Extra Pyramidal System Disorders PDF

29-Nov-24 Extra pyramidal system disorders Dr. Mahmoud ElSayed Midan PhD. Lecturer of PT for neurology CU,SU 1 Role of extra-pyramidal system in movement The h...

29-Nov-24 Extra pyramidal system disorders Dr. Mahmoud ElSayed Midan PhD. Lecturer of PT for neurology CU,SU 1 Role of extra-pyramidal system in movement The human motor system controls both voluntary and involuntary movements. The pyramidal motor system generates voluntary movement.  Voluntary movements are facilitated through the extrapyramidal system and  suppresses unwanted movement.  the extrapyramidal motor system also controls involuntary movement. Associated, postural, and automatic movement, 2 1  29-Nov-24 Based primarily in the basal ganglia 3 Movement disorders or extrapyramidal disorders diseases characterized by abnormal involuntary movements in conscious patients. Damage or dysfunction of the basal ganglia and their brainstem and cerebellar connections is the main cause of these diseases. The abnormal movements may be the only manifestation of a disease or part of the disease affection. 4 2  29-Nov-24 Movement disorders hyperkinetic hypokinetic Chorea, Parkinson’s disease Tremor  reduced voluntary and Myoclonus  involuntary movement. Tics cause extra involuntary movement sometimes interfere with normal voluntary movement. 5 Hyperkinetic Movement Disorders : ass\ observation  Topography task-specificity \  symmetry or asymmetry  Velocity\ rhythm \ posture-  interference with voluntary movement specificity  whether it is suppressible precipitated factors ameliorated interventions. 6 3  29-Nov-24  Chorea: Irregular, unpredictable, brief, involuntary jerking movements involving shifting muscles or muscle groups involving the arms, hands, legs, tongue or trunk. 7 Dystonia: Sustained twisting muscle contractions, repetitive movements or abnormal posture In adults, dystonia is focal in presentation, affecting the neck, the eyes, the jaw or a limb. In children, dystonia can present focally and then generalize over time. The contractions can be painful and may be disabling. 8 4  29-Nov-24 Athetosis: Distal, slow, writhing form of dystonia. It can be seen in combination with chorea. 9  Chorea: Irregular, unpredictable, brief, involuntary jerking movements involving shifting muscles or muscle groups involving the arms, hands, legs, tongue or trunk. Dystonia: Sustained twisting muscle contractions, repetitive movements or abnormal posture In adults, dystonia is focal in presentation, affecting the neck, the eyes, the jaw or a limb. In children, dystonia can present focally and then generalize over time. The contractions can be painful and may be disabling. Athetosis: Distal, slow, writhing form of dystonia. It can be seen in combination with chorea. 10 5  29-Nov-24 Ballismus: Uncontrollable, proximal, flinging movements of a limb that is often due to a lesion in the subthalamic nucleus. 11 Tics: Abrupt, brief, repetitive, stereotypical movements of face, tongue and limbs or vocalizations that may be briefly voluntarily suppressed but is often then followed by a burst of tics when the suppression is removed. 12 6  29-Nov-24 Tourette syndrome: This is a neurological condition that starts between childhood and teenage years and is associated with repetitive movements and vocal sounds. 13 Ballismus: Uncontrollable, proximal, flinging movements of a limb that is often due to a lesion in the subthalamic nucleus. Tics: Abrupt, brief, repetitive, stereotypical movements of face, tongue and limbs or vocalizations that may be briefly voluntarily suppressed but is often then followed by a burst of tics when the suppression is removed. Tourette syndrome: This is a neurological condition that starts between childhood and teenage years and is associated with repetitive movements and vocal sounds. 14 7  29-Nov-24 Myoclonus: Sudden, shock-like movements or a pause in movement. The movements can be restricted to a specific muscle or group of muscles or may be multifocal occurring at the same or different times. Myoclonus can be generated in  the motor cortex,  subcortical areas,  brainstem (reticular myoclonus),  spinal cord(propriospinal myoclonus).  Many healthy individuals experience myoclonus on falling asleep in the form of a hypnic jerk. 15 Tardive dyskinesia: This neurological condition is caused by long-term use of certain drugs used to treat psychiatric conditions (neuroleptic drugs). there are repetitive and involuntary movements such as grimacing, eye blinking and other movements.  Tremor: A tremor is an involuntary oscillation of a body part caused by alternating contractions of reciprocally innervated muscles. Physiologic tremor in present in the limbs but usually is not irritating. Stimulants or anxiety can enhance a physiologic tremor causing it to be visible and sometimes disturbing. Current evidence suggests that tremors come from alterations in a complex central oscillatory cycle that involves neurons in the basal ganglia, brainstem, and sometimes the cerebellum. Tremors are classified by frequency, their relationship to movement, and location. 16 8  29-Nov-24 Tardive dyskinesia: This neurological condition is caused by long-term use of certain drugs used to treat psychiatric conditions (neuroleptic drugs). there are repetitive and involuntary movements such as grimacing, eye blinking and other movements. 17 Tremor: A tremor is an involuntary oscillation of a body part caused by alternating contractions of reciprocally innervated muscles. Physiologic tremor in present in the limbs but usually is not irritating. Stimulants or anxiety can enhance a physiologic tremor causing it to be visible and sometimes disturbing. Current evidence suggests that tremors come from alterations in a complex central oscillatory cycle that involves neurons in the basal ganglia, brainstem, and sometimes the cerebellum. Tremors are classified by frequency, their relationship to movement, and location. 18 9  29-Nov-24 A)Parkinson’s static Disease  Idiopathic PD usually begins above age 50  patients with young-onset PD can have onset of symptoms as early as age 21–40 years.  PD has a dramatic impact on quality of life and a marked reduction in life expectancy. 19 Clinical Features The cardinal symptoms TRAP:  Tremor : resting tremor (that disappears with movement and increased with anxiety),  Rigidity,  Akinesia or bradykinesia (slowed and small amplitude movements),  Postural instability with gait changes. stooped posture shuffling gait 20 10  29-Nov-24 Parkinsonism is the secondary form Parkinson disease that develop from specific causes such as repeated head trauma (boxing), infections of the upper midbrain, medications that affect dopamine transmission, or CNS diseases that damage the nigrostriatal pathway. 21 Pathophysiology: Idiopathic PD slowly progressive death of CNS dopaminergic neurons, in substantia nigra (triggering is not known) motor symptoms of PD manifest when approximate 60–80% dopaminergic neurons are damaged  unable to facilitate normal voluntary  or inhibit involuntary Grossly:  loss of pigmentation in the substantia nigra. Microscopically,  loss of small, pigmented neurons in the substantia nigra  inclusion (Lewy bodies) in remaining. 22 11  29-Nov-24 B) Essential Tremor the most common movement disorder. Essential tremor begin at any age but more evident with increasing age. Although activities of daily living may be difficult such as  feeding,  drinking,  and writing a small percentage of patients with essential tremor seek medical attention. 23 Essential tremor can be missdiagnosed as Parkinson disease— likewise in a young patient (under 40 years), other rare causes of tremor should be excluded such Wilson’s disease medical conditions such as hyperthyroidism. 24 12  29-Nov-24 Pathophysiology Familial or genetic predisposition. Structural lesions have not been recognized. the generators of essential tremor are widespread in the brain including  the motor cortex  Thalamus  Cerebellum  brainstem, such as the inferior olivary nucleus. These are the same centers that control voluntary movement through a thalamocortical relay. 25 Clinical Features The characteristic history is slowly progressive symptoms that began in middle age Essential tremor: (postural)As the patient sustained with arms outstretched  bilateral tremors of the hands  medium to high frequency  low amplitude  absent at rest  worsens with anxiety, coffee, and some medications. 26 13  29-Nov-24 Tremor severity vary and can interfering with ADL  Writing  Drinking Tremor also may involve  Head  Legs  Voice 27 clinical diagnosis based on history and exam. the tremor is the predominant symptom. Patients with essential tremor could have ❑mild gait imbalance ❑subtle cognitive changes Not occur ❑Weakness ❑sensory loss ❑changes in deep tendon reflexes ❑not have features of Parkinson disease. 28 14  29-Nov-24 Rest tremor Present at rest with limb supported against gravity (pill-rolling Low-frequency (3–5 Hz), tremor) medium to high amplitude (parkinsonian tremor) Action Present when voluntarily maintaining a limb still against gravity tremor such as holding arms outstretched, bilateral but may be (postural asymmetric. tremor) Low to medium amplitude and medium to high frequency (4– 8 Hz) Present during voluntary movements such as writing or eating (essential tremor, enhanced physiologic tremor) Intention Present during voluntary movement, perpendicular to tremor movement (cerebellar Medium amplitude and low frequency tremor) Often amplifies when limb approaches the target or near the face Interferes with coordination (cerebellar-type tremor as seen in 29 C) Huntington’s (chorea) Disease Autosomal-dominant progressive chorea neurodegenerative disease characterized cognitive decline by behavioral disturbances usually begin in mid-life (30 to 50) except juvenile Huntington’s disease. 30 15  29-Nov-24 Pathophysiology Genetic predisposition Abnormal Huntingtin protein. Studies show it is essential in fetal development as loss of both gene copies leads to fetal death. Abnormal Huntingtin protein causes premature death of selected neuronal populations causing atrophy of the caudate and putamen nucleus and easily visible on gross inspection. Microscopically, intranuclear inclusions containing fragments of Huntingtin protein in the striatum. 31 Major Clinical Features: Irregular, progressive unpredictable, disorders of brief, involuntary Chorea worsens jerking movements over the course of movement, involving the arms, the disease in cognition, hands, legs, frequency, and of tongue, or trunk severity. behavior. characterize chorea. 32 16  29-Nov-24 Major Clinical Features:  Early in the disease, ❑ patients frequently appear restless and mask the involuntary limb movement by ❑ parakinesia incorporating involuntary jerk into a semi-purposeful movement. ❑ Rapid saccadic eye movements become difficult to initiate and quite slow. ❑ motor impersistence (can’t sustain constant voluntary muscle contraction) inability to extend tongue for any period of time or maintaining a steady handshake without varying contraction strengths (milkmaid’s grip). 33 Major Clinical Features: As the disease worsens  stiffness and slowness can appear such as ❑dystonic symptoms (rigidity involuntary muscle contractions) ❑Parkinsonism may appear.  Dysarthria develops with hypophonic irregular speech that becomes unintelligible.  Dysphagia appears late and often contributes to the death of the patient.  At this stage, the patient depends on others for help. 34 17  29-Nov-24 Major Clinical Features:  global progressive decline cognitive capabilities begins before or after chorea onset  cognitive decline lead to loss of executive functions.  Dementia present in most patients.  Behavioral problems: ❑ begin with personality changes (irritability, compulsivity, apathy, and anxiety) ❑ may appear years before the chorea. ❑ Depression develops in one-third of patients and may lead to suicide ❑ Psychosis is uncommon (5 %). 35 Juvenile HD progresses faster than adult onset of less than 20 years more prominent Parkinsonism, especially bradykinesia. marked rigidity severe mental deterioration prominent motor and cerebellar signs Dysarthria dysphagia. ❑Myoclonus ❑Tics 36 18  29-Nov-24 diagnosis Neuroimaging demonstrate ❑ progressive caudate atrophy parallels the loss of cognitive function and ❑ progressive putamin atrophy parallels with motor decline. The definite diagnosis is made with genetic testing. While The clinical diagnosis is usually made based on: onset in mid-life with typical chorea, cognitive loss, and behavioral changes positive family history neuroimaging demonstrating caudate atrophy. 37 Continue extrapyramidal system (movement disorders) 38 19  29-Nov-24 DYSTONIA neurological movement disorder characterized by: involuntary muscle contractions that cause slow repetitive movements or abnormal postures It can sometimes be painful. There are several forms of Dystonias that may affect: ▪ only one muscle ▪ groups of muscles ▪ muscles throughout the body 39 Symptoms  initially symptoms can be very mild  appeared with prolonged exertion, stress, or fatigue.  Over time, the symptoms become more prominent and widespread  Symptoms include: ▪ foot cramp ▪ tendency for one foot to turn or drag ▪ worsening in handwriting after writing several lines ▪ involuntary turning of the neck ▪ Rapid and uncontrollable blinking of both eyes ▪ Tremor ▪ Difficulty speaking. 40 20  29-Nov-24 Symptoms  Dystonia can occur at any age, but some forms are often divided as: early onset (childhood) dystonia: begins with symptoms in the arms and legs and may progress to involve other regions of the body adult-onset dystonia: usually is located in one or adjacent parts of the body, most often involving the neck and/or facial muscles. Some symptoms occur after periods of exertion or fluctuate over the day. 41 Generalized dystonia Focal dystonia Multifocal dystonia involves two or more unrelated body parts. Segmental dystonia and Hemidystonia involves two or more adjacent parts of the body 42 21  29-Nov-24 Cervical dystonia: (spasmodic torticollis)  most common focal dystonia affects neck muscles causing head turning to one side or to be pulled forward or backward. Sometimes the shoulder is pulled up. mostly first manifested in midlife. It begins slowly reaching a plateau over months or years. spontaneous remission may occur. 43 Blepharospasm: 2nd most common focal dystonia, is the involuntary, forcible closure of the eye. Start with increased blinking. Spasms may cause the eyelids to close completely,  resulting in “functional blindness” usually both eyes are affected. 44 22  29-Nov-24 Oromandibular dystonia affects the muscles of the jaw, lips, and tongue. It may cause difficulties with opening and closing the jaw,  speech and swallowing. 45 Cranial dystonia affects the muscles of the head, face, and neck The term Meige syndrome is sometimes applied to cranial dystonia accompanied by blepharospasm. 46 23  29-Nov-24 Spasmodic dysphonia (laryngeal dystonia) involves the muscles that control the vocal cords, resulting in strained or breathy speech. 47 Task-specific dystonia: (Musician's dystonia ) occurs only during a particular repetitive activity. As during writing in writer's and typist's cramp that affects hand and forearm muscles. Musician's dystonia is focal dystonia affecting musicians’ hand or mouth. 48 24  29-Nov-24 Thank you 49 25