2Extrapyramidal system consists of: cortical areas 4, 6, 8the basal ganglia: n. caudatus, n.lenticularis (putamen, globus palidus)the nuclei of brain stem (black substance, red nucleus, vestibular nuclei, reticular nuclei, nucleus of Darkshevych, Lues’ body, lower olives)spinal cord: -motor neurons and -small motor neurons, which are located in anterior horns of the spinal cord
3Two parts of Extrapyramidal system: Pallidumglobus palidusblack substancered nucleivestibular nucleinucleus of Darkshevychlower olivesLues bodyPallidum is phylogenetically older then striatum. That’s why in newborn babies pallidum dominates
4Two parts of Extrapyramidal system: Striatumcortical areas 4,6,8n. CaudatusPutamenOnly at the age of 4 – 5 months old striatum starts to influence on motor functions
6Connections of Extrapyramidal system: Pallidum and striatum are closely connected with each other by means of such pathways:nigrostriatal (dopaminergic) – black substance – nucleus caudated – it inhibits the neurons of striatumstrionigral (GABA-ergic)- nucleus caudated (GABA) – black substance – it controls production of dopamine.
8Functions of Extrapyramidal system It prepares muscles to smooth economical movementsIt determines the postureIt makes automatical involuntary regulation of active conscious movementsIt provides automatical stereotyped movements and reflector protective movementsIt provides motor manifestation of emotions
9Extrapyramidal system’s lesion: Parkinson’s syndrome (lesion of pallidum)Syndrome of involuntary movements (lesion of striatum)
10Parkinson’s diseaseIn 1817 – James Parkinson described the major manifestation of this syndromeIn 1874 – this disease was called after James Parkinson – Parkinson’s diseaseIn 1920 – Tretiakov noticed that the greater cell loss in substantia nigra, the lower concentration of dopamine is in striatum and more severe the degree of clinical Parkinsonism.
11E t i o l o g yAccording the modern investigations in base of this disease is inborn dificiency of tyrosintransferase enzyme in dopamine.Parkinsonism may be defined in biochemical term as an inborn dopamine deficiency state.
12Basic symptoms of Parkinson’s syndrome: Hypokinesia (Akinesia)RigidityTremor
13The main pathogenetic mechanisms: Great cell loss in the substantia nigra, low concentration of dopamine in striatum, the influence of striatum on pallidum. As a result akinesia occurs.The main cause of rigidity is increasing of tonic reflex on muscles tension.The main source of tremor is thalamus ( its nucleus ventrolateralis ).
14Hypokinesia (Akinesia) Bradykinetic and akinetic stateThe gait is shuffling and the steps are slightParallel footprintsThe loss of associated swinging of the arm or arms when walking - (acheirokynesis)A lack of mobility of facial expression (Bechterev’s symptome)Infrequent blinking of the eyelids (Mary’ s symptome)Fixed look
15Hypokinesia (Akinesia) Inertia of rest (that means it is very difficult for patient to start moving)Inertia of movement (the so-called propulsive gait, lateropusion or retropulsion)Micrography – handwriting is too smallSpeach is quite and inexpressive (bradylalia)Paradoxical kynesia is possible after strong impression or great emotions
16plastic type of increased muscle tone: Rigidityplastic type of increased muscle tone:Cogged-wheel symptom (in carporadial joint)Tonus increases in course of evaluation of nervous system stateTonus is expressed in the same manner in the group of flexors and extensors
17Tremor:Are much more expressed in distal parts of extremities, sometimes tremor of lips or lower jaw can occurIt looks like coins countingIt is much more expressed while resting. It disappears or decreases while movingIts frequency is 3 – 6 times per second
18Other features of Parkinson’s disease Bradyphrenia (thoughts are too slow)Bradymnesia (recollection is slow too)While speaking such patients are boring (akairia - Astwatsaturov symptome)Usually they are in a bad mood. Depression is very typical for the patients with Parkinson diseaseSometimes they have autonomic disordersParasympathetic nervous system dominates in such patients – they have running saliva (aeriel symptome), hyperhydrosis, fatty skin and type of hair, bradicardia and arterial hypotension
19The symptom of air pillow or Vartenberg symptom – T e s t s:The symptom of air pillow or Vartenberg symptom –The patient is lying down. One props up his head a little bit and then quickly takes his hand out. Normally the head is falling down. But in patients with Parkinson disease the head stays in the same position for a while.
20T e s t s:Westfahl’s phenomena of paradoxical muscle constriction – While foot extension it stays in the same position for a while.Hand extension test.Test of knee flexion – The patient is lying on his abdomen; his lower extremities are bended in knees. In patients with Parkinson disease the legs are fixed in this position for several minutes.
21The symptom of Noica–Haneva Tests: While evaluations of muscle tone one asks the patient to raise his opposite extremity. In patients with Parkinson disease the tonus suddenly increases.
22Clinical forms of Parkinson disease: Rigid (hypokynesia dominates)TremblingMixed :rigid – trembling or trembling – rigid
23The degree of severity (according to Petelin): The first degree - expressed one or two main symptoms. The patient preserves professional and home activityThe second degree – The patient is disabled professionallyThe third degree – The patient cannot take care of himself
24Symptomatic Parkinsonism Craniocerebral trauma ( Muhammad Ally)CO, Mn intoxicationBrain tumorEncephalitis (Economo)StrokesCerebral atherosclerosisMedicinal parkinsonism (reserpinum, neuroleptics)Poisoning of heavy metals
27Hyperkynetic–hypotonic syndrome: The main clinical signs of this syndrome are:Muscular hypotoniaInvoluntary movements – hyperkynesis.Involuntary movements are characterized by:the amplitude of the movementlocation of muscle involvedthe ratethe duration of contraction and the relaxation
28Kinds of hyperkynesis Chorea Athetosis Choreoathetosis Ballism and hemiballismMyoclonusTorsion spasmTicsFacial crampTremor
29C h o r e aspontaneous, irregular, purposeless and asymmetric movementsthey are present at rest and subside during sleepeyes and tongue symptom ( or Hersonsky’s symptom ) - the patients are unable to maintain tongue protrusion for more than a few seconds;Hordon’s II symptom – while checking knee – reflex crus stays in the position of extension for a while and then slowly goes down;Cherni symptom - pathologic sudden abdomen at breath.The most common diseases - Huntington’s chorea (inherited disease), rheumatic subcortical encephalitis or chorea Sydenhams (juvenile disease), atherosclerotic chorea, chorea gravidarum, electric chorea.
30A t h e t o s i sMovements are slower and more sustained than choreiform movementsthey affect primarily the distal portion of extremitiessnakelike movement of any combination of flexion, extension, adduction and abduction in varying degreesThey are regularly associated with increased muscular toneIt is supposed that athetosis is the result of nucleus caudated lesion.Choreoathetosisis a term selected to describe movements that are intermediate between chorea and athetosis.
31Ballism and hemiballism Its the more or less continues gross abrupt contractions of axial and proximal muscles of the extremitiesIn the most cases this movement disorder is confined to one side of the body (hemiballism)It may be associated with hypotonia
32Torsion spasm Twisting or turning movements The muscles of trunk and neck are involvedSometimes torticollis can occurUsually it is the result of putamen lesion
33MyoclonusIs a jerking movement of one or more muscle groups (for example palatine, tongue, pharynx, larynx, diaphragm and skeletal muscles)Usually only one muscle group is involvedThey are synchronous in most of cases and sometimes they are asynchronousTheir frequency is about 15 – 18 per minuteThey may be induced by visual, tactile, or auditory stimuli (stimulus-sensitive myoclonus) or by the initiation of the voluntary movement (intention myoclonus).
34T i c s are an involuntary compulsive stereotyped movements they may be simple or complextics may involve any portion of the body ( they are most common about the face where they are manifest as blinking, grinning, smirking, lip licking, nose wrinkling)Facial crampis tonic seizure in facial musclesTremorIs rhythmical jerking of arms, legs or headIts frequency is about 4 – 6 per second
38C e r e b e l l u mis a reflexional organ of coordination of movements, equilibrium and muscular tone
39C e r e b e l l u m Two large lateral hemispheres Contains :Two large lateral hemispheresFlocculo-nodular lobe (vermix)Three pairs of peduncles.
40Grey matter of cerebellum Is presented by cortex of hemispheres and nuclei of cerebellum:Nucleus fastigiiNucleus globosusNucleus emboliformisNucleus dentatus
41Cerebellum peduncles: The lower peduncles (corpora restiformia) provide connection with oblong brain and spinal cord:Tr. spinocerebellaris dorsalis (Flexig’s)Tr. vestibulocohlearis (from nuclei vestibularis to nucleus fastigii)Tr. olivocerebelaris (from lower olives to nucleus dentatus)Fibre arcuate externe (from nuclei Holl and Burdach to hemispheres and vermis)
42Cerebellum peduncles: The middle peduncles (pedunculum cerebellaris medii) provide connection with ponsTr. pontocerebellaris. They connect nuclei of pons with the opposite hemisphere of cerebellum
43Cerebellum peduncles: The upper peduncles of cerebellum (pedunculi cerebellaris superior) connect cerebellum with middle brain. They include two systems:Afferent one – from spinal cord to cerebellum – tr. spinocerebellaris ventralis (Hover’s)Efferent one – from cerebellum to the structures of extrapyramidal nervous system – tr. cerebellotegmentalis et tr. dentorubralis.
44The way of cerebellum correction The first neuron – tr. fronto-temporo – occipito- pontinusThe second neuron – tr. pontocerebellaris (pontino- cerebellaris decussation)The third neuron – tr. cerebello – dentatusThe forth neuron – tr. dentorubralis (Vernekink’s decussation)The fifth neuron – tr. rubrospinalis (Forel’s decussation)The sixth neuron – tr. spinomuscularis
46The main functions of cerebellum: body equilibriumregulation of muscle tonecoordination of movementssynergy
47Hemispheres function are: Vermix function are:Equilibrium of the bodyRegulation of muscle toneHemispheres function are:Coordination of the movements of extremitiesSynergy of the muscle
48A t a x i ameans disturbances of equilibrium of the body and coordination of movements.Cerebellum lesion produces cerebellar ataxia:Static (lesion of vermix)Dynamic (lesion of hemispheres)
49Kinds of ataxias: Cerebellar (cerebellum lesion) Sensitive (loss of deep joint sense)Frontal (cortical) (lesion of Frontal lobe)Vestibular (lesion of vestibular apparatus)Hysterical (in case of Hysteria)Mixed
50Cerebellar ataxia Attactic gait – patient can’t to walk Disorders of equlibrium – patient can’t to standMuscular hypotoniaIntention tremor – is dynamic tremor (it is more expressed while moving and disappears while rest)Nystagmus: horizontal, vertical, vertical rotary, or dissociated (different in each eye)Scanning speech –patient can’t to speak slowlyMacrographia – handwriting is too largeDysmetria (disturbed ability to gauge distances)Adiadochokinesia (disturbed ability to perform rapid alternating movements)Asynergia – disturbed ability to controls function of antagonists muscles
51Symptoms of lesion Cerebellar lower peduncles lesion can cause: Cerebellar ataxiaBulbar syndromeSometimes pathology of Holl’s and Burdach’s nuclei is associated with this lesion. Then ataxia is complex and is called cerebellar – sensitive ataxia.Cerebellar upper peduncles lesion can cause:Cerebellar ataxia at the side of lesionTrochlear Nerve lesionMidbrain lesion symptoms
52Symptoms of lesion Nucleus ruber lesion can cause: Cerebellar ataxia in the opposite extremitiesResting tremorWebber’s syndrome or paresis of convergence may occurPontino – cerebellar angle lesion manifests as:Cerebellar disorders and pathology of VII, VIII, V, VI pairs of CNs at the side of lesionPyramidal and sensory hemisyndrome on the opposite side