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Dr Hardik K ParmarDr Hardik K Parmar
Student of Dr S.S.DesaiStudent of Dr S.S.Desai
But expression of a well made man But expression of a well made man appears not only in his face,it is in his limbs and joints also.appears not only in his face,it is in his limbs and joints also.
- walt whitman- walt whitman
Are the movements that you do not control;
you make it so fast that your cerebrum doesn´t receive the information.
Are the movements which we control.
The Nervous System transmits the information of the action to the cerebrum.
Reflex:When you blik, when somebody heats you, you shake.
Voluntary movements: when you walk, you throw a ball, when you eat, …
We blink 20.000 times a day.
Yawns are contagious.
The sneeze is an involuntary movement.
Basal ganglia are group of the neuclei located subcorticallywhich take part in motor movements of body.
Abnormal increment or decrement in its parts causes various movements disorders.
Hyperkinetic hypokinetic
Tremor(MC) PD(2nd MC)
Chorea Apraxia
Dystonia Hypothyroid slowness
Ballism rigidity
Myoclonus
Tics
Ataxia
myokymia
myorrhythmia
Hypnogogic myoclonus
Benign fasciculation without LMN disorder
Physiological tremorLow amp, high freq
DISORDER LESION
Chorea Striatum, STN(red neucleus)
Athetosis Diff hypermyelination of corpus striatum & hypothalamus
Dystonia U.K.(Basal motor neuclei)
Hamiballismus H’age in C/l STN
Rest & postural tremors
Mid brain, sup cerebeller pudencle
RhythmicInvoluntary movementsOf fingers, hand, arms, legs,tongue, or headDue to alternatecontaction and relaxation of oppo mus groups
Sometimes they can be so fine that they cant be easily recognised
Put a paper on dorsum of an out streched hand
MC cause is anxiety OTHERS: Psychogenic,Post traumatic(2-8 Hz), Rx: Propranolol, Primidone, gabapentine, BZD
Athotosis, Ballismus, Chorea & DystoniaShould not be thought as a separate entity but as a different manifestation of same spectrum as they often coexist
Tics:cant be suppresed by voluncontrol
“rapid, brief, shock-like, jerky, involuntary movements”
May be caused by active muscle contraction - positive myoclonus
May be caused by inhibition of ongoing muscle activity- negative myoclonus ( eg. Asterixis )
Generalised - widespread throughout body Focal / segmental – restricted to particular part of body
Hypnogogic: occurs during sleep
Action myoclonous: asso with voluntary movements
Reflex/startle:In response to external stimulus
Reversible:Renal failure, hypocalcemia
D/D from tics: interfere with normal movements & not suppressible
Symptomatic i.e secondary to disease process- Neurodegenerative eg. Wilson’s
disease- Infectious e.g CJD, Viral encephalitis- Toxic e.g. penicillin, antidepressants- Metabolic - anoxic brain damage
- hypoglycemia- hepatic failure ( “
asterixis” )- renal failure- hyponatremia….. And
others
Valproic acid is drug of choice
May respond to benzodiazepines e.g. clonazepam, piracetam, primidone, lamotrignine
Rapid, flinging, rotatory, Violent movement of larger amplitude of axial or prox parts of limb, irreguler, U/l, disapp during sleep
Almost always unilateral and therefore
known as HEMIBALLISMUS
Patient may hurt himself
Can lead to exhaustion
Semi purposive, darting, jerky, short-lasting, centrifugal, affecting limbs (face & tongue sos)
Hypotonia + but reflexes are also +nt May be hyperextended joints
In the limbs chorea refers more to distal movements ( as proximal movements usually called ballismus)
Patients often attempt to conceal involuntary movements by superimposing voluntary movements onto them e.g. an involuntary movement of arm towards face may be adapted to look-like an attempt to look at watch
Chorea molles:Marked hypotonia with very minimal involun
move
Causes: rhumatic fever,encaphalitishuntington’s dispergnency(chorea graviderum)congenital(rarely)
Anoxic brain damage ( post – CPR ) Systemic lupus erythematosis Hepatic failure Endocrine - Thyrotoxicosis
- Addisons Electrolyte - Low Ca, Mg,
- High Na Polycythemia rubra vera
Mainly children / adolescentsComplication of previous group A
streptococcal infectionUsually no recent history of
infectionAcute / subacute onsetMay have behavioural problems Usually remits spontaneously
Chorea of any cause that begins in pregnancy
May represent recurrence of Sydenham’s chorea.
Most commonly associated with anti-phospholipid syndrome +/- SLE
Usually resolves spontaneously
Dopamine receptor blockers Riluzole: corticostrial glutamate release
inhibitor Remacemide: glutamate/NMDA receptor
antagonist Co Q 10: UK mecha, possible behavioural
improvement Anti convulsant: valproate
Slow, snake-like, writhing, worm-like movements of dynamic in nature starting at fingers and then spreades proximally which causes abduction & int rotation of UL
Increased on voluntary movements Disapp during sleep Can also affect face and tongue Often use term “ choreoathetosis ” due to
overlap between syndromes ( chorea referring to less smooth , more jerky movements)
Causes: CP, hepatic failure
Sustained or repetative involuntary mus contraction freq asso with twisting and assumption of abnormal postures.
Due to co-contraction of agonist and antagonist muscles in part of body
Can be thought of as an athetoid movement that “gets stuck” for a period of time; thus, a patient with choreoathetosis may perform an involuntary movement in which his hand and fingers are twisted behind his head. He may hold this position for a few moments before his hand moves back in front of his body.
The part of the movement when the limb was held, unmoving, in an abnormal position would be considered a dystonia ( may occur alone).
Idiopathic torsion dystonia(oppenheim’s
dystonia) Hereditary and sporadic forms Variable inheritence(AD) DYT 1 gene mutation on Chr-9-protein torsin A High incidence in Ashkenazi Jews Onset may be in childhood / adulthood(<26yr) Affects limbs then progress prox Trial of L-dopa usually initiated Level of disability variable
DRD or segawa variant (DYT 5) Affects production of tyrosine hydroxylase
and thereby formation of dopamine 1-12yrs, foot dystonias that interefers with
walking which worsens as day progresses and disapp during sleep
Excellent response to L-dopa
Blepharospasm: involuntary forceful closure of eyes
OMD: mus of lower face, lips, tongues(MEIG’S syn is combo of bleph &OMD)
Torticollis :Tendency of neck to twist to one side.
spasmodic dystonia: involves vocal cords(choking due to adductor mus involv)
Limb dystonias: writer’s cramps, musician’s cramp
Secondary dystonias: neuroleptics, chronic levodopa Rx, CO poisioning
Dystonia plus syndromes: as a part of other neurodegenrative disorder HD, PD, Wilson, CBGD, PSP etc..
Botulinum toxins Medical:
Dopa antDopa depleting agentsAnticholinergics(trihexyphenidyl)BeclofenClonazepamAnticonvulsant
Sx:Peripheral dennervationDBSU/l thalamotomy
Recurrent, sterotyped, seemingly purposeless abnormal movements
May be suppressed voluntarily or with distraction
Voluntary suppression leads to anxiety and a build-up of internal unrest.
Worsen under stress
Education clonidine Guanafacine Atypical neuroleptics(resperidone,
olanzapine) Classical neuroleptics(haloperidol,
fluphenazine) Behavioural therapy
Movement disorders are often difficult to define precisely, but have similar differential diagnoses.
They are often a manifestation of a more widespread neurological or internal medical problem.
Other than the specific treatments mentioned, most details of therapy are beyond the scope of this lecture
In some cases treatment includes treatment of underlying cause e.g. Wilson’s disease
Harrison’s priciples of internal medDe jong’s neurologyYellow oza & Dr S.S. desaiDr Sheetal D vora