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