TOPICS IN NEUROLOGY: TREMOR/PSEUDOSEIZURE
Educational Objectives
| The goal of this program is to improve the diagnosis and management of tremor and pseudoseizure. After hearing and assimilating
this program, the clinician will be better able to:
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 | 1. Distinguish between essential tremor (ET) and Parkinson’s disease (PD).
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| 2. Educate patients about ET and implement a management plan.
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| 3. Identify drug-induced tremor and PD.
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| 4. Recognize red flags for pseudoseizure.
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| 5. Discuss diagnosis of pseudoseizure with patients and recommend plan of treatment.
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Faculty Disclosure
In adherence to ACCME Standards for Commercial Support, Audio-Digest requires all faculty and members of the planning
committee to disclose relevant financial relationships within the past 12 months that might create any personal conflicts of interest.
Any identified conflicts were resolved to ensure that this educational activity promotes quality in health care and not a
proprietary business or commercial interest. For this program, the faculty and planning committee reported nothing to disclose.
Acknowledgments
Dr. Reich was recorded at Current Topics in Geriatrics, presented by the Johns Hopkins School of Medicine, Department
of Geriatric Medicine, and held January 17-19, 2008, in Baltimore, MD; Dr. Noe was recorded at Clinical Reviews
2007: A Primary Care and Internal Medicine Update, presented by Mayo Clinic College of Medicine and Mayo
School of Continuing Medical Education, and held March 21-24, 2007, in Scottsdale, AZ. The Audio-Digest Foundation
thanks the speakers and the sponsors for their cooperation in the production of this program.
| TREMOR: THE INS AND OUTS OF BACK AND FORTH —Stephen G. Reich, MD, Professor, Department of
Neurology, University of Maryland, School of Medicine, Baltimore
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| Definition: rhythmic oscillation of body part, involving any skeletal muscle; regularity of oscillation distinguishes
tremor from other hyperkinetic movement disorders (eg, chorea, dystonia, ballismus)
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| Classification: based on position in which tremor maximally activated; resting tremor—occurs when limb not in
use; generally associated with Parkinson’s disease (PD); postural tremor—occurs when maintaining posture (eg,
holding arms straight out); includes essential tremor (ET) and enhanced physiologic tremor; kinetic tremor—
associated with purposeful movement (eg, touching nose with finger); includes simple kinetic tremor (movement
not visually guided), visually activated kinetic tremor (with visually guided movements), and intention tremor
(tremor worsens as target approached, ie, terminal accentuation)
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| Differential diagnosis: ET and PD responsible for majority of tremors seen in primary care practices; other possibilities
include drug-induced tremor, metabolic etiologies (eg, hyperthyroidism), and Wilson’s disease (in younger
patients); history and minimal work-up usually rule out rarer etiologies
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| Distinguishing ET from PD: primarily based on history and physical
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| Age of onset: somewhat helpful; PD usually begins between 55 and 65 yr of age; tremor that begins at much
younger or much older age not likely caused by PD
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| Duration of symptoms: ≈66% of patients with PD have tremor; these patients tend to present within 6 to 12 mo of
onset; patients with ET often report long histories (years or decades) of tremor with very slow progression
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| Family history: positive family history among first-degree relatives much more common with ET than with PD
(only ≈5% of patients with PD have autosomal dominant form); detailed questions about, eg, shaky voice and
tremulous handwriting may be necessary to elicit history (signs often attributed to age)
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| Effect of alcohol: drinking small amount of alcohol tends to temporarily suppress ET but not tremor of PD
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| Physical examination: location of tremor—upper limbs commonly involved in ET and PD; tremor involving head
or voice usually signals ET; tremor of lips, tongue, or jaw usually signals PD; handwriting—not tremulous but
small with PD; normal-sized but tremulous with ET; associated signs—absent in patients with ET; in patients with
PD, characteristic signs include hypomimia, decreased arm swing, decreased vocal volume, trouble getting up from
chair, and bradykinesia; tremor at rest—almost always signals PD; kinetic tremor—signals cerebellar disorder only
when terminal accentuation present; otherwise, highly suggestive of ET; frequency—tremor of PD somewhat
slower than ET, but difficult to distinguish at bedside; morphology—PD tremor “pill-rolling,” primarily with flexion
and extension; lateralization—helpful for diagnosis; tremor associated with PD almost always begins unilaterally;
ET almost always bilateral, but may be asymmetrical (ie, worse on one side)
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Essential Tremor
| Epidemiology: most common movement disorder; incidence and prevalence increase with age; many patients do
not seek medical attention for tremor; etiology unknown; genetic in most cases, although gene not yet isolated;
pathophysiology—likely localizes to (or near) cerebellum (eg, inferior olive), but cerebellum structurally normal
upon imaging; risk of developing PD—controversial; ET may slightly increase risk, but most patients do not develop
PD; new-onset tremor at rest (in patient with ET) warrants follow-up
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| Outdated terminology: former terms included “benign essential tremor,” “senile tremor,” and “familial tremor”;
however, tremor not necessarily benign, because it (and associated embarrassment) may interfere with activities of
daily living [ADLs]; tremor not part of normal aging, and may occur in young patients as well; ET also may occur
sporadically (ie, without positive family history)
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| Postural ET: bilateral tremor with maintenance of posture (more prominent in certain positions); tremor persists
with intentional movement but has no terminal accentuation
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| ET of head and voice: head—rhythmic oscillation of head; may be horizontal or vertical; patient may also have
tremor in upper limbs; do not confuse with cervical dystonia (torticollis); voice—shaky; often source of embarrassment;
tremor also may involve chin and mouth
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| Diagnostic pitfalls: failure to recognize drug-induced tremor—drug-induced tremor also occurs with maintenance of
posture but has very low amplitude and high frequency; good drug history important; failure to recognize PD—
patients may have postural tremor, but other signs of PD also present; misdiagnosis of ET as PD—patients with
longstanding ET may develop parkinsonian signs (eg, gait ataxia) but do not develop dysmetria or cerebellar eye
signs; minimizing patient complaints and failure to diagnose—avoid attributing tremor to nervousness, advanced age,
or excessive use of caffeine; although these factors may exacerbate physiologic tremor, they do not cause persistent
tremor; confusion with cerebellar tremor—no terminal accentuation of tremor in patients with ET; failure to recognize
cervical dystonia—patient has jerky movements and involuntary turning or tilting of head; patients with ET of head
have normal position of head and neck; failure to reassess over time—clinical diagnosis leaves room for error
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| Treatment: most patients concerned about PD; reassurance and education about ET (eg, very slow progression) often
sufficient; treatment necessary only if tremor interferes with ADLs; assessment of treatment success—identify
and follow specific ADLs affected by tremor; tremor decreases in amplitude (but does not resolve) in ≈66% of
patients
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| Medical therapy: first-line—propranolol and primidone equally effective at reducing amplitude of ET;
propranolol—60 to 320 mg/day; long-acting formulation available; primidone—anticonvulsant; begin with
low dose (25 mg at night); increase slowly; warn patients about potential “first-dose phenomenon” (transient
dizziness, headache, and nausea may occur day after first dose); propranolol and primidone—evidence for synergism
when used together; second-line medications—less effective; alprazolam (avoid in elderly patients);
atenolol (but propranolol preferred); gabapentin; sotalol; topiramate; botulinum toxin—consider for patients
with medically refractive tremor, especially ET of head and voice (patients respond less well to oral agents,
but often don’t require treatment); can cause transient weakness
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| Surgery: thalamotomy—C-level evidence; rarely used; deep brain stimulation—device inserted into thalamus; insufficient
evidence of effectiveness in ET of head or voice; good option for medically refractory ET of upper
limbs; expert center recommended
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Parkinson’s Disease
| Presentation: unilateral or markedly asymmetric tremor at rest; rarely involves voice or head; commonly involves
chin, lips, or tongue; other signs of PD also present (eg, poor arm swing, bradykinesia); tremor suppresses with intentional
movement
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| Re-emergent tremor: tremor returns within 5 to 10 sec of maintaining posture (do not confuse with postural ET),
but remains unilateral
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| Progression: tremor may begin in lower limbs (still rhythmic, unilateral, and accompanied by other signs of PD);
ipsilateral progression (ie, up or down same side of body) occurs before contralateral progression
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| Jaw tremor: tremors involving lower face almost always signal PD (but ET of voice also may involve chin); tremor
generally present in other areas (eg, upper arms) as well
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Cerebellar Tremors
| Intention tremor: exhibits terminal accentuation; diagnosis—although patients with cerebellar disease often have
tremor, it rarely is presenting complaint; differential diagnosis—kinetic variant of ET (more likely diagnosis); other
characteristics—dysmetria; inaccurate movements; localization; cerebellar signs—ataxic voice; truncal titubation;
nystagmus; scanning dysarthria; cerebellar eye signs; “drunken sailor” gait; asymmetry—tremor may be worse on
one side
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Other Tremors
| Drug-enhanced physiologic tremor: fine postural tremor, may persist months after drug discontinued (complicates
diagnosis); important to ask about all medications taken during previous 12 mo; medications—many commonly
used drugs may enhance physiologic tremor in elderly patients; patients taking divalproex (eg, Depakote)
valproic acid, or lithium commonly have fine postural tremor (generally not problematic)
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| Drug-induced parkinsonism: typically atremulous and symmetrical; may mimic PD; concomitant tardive dyskinesia
(hyperkinetic movements) signals drug-induced parkinsonism; may take long time to resolve; take drug history
of past 12 mo; medications—antinausea agents; metoclopramide; dopamine-blocking or dopamine-depleting
agents; tardive dyskinesia—may present as mouth movements or puckering; resolution—spontaneous, but may require
several months
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| Primary orthostatic tremor: relatively uncommon, but highly distinctive; low-amplitude, high-frequency tremor
of lower extremities that only occurs when standing; characteristic complaint—patient cannot stand comfortably
but has no trouble walking; patient may not recognize presence of tremor and may become phobic about standing;
examination—instruct patient to stand unsupported as long as possible; palpate legs (low-amplitude high-frequency
tremor); consider auscultating legs (may hear tremor) or performing electromyography (EMG); etiology—likely
related to ET; treatment—some reports of response to clonazepam; speaker generally uses same agents as for management
of ET (eg, gabapentin)
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| Task-specific tremors: include primary writing tremor, dystonic writer’s cramp, and musician’s dystonia; not
present during other activities; related to ET; no tremor at rest or with maintenance of posture; treatment—same as
for patients with ET
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| Palatal tremor: formerly called palatal myoclonus; rhythmic oscillation of soft palate; types—primary (may
present as rhythmic tinnitus or ear clicking, caused by opening and closing of eustachian tube); secondary (eg, after
stroke involving posterior circulation; often asymptomatic until nearby muscles affected); component of degenerative
syndrome; psychogenic
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| Wilson’s disease: rare disorder, presents in younger patients; clinical suspicion—unexplained tremor in patient <50
yr of age; testing—serum ceruloplasmin (not serum copper); free copper (calculated); urinary copper (elevated);
slit lamp examination for Kaiser-Fleischer rings
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| Psychogenic tremor: rare in general practice
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| PSEUDOSEIZURE: DIAGNOSIS AND TREATMENT —Katherine H. Noe, MD, PhD, Assistant Professor, Department
of Neurology, Mayo Clinic Arizona, Scottsdale
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| Definition: psychogenic nonepileptic seizures (PNES; preferred term); episodes of altered movement, awareness,
and sensation, resembling epileptic seizure; etiology—psychologic, not physiologic; no abnormal electric discharges
in brain
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| Incidence: estimates vary; considered rare, relative to epilepsy, but misdiagnosis likely has resulted in underestimation;
≈30% of patients admitted to tertiary referral centers for video electroencephalography (EEG) monitoring
have PNES, not epilepsy; 20% of patients referred for surgical consultation for refractory epilepsy have PNES;
demographics—most common in young adults, but may occur at any age; 75% of cases occur in women
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| Associated psychologic disorders: somatoform disorders—somatization or conversion disorders; psychologic
distress manifests as real physical symptoms (not feigned); dissociative disorder—rare; disruption of consciousness,
perception, and memory, not associated with physical cause; legitimacy—majority of patients with PNES do
not have factitious disorder or malingering
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| Diagnostic clues: therapeutic failure of ≥2 anticonvulsants, especially if patient claims medication made condition
worse; multiple types of seizure, including nonstereotyped seizures; atypical clinical features; red flags in history;
no abnormal findings on EEG or magnetic resonance imaging; red flags—history of sexual, physical, or emotional
abuse (present in ≈25% of cases); concurrent psychiatric diagnosis (eg, depression, anxiety, posttraumatic stress
disorder, borderline personality disorder) present in most patients; chronic pain (eg, headache, pelvic pain, atypical
chest pain, fibromyalgia) present in ≈25% of cases; seizures never witnessed or always witnessed; seizures occur
only when patient awake
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| Clinical features: duration of seizure—epileptic seizures generally last ≤2 min; PNES may last much longer; PNES
movements—upper and lower body movements out of phase; side-to-side head movements; pelvic thrusting; back
arching; symptoms may wax and wane; other features—crying or screaming, before, during, or after seizure; maintained
awareness during event; ability to respond (but only in whisper or “baby talk”) during event
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| Differential diagnosis: frontal lobe seizure—associated with unusual motor manifestations; EEG findings may be
normal, even during event; temporal lobe epilepsy—aura; intense fear and anxiety; patient may have palpitations
and shortness of breath (often misdiagnosed as panic attack)
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| Discussing diagnosis with patient: scripts available (but speaker avoids); be straightforward with patient; discussion
points—symptoms real (patient not “faking it”); relatively common diagnosis (patient not alone); treatment
available; anticonvulsants—discontinue; discussion pitfalls—avoid minimizing diagnosis or prognosis (eg, “tests
are all normal”; “medication won’t help”; “it’s all in your head”); discuss diagnosis with patient in straightforward
manner
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| Treatment: discontinue anticonvulsant; educate patient and family; refer to psychiatrist if patient has underlying depression
or anxiety; mainstay of therapy—ongoing cognitive-behavioral therapy; stress management; addressing
history of abuse; pain management
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Suggested Reading
Alvarez MV, Evidente VG: Understanding drug-induced parkinsonism: separating pearls from oysters. Neurology
70:e32, 2008; Alvarez MV et al: Differentiating Parkinson’s disease from other parkinsonian disorders. Semin Neurol
27:356, 2007; Dodrill CB: Do patients with psychogenic nonepileptic seizures produce trustworthy findings on
neuropsychological tests? Epilespia 49:691, 2008; Haddad PM, Dursun SM: Neurological complications of psychiatric
drugs: clinical features and management. Hum Psychopharmacol 23(Suppl 1):15, 2008; Lorenz D et al:
Evaluation of a screening instrument for essential tremor. Mov Disord Mar 31, 2008 [Epub ahead of print]; Kuyk J et
al: Psychological treatment of patients with psychogenic non-epileptic seizures: an outcome study. Seizure Apr 3,
2008 [Epub ahead of print]; Louis ED et al: Dietary epidemiology of essential tremor: meat consumption and meat
cooking practices. Neuroepidemiology 30:161, 2008; Lyons KE, Pahwa R: Deep brain stimulation and tremor. Neurotherapeutics
5:331, 2008; McKeon A et al: Whole-body tremulousness; isolated generalized polymyoclonus. Arch
Neurol 27:356, 2007; Poewe W: Non-motor symptoms of Parkinson’s disease. Eur J Neurol 15(Suppl 1):14, 2008;
Reuber M: psychogenic nonepileptic seizures: answers and questions. Epilepsy Behav 12:622, 2008; Shill HA et
al: Pathologic findings in prospectively ascertained essential tremor subjects. Neurology 70:1452, 2008; Tan EK et
al: Evidence of increased odds of essential tremor in Parkinson’s disease. Mov Disord Mar 28, 2008 [Epub ahead of
print]; Weintraub D et al: Parkinson’s disease – Part 1: pathophysiology, symptoms, burden, diagnosis, and assessment.
Am J Manag Care 14(2 Suppl):S40, 2008.
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