Chorea

Chorea is derived from the Greek word ‘choreia’ meaning a dance. are rapid,
purposeless, irregular, jerky movements that seem to flow randomly from one
part of the body to another.

Causes

Chorea has many causes which can be simply divided into inherited and

 acquired :1-Inherited causesHuntington’s diseaseWilson’s disease

 2-Acquired causesStrokeDrug induced:(Levodopa and other

dopaminergic drugs, Antipsychotic drugs, Oral contraceptives,
Phenytoin)Chorea gravidarum(during
pregnancy)ThyrotoxicosisSystemic lupus
erythematosus/antiphospholipid syndromePost-infective Sydenham’s
chorea

 Huntington’s diseaseHuntington’s disease (HD) is a slowly progressive

autosomal dominant neurodegenerative disorder and the most
important inherited cause of chorea discovered by George Huntingtonin
In 1993, the causative gene defect was identified as a CAG triplet
repeat expansion mutation in the HD gene on chromosome 4. Onset is
usually in adult life with a mean age of about 40 years It progresses
inexorably, with death occurring 15–20 years from HD.Clinical
features Motor featuresSubtle motor abnormalities seen early in the
disease include general restlessness, abnormal eye movements, and
fidgety movements of fingers, hands and toes during stress or when
walking. Also Dysarthria.

5 Cognitive featuresCognitive abnormalities are variable but universal in HD.

The key cognitive abnormalities seen are impaired executive function with
poor planning and judgment, disorganized actions.Psychiatric
featuresPsychiatric symptoms are common, particularly depression and
anxiety. Irritability is also very common and some patients become
aggressive.

6 Investigation MRI imaging to exclude stroke

Wilson screen(Serum caeruloplasmin, serum copper ,Urinary copper level and
KF ring)Pregnancy testAnti-dsDNA,ANAThyroid function testsAntistreptolysin
O (ASO) titre
7 TreatmentNo intervention currently slows or halts the degenerative
process.Drug used to treat choreaChorea usually responds to treatment with
dopamine receptor blockers or dopamine depleting agents:.dopamine receptor
blockers eg.,Olanzapine,Risperidone,QuetiapineDopamine-depleting agents
eg.Reserpine,TetrabenazineBenzodiazepines eg. ClonazepamDrugs Used to
Treat Depression and irritability in Huntington’s Disease:Selective serotonin
reuptake inhibitors eg.FluoxetineTricyclic anti depressants eg.Nortriptyline

8 Sydenham’schorea (SC):
One of the major manifestations of rheumatic fever, appears months after the
index streptococcal infection in children with rheumatic fever,The usual clinical
picture of SC is the insidious development of choreic movements in a girl
during childhood or adolescence. The chorea evolves over weeks, then
recedes within 6 months .Treatment: is valproicacid .

9 Dystoniais a disorder marked by the presence of involuntary, sustained

muscle contractions causing abnormal postures. Pain may accompany the
motor symptoms of dystonia.

10 Classification of Dystonia
Body DistributionFocal: single body regionSegmental: contiguous body
regionsGeneralized: both legs and at least one other body regionMultifocal:
noncontiguous body regionsHemidystonia: involving one half of the
bodyEtiologyPrimary: Dystonia is only sign; may be sporadic or
inheritedSecondary: Dystonia occurring associated with a defined etiology or
associated with other neurologic abnormalities

11 Primary dystoniaYoung onset dystonia (before the age of 26 years) most

commonly manifests with limb onset dystonia, followed by subsequent
generalization In severe cases, patients can suffer disabling postural
deformities and was referred to in the older literature as dystonia musculorum
deformans or Oppenheim’s dystonia It has an autosomal dominant
inheritance. This condition is more common in Ashkenazi Jewish, with a
possible founder in Eastern Europe about 3 centuries ago.

12 Focal DystoniasThese are the most common forms of dystonia. They

typically present in the fourth to sixth decades and affect women more than
men. The major types areblepharospasm—dystonic contractions of the
eyelids with increased blinking that can interfere with reading, watching TV,
and driving.Oromandibular dystonia (OMD)Cervical dystonia—dystonic
contractions of neck muscles causing the head to deviate to one side
(torticollis), in a forward direction (anterocollis), or in a backward direction
(retrocollis).task-specific dystonia such as handwriting (writer's cramp), playing
a musical instrument (musician's cramp)
14 Secondary DystoniasThese develop as a consequence of drugs or other
neurologic disorders eg: Wilson disease ,levodopa,meticlopromide
etc...Investigation:MRI of brain and cervical spinWilson screen eg(serum
copper ,serum ceruloplasmin ,KF ring)Genetic study

15 Treatment Treatment of dystonia are:

treatment of a primary underlying condition is available eg: Wilson's
diseaseanticholinergics (e.g., trihexyphenidyl)Baclofen(muscle
relaxant)Clonazepam (bezodiazepin)Tetrabenazine(dopamin depleting
drug)Botulinum toxin has become the preferred treatment for patients with
focal dystoniaDBS of the pallidum can provide dramatic benefits for patients
with primary dystonia.

16 Oculogyric crisisIs the name of a dystonic reaction to certain drug or

medical condition .the term "oculogyric " refer to rotating of eyeballs also crisis
associated with backwards and lateral flexion of the neck ,widely opened
mouth, toungue protrusion and agitationCauses :some eg:Neuroleptic drugs
eg: olanzapinMetoclopramid and domperidonTreatmentantimuscarinic
eg:procyclidin ,benzatropindiphenhydraminediazepam

17 TicBrief, repeated, stereotyped muscle contractions that are often

suppressible. Can be simple and involve a single muscle group or complex
and affect a range of motor activities.Tourette's Syndrome (Ts)TS is a
neurobehavioral disorder named after the French neurologist Georges Gilles
de la Tourette, It predominantly affects males, TS is characterized by multiple
motor tics often accompanied by vocalizations (phonic tics). Associated
behavioral disturbances include anxiety, depression, attention deficit
hyperactivity disorder, and obsessive-compulsive disorder.

18 TreatmentPatients with mild disease often only require education and

counseling (for themselves and family members). Drug treatment is indicated
when the tics are disabling and interfere with quality of life. Therapy is
generally initiated with the alpha-agonist eg: clonidine and Guanfacine also
atypical antipsychotic can be used ,the DBS(deep brain stimulation) is going
on .Hemifacial spasmThis is a benign, usually painless but often distressing
condition, characterized by unilateral, involuntary, irregular tonic or clonic
contractions of muscles supplied by the facial nerve. It is now generally
accepted that hemifacial spasm is usually caused by extrinsic compression of
the root entry zone of the facial nerve, generally by vascular structures such.

19 as the vertebral or basilar arteries or their branches .

Drug treatment with carbamazepine, gabapentin, clonazepam and baclofen
are rarely very effective and seldom result in resolution of symptomsBotulinum
toxin injection into affected muscles is now the first line for those patients who
want treatmentSurgical decompresion.

20 Athetosis Hemiballismus
Hemiballismus is a violent form of chorea composed of wild, flinging, large-
amplitude movements on one side of the body. Proximal limb muscles tend to
be predominantly affected. The movements may be so severe as to cause
exhaustion, dehydration, local injury, and in extreme cases, death. The most
common cause is a partial lesion (infarct or hemorrhage) in the subthalamic
nucleus (STN).AthetosisSlow, distal, writhing, involuntary movements with a
propensity to affect the arms and hands.causes :the same as chorea.