Movement disorders

Dr. Mohamad Shaikhani.

1.Tremor:Rest tremore of parkisonism.Action tremoreFlabing tremor of organ failureIntention tremore of cerebellar diseases2.Myclonus3.Tics 4.Extrapyramidal movement disorders

CAUSES OF ACTION TREMOR :Exaggerated physiological tremor Essential tremor (familial) Wilson's disease Postural ('Holmes', 'rubral') tremor Multiple sclerosis Intention tremor of cerebellar lesions

CAUSES OF EXAGGERATED PHYSIOLOGICAL TREMOR

Anxiety/Fatigue Endocrine Thyrotoxicosis Cushing's disease Phaeochromocytoma Hypoglycaemia Drugs β-agonists (e.g. salbutamol) /Theophylline Caffeine /Lithium Dopamine agonists /Sodium valproate Tricyclics /Phenothiazines /Amphetamines Toxins Mercury /Lead /Arsenic Alcohol withdrawal

CAUSES OF ASTERIXIS (flabing tremor)Renal failure Liver failure Hypercapnic Resp Failure Drug toxicity (e.g. phenytoin) Acute focal parietal or thalamic lesions

Myoclonus:Brief, isolated, random, non-purposeful jerks of muscle groups in the limbs, may occur normally at the onset of sleep (hypnic jerks). A myoclonic jerk is a component of the normal startle response which may be exaggerated in some rare (mostly genetic) disorders. Unlike EP movement disorders, myoclonus may occur in disorders of the cerebral cortex, when groups of pyramidal cells fire spontaneously.Causes: Myoclonic epilepsy in which the jerks are fragments of seizure activity. Subcortical structures.Rarely, from diseased segments of the spinal cord.

Treatment:Myoclonus, especially of cortical origin, often responds to

clonazepam, sodium valproate or piracetam.

Tics: Repetitive semi-purposeful movements as blinking, winking, grinning or screwing up of the eyes. Distinguished from other involuntary movements by the ability of the patient to suppress their occurrence, at least for a short time. An isolated tic may be no more than a mild embarrassment, but may become frequent at certain times in childhood & then disappear. The uncommon syndrome of Gilles de la Tourette consists of a tendency to multiple tics & odd vocalisations, with obsessive behavioural abnormalities. The pathogenic basis is not understood, but there may be some

response to major tranquillisers.

ExtrapyramidalMovement disorders

Movement disorderMovement disorder Site of lesion.Site of lesion.

Parkinsonism.

Chorea.Dystonia& athetosis.

Hemibalismus.

Parkinsonism.

Chorea.Dystonia& athetosis.

Hemibalismus.

Substantia nigra & corpus striatum.

Caudate nucleus.Putamin & corpus striatum.

Subthalamic nucleus.

Substantia nigra & corpus striatum.

Caudate nucleus.Putamin & corpus striatum.

Subthalamic nucleus.

Other movement disorders:

Chorea

Hemibalismus.

Athetosis & dystonias:

Tardive dyskinesia:

Chorea, athetosis, ballism & dystonia :

Non-rhythmic involuntary movements may be combinations of fragments of purposeful movements & abnormal postures. All due to imbalance of activity in the complex basal ganglia circuits.

Hemibalismus:

•Treatment in the same as chorea & sometimes by stereotactic thalamatomy.

More dramatic ballistic movements of the limbs usually occur unilaterally (hemiballismus) in vascular lesions of the subthalamic structures.

Wild flailing proximal movements of the arms & legs on one side of the body due to small vascular lesions of the contralateral thalamic nuclei occurring usually in elderly & ceases within few weeks .

Chorea(dance in Greek) : �Jerky semipurposive uncontrollable movements of limbs , face & trunk ,increase with anxiety & disappear during sleep.Jerky, small-amplitude, purposeless involuntary movements. In the limbs they resemble fidgety movements, & in the face, grimaces. They suggest disease in the caudate nucleus (as in Huntington's disease) or excessive activity in the striatum due to dopaminergic drugs used in Parkinson disease.

Chorea :causes Hereditary Huntington's disease /Wilson's/Neuroacanthocytosis Porphyria Paroxysmal choreoathetosis Cerebral birth injury (including kernicterus)Cerebral trauma Drugs Levodopa /Dopamine agonists /Phenothiazines /Tricyclics Oral contraceptive Pregnancy Endocrine: Thyrotoxicosis /Hypoparathyroidism /Hypoglycaemia Infective/inflammatory Post-streptococcal (Sydenham's chorea) Henoch-Schönlein purpura Creutzfeldt-Jakob disease Antiphospholipid antibody syndrome SLE

Vascular: Lacunar infarction /Arteriovenous malformation

Athetosis & dystonias:

• Athetosis is slow writhing distal movements usually of fingers , hands ,toes & feet.

• 1. To twist, as in pain, struggle, or embarrassment.

• 2. To move with a twisting or contorted motion.

• 3. To suffer acutely.

• Dystonias are sustained abnormal postures of limbs , neck ,trunk, torticolis, tongue protrusion or fixed upward deviation of the eyes ( occulgyric crisis). Both caused by birth injuries , kernictrus , hypoxia, vascular lesions & phenothiazines.

• These are often combined with chorea (& have a similar list of causes)& are then termed 'choreo-athetoid' movements.

Athetosis & dystonias:

• Describe the movement disorder in which a limb (or the head) involuntarily takes up an abnormal posture.

• This may be generalised in various diseases of the basal ganglia or may be focal or segmental, as in spasmodic torticollis when the head involuntarily turns to one side.

• Other segmental dystonias may cause abnormal disabling postures of a limb to be taken up during certain specific actions, such as in writer's cramp or numerous other occupational 'cramps'.

Athetosis & dystonias:

Treatment:• Anticholinergics as benzhexol , antihistamines, diazepam &

baclofen .• Resistant dystonias as torticolis may respond to botulinium toxin

injection or stereotactic surgery.• The segmental dystonias can be treated by botulinum toxin

injection to the responsible muscles, to overcome the abnormal distribution of muscle activity for a period of time.

Tardive dyskinesia:

• Involuntary movements as facial grimacing , chewing movements ,tongue movements following the use of phenothiazine or buterphenones usually for some years.

• thought to be due to receptors super sensitivity to these drugs .

• Treatment is difficult but withdrawal of the causative drugs & giving drugs as sulpride or tetrabenazine may be helpful.

Paraneoplastic neurological features:

• Remote non metastatic neurological features of cancer.

Paraneoplastic neurological features:Brain & cerebellum.

Photorec retinal degeneration. Visual loss to blind SCC,rarely cerv

Limbic encephalitis. Agitation,confusion, dementia. SCC

Brainstem = Nystagmus,diplopia,vertigo. SCC

Subacute cerebellar degener Ataxia, dysarthria. SCC,Ovarian,breast,Hodgkin.

Opsoclonus – myoclonus. Dancing eye & feet,ataxia. Neuroblastoma, Lung Ca.

Paraneoplastic neurological features:spinal cord.

Site Features Causative Ca.

Necrotizing myelopathy. Para, quadriplegia,areflexia, sensory loss, bladder dysf.

SCC, lymphoma

Subacute motor neuropathy. Nystagmus,diplopia,vertigo. SCC

Paraneoplastic neurological features:Peripheral nerve.

Site Features Causative Ca.

Gullain Barre. Assending paralysis, areflexia. Hohgkin lymphoma

Chronic demyelinating PN. Chronic weakness & sensory loss

Lung, breast,gastric,lymphoma,myeloma.

Neuropathy with paraproteinemia.

Chronic, sensory or motor. Myeloma specially osteosclerotic type.

Opsoclonus – myoclonus. Dancing eye & feet,ataxia. Neuroblastoma, Lung Ca.

Subacute sensory & sensimotor neuropathy.

Distal sensory & motor loss with areflexia.

SCC & others.

Paraneoplastic neurological features:NMJ.

Site Features Causative Ca.

Eaton – Lambert or myasthenic syndrome.

Mentioned before. SCC,Thymoma, breast,prostate, stomach.

Myasthenia gravis. = =

Paraneoplastic neurological features:Muscle.

Site Features Causative Ca.

Polymyositis. Proximal weakness,myalgia,CMP, High CK.

breast,ovary, lung,lymphoma.

Necrotizing myopathy. Rapidly progressive Proximal weakness, dysphagia & dyspnea

SCC, other lung Cas.