved without Hutchinson’s sign being present

Ophthalmologist referral

What Do You Think?

Should herpes zoster in an apparently healthy young or middle-aged adult always trigger a search for HIV infection or occult malignancy?

Treatment

The sooner antiviral therapy is started, the more likely the benefit

oCritical period: 48-72 hrs. after onset of rash

Antiviral therapy to reduce inflammation, relieve acute pain, prevent chronic pain, & prevent ophthalmic complications: 7 days

oAcyclovir (Zovirax): 800 mg q4h 5X/day

oFamciclovir (Famvir) 500-750 mg tid

oValacyclovir (Valtrex) 1000 mg tid

Some choose to co-administer low-dose corticosteroids to reduce inflammation and chance of postherpetic neuralgia

Steroids, however, carry a risk for potentiating the infection as well as producing adverse effects

Postherpetic neuralgia (PHN)

“The pain that lingers”

Seen almost exclusively in elderly patients

May persist weeks to months, even years

May result from damage to the sensory ganglia caused by virus

Treatment for PHN

Topicals: capsaicin cream (Zostrix), Burrow’s solution, calamine lotion, ethyl chloride spray, lidocaine/prilocaine cream

Systemic: tricyclic antidepressants (Elavil), anticonvulsants (Tegretol), antivirals (Zovirax)

Other: TENS, lidocaine injection, nerve block injections, nerve resectioning

Prevention of Herpes Zoster

oZostavax vaccination

oWho should receive vaccine

oMedicare coverage; charging for the vaccination

4.Temporal Arteritis (As known as Giant Cell Arteritis)

When an elderly person develops a H/A, it is important to include temporal arteritis in the list of differential diagnoses! (also consider it in older adult with high ESR and normal WBC even if HA is absent)

Rare in persons under 60 years old

A vasculitic disorder

  • occurs when the temporal arteries, which supply blood to the head and brain, become inflamed or damaged

Produces temporal H/A

Clinical Presentation

Headache (75-80% of patients)

  • Jaw claudication (pain with chewing) (50% of patients)

Local tenderness to palpation of the forehead and temporal arteries

Visual loss (15-30%)

  • Polymyalgia rheumatica (34-60% of patents)

Systemic c/o weight loss, anorexia, weakness, and low-grade fever (15% of patients) may precede H/A

Assessment/Diagnostic Studies

ESR elevated (often > 50-80)

Increased serum globulins

Diagnosis is confirmed by a temporal artery biopsy, whichshows distinctive inflammatory changes (biopsy can be done up to 2-4 weeks after steroid started)

Clinical Management

Immediate initiation of high-dose steroid treatment

orelieves H/A and systemic sx.

onormalizes ESR in about 4 weeks

Medical emergency: ischemia may cause blindness due to retinal infarct, MI, CVA

Many episode-free after 1-2 years of treatment

Current Research: Patients with giant-cell arteritis (GCA) had higher rates of heart attacks, strokes, and peripheral artery disease than people without GCA.

  • Unsure of causation
  • Recommendation: appropriate preventative measures, noting of early warning signs, and prompt treatment
  • (Source: Tomasson, G, Peloquin C., et al, Risk for cardiovascular disease early and late after a diagnosis of giant-cell arteritis: A cohort study. Annals of Internal Medicine, 2014.)

B.Central Disorders

  1. Headache

Headache is a subjective feeling of pain caused by a variety of intracranial and extracranial factors.

  • One of the most common complaints in adult and children, with most headaches being self-treated with an OTC analgesic.
  • The vast majority of headaches are acute, self-limited, and do not pose imminent danger or serious sequelae.

Goals for the practitioner in evaluating a headache are to:

1.Identify life-threatening causes of headache,

2.Diagnose treatable disease associated with some headaches, and

3.Provide symptom relief.

Primary headaches: characterized by the absence of structural pathology or systemic disease; they account for > 90% of all headaches.

  • Migraine
  • Tension-type headache
  • Cluster headache and other trigeminal autonomic cephalgias
  • Other primary headaches

Secondary headaches: those caused by an identifiable organic pathology, one that is confined to the meninges or to the cerebral parenchyma. These include headaches attributed to:

  • head/neck trauma, cranial or cervical vascular disorder, non-vascular intracranial disorder
  • substance or its withdrawal
  • infection
  • psychiatric disorder

Pain from headache arises from stimulation of pain-sensitive structures of the head and brain caused by traction, inflammation, vascular dilatation, muscle contraction, or dysregulation of ascending brainstem serotonergic systems.

Generally:

•Pain arising from disordered function, damage, or inflammation of structures located anterior to and above the tentorium is felt in the front of the head

•Pain felt in the back of the head arises from structures located below the tentorium

•Extracranial structures that are sensitive to pain include the skin, scalp, blood vessels, facial muscles, eyes, ears, teeth, nasal cavity, mucous membranes of the mouth and pharynx, and the TMJ.

•The substance of the brain itself is not sensitive to pain, but sensitive structures include the blood vessels, sensory nerves, and ganglia.

60% of new-onset headaches and 90% of chronic headaches are migraine or tension.

First, identify if this is a life-threatening headache!

What clues indicate this is a potentially serious, life-threatening headache?

  • may first need to assess whether the patient is fully oriented before proceeding with further history (Mini-Mental State Examination)
  • If patient shows a mental status deficit, transport for immediate emergency treatment

Warning Signs of Serious Causes of Headache:

  • Stiff neck
  • Disturbed consciousness
  • Abrupt onset of severe pain (“worst headache ever”)
  • Neurological deficit
  • Progressively worsening
  • Vomiting
  • New onset in persons 50 years or older

Key Questions

•How did the headache begin?

•On a scale from 0 (no pain) to 10 (worst pain ever) how severe is the pain?

•Is there a history of recent trauma to the head?

•Was there a loss of consciousness?

•Do you notice any other symptoms associated with the headache pain?

•Do you have any chronic health problems?

* Sudden onset of a severe headache without a history of chronic headache suggests an intracerebral hemorrhage (ICH) secondary to a ruptured aneurysm or vascular anomaly.

Headache of ICH without a history of trauma is rare in children and adolescents, but the incidence increases with age, especially in persons over 50 years old or in someone who is being treated with anticoagulation therapy.

After determining the headache is not serious, how can I narrow down the causes?

Key Questions

•Where does it hurt?

  • Pain secondary to trauma or inflammation is perceived as near the site of insult
  • Tension headaches are described as a “hatband” distribution of pain by adults, but as a generalized headache or discomfort by children.
  • Orbital pain is seen with increased intraocular pressure.
  • Periorbital pain may present with sinusitis, migraine, trigeminal neuralgia, or it may be a sign of ocular disease.
  • Contraction of muscles of the head and neck cause headaches that are nonpulsatile, occurring in the occipital and paracervical region.

•What does it feel like?

•When does it occur? What makes it worse?

•How long have you had this headache?

•Can you tell when it is coming on?

  • A moderately intense, constant throbbing headache is associated with dilatation of the cerebral arteries.
  • Severe pain indicates an expanding lesion, such as a tumor or hematoma, edema, or enlargement of the ventricles secondary to hydrocephalus.
  • Migraine headache pain is steady or throbbing and is usually limited to the same side; caused by the production of various substances on dilated arteries that sensitize those arteries to pain.
  • Cluster headaches (uncommon in children) are the result of an unknown vascular change that occurs within a period of 5 minutes.
  • Tension (muscle contraction) headaches usually occur at school or work and often disappear on weekends and vacations or during periods of relaxation.

What does the chronicity of pain suggest?