Tremor. Mario Zappia. Università degli Studi di Catania

Transcription

1 Tremor Mario Zappia Università degli Studi di Catania

2 Tremor: Definition Rhythmical, Involuntary, Oscillatory movement of a body part Classification of Dyskinesias Based on Rhythmicity Rhythmical Dyskinesias: Tremor Clonus Myorhythmia (Myoclonus) (Dystonic Dyskinesias) Not- Rhythmical Dyskinesias: Chorea Athetosis Dystonic Dyskinesias Ballisms Tics and Stereotypes Myoclonus

3 Clinical Assessment of Tremor Syndromes Topography: Head (Chin, Face, Tongue, Palate), Limbs (Distal, Proximal), Trunk Activation Condition: rest, action, goal-direction, posture, task Rhithmicity Characteristics: Amplitude and Frequency (Low < 4 Hz, High > 7 Hz), Modulation Associated Movements: mirrors and dystonic outflows Adapted from: 1998 Consensus Statement of the Movement Disorder Society

4 Clinical Assessment of Tremor Syndromes Specific examinations for assessment of: Akinesia/bradykinesia Muscle tone (including Froment s sign for the upper and lower extremity and coactivation sign for psychogenic tremor) Postural abnormalities Dystonia Cerebellar signs Pyramidal signs Neuropathic signs Systemic signs (thyrotoxicosis and so forth) Gait and stance (orthostatic tremor) 1998 Consensus Statement of the Movement Disorder Society

5 Clinical Assessment of Tremor Syndromes Specific data from the medical history: Onset of tremor Family history of neurologic diseases (especially tremor) Alcohol sensitivity Sequence of spread of tremor (including symmetry and laterality) Associated diseases (restless legs, polyneuropathy) Medication Drug abuse 1998 Consensus Statement of the Movement Disorder Society

6

7 Essential Tremor Classical and Controversial Clinical Aspects

8 Mov Disord 2010

9 P.G. (66 years old) History Tremor since 2007 RBD Examination Mild bradykinesia/rigidity upper limbs R>L Arms tremor at rest R>L Chin tremor at rest Brain MRI Subcortical vascular lesions Single-axial accelerometer Tremor at rest frequency: 4.4 Hz Spect DaT-SCAN Normal Last Visit: October No Changes.

10 Follow-up:11.1 years (range, 8-25 years)

11

12 Polygraphic recording of cervical tremor when seated. The patient looks straight ahead in A, counts backward in B Alternate pattern

13

14 S.M. (73 years old). Family History Consanguineous parents Father s sister: head and vocal tremor Two brothers: unspecified Motor Neurone Disease; unspecified Sensitive Axonal Polyneuropathy History 15 years ago: head and vocal tremor. Instability during walking Brain MRI: n.t.r. Spect DaT-SCAN: normal Blink reflex recovery cicle: enhanced R2 inhibition Genetics: negative SCA1-2-3 Patient Third Brother.

15

16 Essential Tremor Pathophysiological Aspects

17 Glassy, eosinophilic swellings in the molecular layer adjacent to Purkinje cell bodies.

18

19

20 Is ET really caused by Alcohol Consumption? possibility that participants who developed incident ET between the baseline and follow-up evaluations had preclinical ET at baseline % of the incident cases of ET had no baseline in-person clinical evaluation, so a diagnosis of ET at the time of enrollment was excluded using only the analysis of handwriting samples, with possible initial misdiagnosis and then overestimation of the incident cases at the time of follow-up.

21

22

23 Moderate Alcohol Consumption and Neuroprotection Compared with abstention, consumption of 1 to 6 drinks weekly was associated with a lower risk of incident dementia. Mukamal KJ et al., JAMA 2003 OR 1,4 1,2 1 0,8 0,6 0,4 0,2 0 J-Shape effect 1,22 0,65 0,46 0,69 <1 1 to 6 7 to Weekly alcohol consumption (drinks) A 34-year follow-up prospective population study showed wine and spirits may display opposite associations with dementia. Thus, at least part of the association for wine may be explained by components other than ethanol. Mehlig K, Am J Epidemiol 2008

24

25 Red Wine Resveratrol Protective Effect Resveratrol is a polyphenolic compound derived from grapes and red wine Produces the inhibition of microglial activation and the subsequent reduction of proinflammatory factor release Resveratrol-mediated neuroprotection has been attributed to the inhibition of NADPH oxidase Protectes dopaminergic neurons against lipopolysaccharide (LPS)-induced neurotoxicity in concentration and timedependent manners. Zhang F, Mol Pharmacol 2010

26 Essential Tremor Assessments

27

28

29 Other Tremors Clinical Examples

30 D.V. (50 years old) History Family history for gait disorders History Onset in 2010 with deflection of mood and anxiety Pain in the neck and right shoulder with associated tremor of the hand Examination Lateralization of the trunk to the right Right laterocollis Deficit of postural reflexes Hypophonia Hypomimia Rest tremor of the right upper limb Postural tremor of the upper limbs (right>left) Moderate rigidity of the upper limbs (right>left) Moderate bradykinesia of the upper limbs (right>left) Peak-of-dose Distonia/Athetosis of the right foot

31 Brain MRI Scan Axial T1 Axial T2 Axial Flair

32 Brain CT Scan

33 Q.A. (55 years old) Family Hystory Family history of involuntar movements: a son with deflection of mood, anxiety, motor and vocal tics. Hystory Onset in 2010 with generalized weakness and bradikinesia, right hand tremor. Three years ago: tinnitus treated with flunarizine. In 2010 improvement after stopping treatment with flunarizine. Two years ago: muscle cramps in the left hand and bilateral plantar introversion, not correlated with exercise. Improvement after muscle strain. Examination Hypomimia Reduced pendular movements Slight postural and kinetic asymmetric tremor of the upper limbs Moderate rigidity of the trunk and upper limbs Slight bradikinesia of all four limbs Hyperreflexia of the deep tendon reflexes in all four limbs

34 Brain CT Scan

35 Movement disorders accounted for 55% of the total symptomatic patients

36 Hereditary motor-sensory neuropathy and movement disorders. Cardoso FE, Jankovic J. Muscle Nerve Sep;16(9): S.S. (54 years old) Hystory Age 40: lower limbs weakness, unsteadiness, gait disturbance

37 B.E. (55 years old) History 5 years ago writing trouble with tremor October 2010: tinglings to the left limbs Neurological examination Hypoactive deep tendon reflexes Not-elicitable plantar reflexes Mild action tremor and writing tremor Rhynopharynx Cyst Blood Exam: Anti-Chlamidia Pneumoniae IgA/IgG, NSE (low) (anti-hcv neg.) CSF exam: proteins (high) EMG: sensitive-motor neuropathy SSEP lower limbs: central conduction time (high) VEP: left P100 latency (high) MTS: Upper limbs: MEPS latency (high); Left lower limb: central motor conduction time (high)

38 Brain MRI Scan

39

40

41 R.R. (69 years old) History Ischemic Heart Disease In 2010 sudden weakness right limbs and dysarthria. Remission after days. Examination Weakness of lower facial muscles on the right side Spastic gait to the right Pseudo-rhythmical distal athetosis on the right hand with abnormal posture and loss of proprioception ( thalamic hand )

42 Brain MRI Scan

43 Myoclonic dystonia with predominating myoclonus and thalamic hand associating dystonic posture and slow, pseudo-athetoid movements, both related to controlateral lesions in the Vim and Vc nuclei of the Thalamus.

44 S.G. (60 years old) History Hypertension Neurosensorial hypoacusis Depression treated with neuroleptics and antidepressants Onset since 2 years ago with left hand tremor Neurological examination Left upper limb rest tremor and slight bilateral postural tremor characterized by disappearance after distraction.

45