Professional Documents
Culture Documents
Learning Objectives
Outline challenges to accurate and timely diagnosis of Parkinson disease (PD) in the primary care setting Compare clinical signs and symptoms of PD and other parkinsonian syndromes with those of essential tremor Recognize when neuroimaging is appropriate in the diagnostic workup of patients with suspected parkinsonian syndromes
More common in whites vs blacks or Asians More common in Midwest and Northeast vs other regions; more common in urban vs rural counties
Watts RL, et al[1]; Willis AW, et al. [2]
Not sensory
MPTP exposure
Poor response to levodopa
Hughes AJ, et al.[3]
Follow-up studyb:
Criteria applied prospectively in 100 cases 90% accuracy on autopsy
SWEDD=scan without evidence of dopaminergic deficit; indicates misdiagnosis of parkinsonism. Seibyl J, et al.[5]
Less common
ALS-Parkinsonism-Dementia/of Guam Alzheimer with extrapyramidal signs Rigid variant of Huntington disease
Parkinsonian Syndromes
Parkinsonian syndromes refer to neurologic disorders associated with a loss of dopamine neurons
PD (~85% of cases of parkinsonism)a MSA (< 5%)a PSP (< 5%)a
Does not include cases of clinical parkinsonism where there is not an associated loss of dopamine neurons
Drug-induced parkinsonism (7%-35% of all cases of parkinsonism)a,b,c
a. Rajput AH, et al[6]; b. Kgi G, et al[7]; c. Thanvi B, Treadwell S[8]; d. Thanvi B, et al.[9]
What Is ET?
ET is a specific neurologic disorder Also known as familial tremor Tremor involves hands, head, voice, sometimes legs and trunk Essential means
There is no other cause for tremor There are no other neurologic signs
Manifestations of ET
Symmetric tremor Tremor is the single manifestation (can include hand, vocal, head, trunk, leg tremors) Postural, kinetic (associated with action) tremor
ET frequently is not diagnosed ET usually starts age 40 y but can be seen in childhood ET symptoms start insidiously and progress slowly ET is not dangerous but can be disabling
Louis ED.[10]
Is ET Related to PD?
NO, NO, NO
ET tremor differs from PD ET patients do not feel slow or stiff ET patients do not have difficulty walking ET does not progress into PD Some patients can have both diagnoses
Areas of Uncertainty
Definite PDa Asymmetric tremor Areas of Clinical Uncertaintyb-f Mixed tremor types Definite ETg Symmetric tremor
Resting tremor
Bradykinesia
Questionable bradykinesia
Questionable response to dopaminergic therapy
Rigidity
Ambiguous/Overlapping Symptoms PD symptoms ET symptoms + postural and kinetic tremors + symmetric tremor + symmetric tremor + no bradykinesia + no rigidity + rest tremor + cogwheeling present
a. Lees AJ, et al[11]; b. Hauser RA, et al[12]; c. Pahwa R, et al[13]; d. Hauser RA, et al[14]; e. Solida A, et al[15]; f. Marshall V, et al[16]; g. Bhidayasiri R.[17]
Case Presentation
Woman age 60 y presents for evaluation of a long-standing tremor 7-year history of bilateral symmetric hand tremor that occurs with activity and is especially bothersome when she writes or holds a cup Tremor improves with wine. Her father had a similar tremor Not bothersome but has increased in past several months Recently noticed occasional right-arm tremor at rest
Dopamine D2 Receptor
Mitochondrion
Dementia with Lewy bodies, PD with dementia, MSA, PSP, corticobasal degeneration Vascular parkinsonism ET Drug-induced parkinsonism Psychogenic parkinsonism Alzheimer disease
Case Conclusion
The patient had a history of long-standing ET supported by a positive family history (present in 50% of patients) and response to alcohol In addition to the bilateral action tremor typical of ET, the examination demonstrates subtle features of asymmetric rest tremor, suggestive of early PD The clinical diagnosis is ET but you are concerned that she may have early PD in addition Two possible approaches
Clinical follow-up to see if she develops clearer features of parkinsonism Obtain a dopamine transporter SPECT scan
Summary
PD and ET are clinical diagnoses, but symptoms can sometimes overlap, causing misdiagnosis Timely and accurate diagnosis of PD and ET will advance appropriate treatment and improve the patients quality of life Patients with atypical symptoms may benefit from dopamine transporter SPECT to differentiate between a parkinsonian syndrome and ET