os Neurology Edited by professor L. Sokolova ADO\OINSN Xe oN oe] ) ‘2 a iSMINISTRY OF HEALTH OF UKRAINE NEUROLOGY Edited by L. SOKOLOVA, M. D., D. Sc. Professor of Neurology Department of Neurology Bogomolets National Medical University, Kyiv Textbook for students of higher medical institutions of IV level of accreditation 4704 Npunariiad grunt naesanbuvn sana «KUL EMGYHKNY YHIB PALM» genta ued Ko 16478809 BIBNIOTEERA Vinnytsia Nova Knyha 2012YAK 616.8(075 BBK 56.12873 Ho Pexamendosauo Minicmepemeou oxopone xdopoe'a Yxpainu ax nidprunux dra cmydenmis suupe veduunue noeuerenu saxiasie IV pisxa axpedumanii, xi onanogyome naeyeriony Ouctquntiny aneriiteeKore Moso9 (npomoxo1N? 2 610 01.06. 2012p.) Contributors: Larysa Sokolova, Olena Myalovitska, Victoria Krylova, ‘Tetyana Illyash, Volodymyr Meluyk Reviewers: 1. A, Grygorova — the Head of Department of Neurology. Kharkiv National Medical University, Professor of Neurology, D, Se. L. L, Korsunska — the Head of Department of Neurology, Crimea State Medical Univer- sity named after S, 1, Georgievsky. Professor of Neurology. D. Se ¥. P. Lysenyuk — the Head of Department of Neurology and Rehabilitation Medicine, Bogomolets National Medical University, Professor of Neurology. D. Se Henpoxtoria = Neurology : Textbook for stud. of higher med. institutions H40 /L. Sokolova, O. Myalovitska, V. Krylova [et al.] : ed. by Prof. L. Sokolova. — Vinnytsia : Nova Knyha, 2012. — 280 p. + il. ISBN 978-966-382-426.0 The textbook corresponds tothe Program in Neurology for students and consists of two parts: general neurology, and special one. Each chapter covers the main topics of the course of neurology and includes new scientific data. Chapters contain test questions and clinical problems tor sel control. The textbook is provided with a list of basic concepts and terms to fucilitate orientation in the text, and a list of abbreviations that have been used. For english-speaking students of higher medical institutions with IV level of accreditation, interns, postgraduate students, attending physicians, YAK 616.8(075.8) BBK 56.1273 © Contributors, 2012 ISBN 978-966-382-426-0 © Nova Knyha, 2012CONTRIBUTORS Larysa Sokolova, M. D., D. Sc. Professor of Neurology, Department of Neurology, Bogomolets National Medical University, Kyiv » Reflex-motor function of nervous system. Syndromes of movement dis- orders » Brain cortex. Localization of cortical functions. Disturbances of higher cerebral functions » Demyelinating diseases of nervous system. Multiple sclerosis. Acute dis- seminated encephalomyelitis. Amyotrophic lateral sclerosis » Hereditary diseases with lesion of pyramidal and coordinative systems » Additional researches in Neurology Olena Myalovitska, M. D., D. Sc. Docent of Neurology Department of Bogomolets National Medical Univer- sity, Kyiv » Extrapyramidal system and syndromes of its lesion » Cerebellum and its pathology » Pathology of autonomic nervous system » Infectious diseases of central nervous system. Meningitis. Encephalitis. Myelitis. Acute poliomyelitis. Neurosyphilis » Headache. Kinds and nosological forms of headache. Migraine » Myasthenia and myasthenic syndrom. Hereditary diseases with lesion of extrapyramidal system Victoria Krylova, M.D., Ph.D. Docent of Neurology Department of Bogomolets National Medical Univer- sity, Kyiv » Cerebrospinal fluid. Meningeal and hypertensive syndromes » Closed craniocerebral and spinal trauma » Epilepsy. Nonepileptic paroxysmal states » Hereditary diseases of neuromuscular apparatus » Additional researches in NeurologyTetyana Illyash, M. D., Ph. D. Docent of Neurology Department of Bogomolets National Medical Univer- sity, Kyiv » Sensitive function and its abnormality » Cranial nerves pathology » Brain and spinal cord tumors. Syringomyelia » Diseases of the peripheral nervous system. Vertebrogenic affections of the peripheral nervous system. Mononeuropathies and polyneuropathies Volodymyr Melnyk, M. D., Ph. D. Docent of Neurology Department of Bogomolets National Medical Univer- sity, Kyiv » Cerebral vascular diseases. Slowly progressing and transient disturbanc- es of cerebral blood circulation. Brain strokeCONTENTS PREFACE.. ABBREVIATIONS ..... PART, GENERAL NEUROLOGY 1. Sensitive function and its abnormality ... see 2. Reflex-motor function of the nervous system. Syndromes of movement disorders... 24 8. The extrapyramidal systam and syndromes of its lesion. 4, The cerebellum and it's pathology... 5. Cranial nerves pathology... 6. The autonomic nervous system and syndromes ots lesion. 7. The brain cortex. Localization of cortical functions. Disturbances of higher cerebral functions .. 8. Cerebrospinal fluid. Meningealand hypertensive syndromes 9. Additional methods in neurology... PART Il. SPECIAL NEUROLOGY 10. Infectious diseases of the central nervous system M2 11. Cerebral vascular diseases. Slowly progressing and transient disturbances of cerebral blood circulation. Brain stroke...» cormamamm 180 12. Headache. Kinds and nosological forms of headache. Migraine 160 18. Closed craniocerebral and spinal trauma .....00sonnnc ee) 14, Brain and spinal cord tumors. Syringomyelia essen 180 16. Epilepsy. Nonepileptic paroxysmal states ... 194 16. Diseases of the peripheral nervous system. Vertebrogenic affections ofthe peripheral nervous system. Mononeuropathies and polyneuropathies 0.208 17. Demyelinating diseases of the nervous system. Multiple sclerosis. Acute disseminatedencephalomyelitis. Amyotrophic lateral sclerosis... 18. Hereditary diseases of the neuromuscular apparatus. Myasthenia and myasthenic syndromes, Hereditary diseases with lesion of the pyramidal, extrapyramidal and coordinative systems .. LIST OF ANSWERS.......... i 100 284 REFERENCES AND SUGGESTED READING. soeese 267, INDEX 0-269 Contents | 5PREFACE Diseases of the nervous system occupy a leading position among all dis- eases and are often caused either by primary lesions of nerve structures, or as the cause of pathology of other organs and systems. The main aim of this textbook is to provide information about symptoms and manifestations of neurological diseases and to teach students to identify the main forms of lesions of the nervous system according to the classical approach in neurology —a consistent definition of topical and clinical diagno- ses. The material is based on the authors’ experience in teaching neurology to students of Bogomolets National Medical University (Kyiv). The textbook consists of two parts: general or propaedeutic neurology, and special one. Each chapter covers the main topics of the course of neurology. Chapters contain test questions and clinical problems for self-control. The textbook is provided with a list of basic concepts and terms (index) to facilitate orientation in the text, and a list of abbreviations that have been used. Material of the textbook corresponds to the program in neurology for stu- dents of higher medical schools of IV accreditation level and includes new scientific data. Taking into consideration significant expansion of knowledge about the molecular basis of neurological diseases and the emergence of new methods of diagnosis and treatment, the book provides current medical infor- mation in all sections of special neurology. On behalf of the authors of the textbook | thank all the members of the Department of Neurology of NMU K. V. Antonenko, N. V. Domres, Yu. V. Gele- tyuk, V. Yu. Shandyuk for their help in working on the text and V. S. Dzyuba for technical support in the preparation the material for print. Also | express sincere appreciation to the publishers’ house “Nova Knyha”, led by the editor of the Department of Medical Book 0. V. Marchuk, whose efforts have made it possible to realize our ideas. L. |. SokolovaABBREVIATIONS ACA — anterior cerebral arteries ADEM —acute disseminated encephalomyelitis AF — atrial fibrillation AIDS — acquired immunodeficiency syndrome ALS — amyotrophic lateral sclerosis aPTT — activated partial thromboplastin time AVMs — — arteriovenous malformations BA — basilar artery BEB — blood-brain barrier CA — cerebral arteries CAG — cytosine-adenine-guanine CBC — cerebral blood circulation CBF — cerebral blood flow CK — creatinekinase CMTD = — Charcot — Marie — Tooth disease CMV — cytomegalovirus CNS — central nervous system CRP — C-reactive protein CSF — cerebrospinal fluid CT — computed tomography CTA — CT angiography cvD — cerebrovascular disease DNA — deoxyribonucleic acid DOPA —dihydroxyphenylalanine DVT — deep vein thrombosis DWI — diffusion weighted imaging EBV — Epstein — Barr virus ECG — electrocardiogram ECHO = —echocardiugraply EDSS — Expanded Disability Status Scale EEG — electroencephalography EMG — electromyography EP — evoked potentials Abbrewations | 7EPS — extrapyramidal system ESO — European Stroke Organisation ESR — erythrocyte sedimentation rate FA — Friedreich’s ataxia FLAIR — fluid-attenuated inversion recovery GAA — guanine-adenine-adenine sequence GABA = —gamma-aminobutyric acid 6cs — Glasgow Coma Scale HAART — highly active antiretroviral therapy HD — Huntington's disease HDL — high-density lipoprotein HHV6 — human herpes virus 6 HIV — human immunodeficiency virus HLA — human leukocyte antigen HSV — herpes simplex viruses ICA — internal carotid artery ICH — intracerebral hemorrhage IcP — intracranial pressure IgG — immunglobulin @ INR international normalized ratio la — intelligence quotient IVH — intraventricular hemorrhage LDL — low-density lipoprotein L-DOPA +—L-dihydroxyphenylalanine LGMD —limb-girdle muscular dystrophies MAP. — mean arterial pressure MBP — myelin basic protein MCA — medial cerebral arteries MD — muscular dystrophies MR — magnetic resonance MRA — magnetic resonance angiography MRI — magnetic resonance imaging MS — multiple sclerosis NAA — N-acetylaspartate NG — nasogastric PCA — posterior cerebral arteries 8 | AbbreviationsPCR PD PET PML PNS PP PT PIT Pwi RR rtPA SAH SCA SMA SMN sP SPECT TCD TIA TOE TTE VA VZV — polymerase chain reaction — Parkinson disease — positron emission tomography — progressive multifocal leukoencephalopathy — peripheral nervous system — primary progressive — prothrombin time — partial thrombin time — perfusion weighted imaging — relapsing-remitting — recombinant tissue plasminogen activator — subarachnoid hemorrhage — spinocerebellar ataxias — spinal muscular atrophies — survival of motor neurons — secondary progressive — single photon emission computed tomography —transcranial doppler — transient ischemic attacks — transoesophageal echocardiography — transthoracic echocardiography — vertebral artery — varicella zoster virus Abbreviations | °PART | GENERAL NEUROLOGY1. SENSITIVE FUNCTION AND ITS ABNORMALITY The human capacity to fee! the impact of various exogenous and endog- enous factors on his/her receptor apparatus is called the sensation. Classification of sensation. There are the general (simple) and complex sensation. The general sensation with taking into account the localization of receptors are classified as the superficial (skin and mucous membranes), pro- prioceptive or deep (muscles, tendons, joints), and interoceptive (internal or- gans) sensation. The pain, temperature (heat and cold) and touch belong to the superticial sensation. The proprioceptive sensation includes a sense of passive and ac- tive movements (proprioception), vibration, a sense of pressure and mass, a kinesthetic sense — determination of the skin creases direction. /nterceptive sensation is called a sensation that occurs when internal organs and walls of blood vessels are being irritated. The impulses accepted from internal organs are not sensed in practice under normal conditions. When the interoreceptors are irritated, pain of varying intensity and with a sense of discomfort may occur. Complex kinds: sense of pinprick localization that identifies the place of in- duced irritation; stereognosis is the ability to recognize objects by their palpa- tion (touching); two-dimensional space sense is specified when a patient with the closed eyes can recognize what figure is written on the skin; discrimina- tions are the ability to perceive separately two irritations being simultaneously dealt at close range. Complex kinds of sensation do not have individual analyzers; they are pro- vided with common kinds of sensation. Specific sensation is also distinguished; it includes vision, hearing, smell, taste, touch All the nerve elements that provide perception, information holding and processing belong to the sensory systems (from the Latin word ‘sensus’ — sense) or to the system of analyzers according to |. P. Paviov. They perceive and process irritators of different modalities. Analyzer is a functional system, which consists of receptors, afferent path- ways and the applicable zone of the cortex of the cerebrum. An analyzer pro- vides perception, passage and processing of the homotypic nerve impulses. 1. Sensitive function and its abnormality | 11An analyzer's cortex end are the primary projection zones of the cerebral cor- tex for which somatotopic organization is definitive. Pathways of sensitivity When an irritator is active, a certain sense occurs in the cerebrum as soon as a nerve impulse accepted from a receptor on the relevant navigation comes to sensitive areas of the cerebral cortex. These paths are not identical to super- ficial and deep sensation kinds, but they are surely three-neuronal. Pain and temperature pathway. The first neuron of pathways is featured by the nerve cells of sensitive spinal ganglion. Peripheral processes (dendrites) of these cells within a spinal nerve, plexus, and peripheral nerve are going to relevant parts of the skin (dermatomes), where they end with receptors per- ceiving irritations. Central processes (axons) of spinal ganglion’s cells are go- ing to the spinal cord and participate in the formation of the dorsal (sensitive) root which enters the spinal cord. The fibers end with the cells of posterior horns which are bodies of the second neurons of the pathways of superficial sensation (Fig. 1.1) The axons of the second neuron in each segment of the spinal cord make an intersection through the front white commissure and fall into the lateral spinal funiculus from the opposite side by forming within it the tractus spinothalam- icus that rises up. An intersection in the front white commissure is not made ina horizontal plane, but under a certain angle, thus, the fibers enter the lateral funiculus at 1-2 segments upper, that is important to determine the level of spinal affection. The tractus spinothalamicus fibers conducting thermal and painful irritation of the lower limbs are laterally placed in the lateral funiculus, and the one of the upper limbs are medially placed, i.e. closer to the syringo- cele (canalis centralis medullae spinalis) (Auerbach’s and Flatau’s law). It is of practical importance for the diagnosis of spinal tumors. In the case of extra medullar localization of tumor, pain and temperature disorders are general- izing bottom-upwards from the opposite side (the bottom-up type of sensory disorders), and in the case of intramedullar tumors, sensitive disorders are effusing on the opposite side upwards-bottom (the top-down type of sensation disorders development). The tractus spinothalamicus goes from the spinal cord into the brainstem, where along with the path of the deep sensation types creates a medial lem- 12. | PART 1: Gensral NeurologyFig, 1.1. Pathways of sensitivity: 1 —postcentral gyrus; 2— internal capsule; 3— thalamus; 4— medial lemniscus; 5 — medulla oblongata; 6 — cervical intumescence, lateral funiculus; 7 — lumbar intumescence, lateral funiculus; 8 — posterior horns; 9 — spinal ganglion — 1" neuron of superficial sensation; 10 — spinal ganglion — 1* neuron of deep sensation; 11 — Goll’s and Burdach’s nucleuses. niscus. Having passed through the brainstem the axons of the second neurons end in the lateral ventral intermediate nuclei of the thalamus. The fibers of the third neuron originate from the cells of these nuclei, which form the tractus thalamo-corticalis. It passes through the back third of the posterior limb of internal capsule behind the corticospinal tract, then as a part of the corona radiata it reaches the cortex of the postcentral gyrus. There is a somatotopic arrangement of agents relating the relevant body parts: the sensation of the lower limbs is in the upper cortex part of this gyrus, the sensation of the trunk and upper limb — in the middle one, the sensation of the face — in the bot- tom. An especially large area of the postcentral gyrus is reserved for innerva- tion of distal parts of the upper limbs and face (Fig. 1.2). 4. Sensitive function and its abnormality | 13= Fig. 1.2, Somatotopic organization ane of the postcentral gyrus: 1 — medial lemniscus; 2— thalamus; 3 — corona radiata, Fibers conducting a touch sensation, for the most part, go along with agents of a deep sensation. Pathways of deep and most of the touch sensation fibers. The first neuron of pathways is also presented by nerve cells of a spinal ganglion. Peripheral processes of these cells form sensitive nerve fibers and end with receptors in muscles, joints; the receptors in the skin and deep tissues perceive touch irritations. The axons of spinal ganglion cells within dorsal roots are going into the spinal cord. However, they do not go to the posterior horn, and form on the same side the dorsal funiculus (Fig. 1.1). Being a part of the dorsal funiculus and forming thin and wedge-shaped fascicles (Goll’s fascicles and Burdach’s columns) the fibers by having uninterrupted passage through the spinal cord to the spinal bulb. Fibers of a thin fascicle are long, they conduct proprioceptive impulses from the lower limbs and low part of the trunk, lay more medially, and fibers of a wedge-shaped fascicle are shorter, they conduct proprioceptive sense generated in the pectoral arch and upper limbs and situ- ated more laterally. Axons of the first neurons end on the body of the second neurons in thin (the Goll’s nucleus — nucleus gracilis) and wedge-shaped nuclei (Burdach’s nucleus — the nucleus cuneatus) located in the medulla oblongata. Axons of the second neurons make up the tractus bulbothalamicus, which being within the cord moves to the opposite side by making a full inter- section, and then along with the tractus spinothalamicus takes part in forming 14. | PART 1: General Neurologythe medial lemniscus (lemniscus medialis). The medial lemniscus rises up by passing through the pons varolii, mesencephalon, and ends in the lateral ventral intermediate nucleus of the thalamus. Deep sensation third neuron fibers being along with the superficial sensa- tion a part of the tractus thalamo-corticalis originate from the ventral interme- diate nucleuses of thalamus. They pass through the back third of the posterior limb of the internal capsule, then as a part of the corona radiata crown reach cortex of the postcentral gyrus. As described above, there is a somatotopic distribution of agents’ projection in the postcentral gyrus. Methods of sensitive function study Exploring a sensation, a doctor gets subjective information from a patient about his/her feelings that arise during irritation of the receptor apparatus. Therefore, it is necessary to adhere to certain conditions during the study. The study should be carried out in a quiet atmosphere, in a warm room, with a patient's eyes closed. Irritations should be inscribed on symmetric sections, have the same force and duration, they should be conducted with different intervals, compared the sensation of irritants with “sick” and “healthy” areas. During delimitation of sensitive disorders, one should be aware of some guidelines: the clavicle’s level is approximately equal to the C3 segment; the level of the nipples is equal to the T5; the level of the rib arch — to the T7; the level of the navel — to the T10; the inguinal fold level — to the T12 segment. Study of superficial sensation. Needle is used in order to test pain sensa- tion. A temperature sense is studied with using test tubes filled with hot and cold water. A Touch sense is investigated with using a piece of cotton or paper, with which the skin is abruptly touched. Study of deep sensitivity. The proprioception and vibration are usually stud- ied in clinical practice. The proprioception is tested by performing the passive movements of small amplitude on small joints of the hands, and then on the patient's legs, who is lying with the closed eyes. If any disorders of sensation are found out in small joints (in the distal phalanxes of fingers), it is required to move to larger. A vibration sense should be checked with a tuning fork, which foots put on the bony prominences of the limb and define the time dur- ing which the patient feels vibration. Normal duration of a vibration sensation is 14-16 sec. 1. Sensitive function and its abnormality | 15Study of complex sensitivity. A sense of /ocalization is checked by applying touch irritants to a patient who should determine the place of touching. Stereo- gnosis or a three-dimensional space sense is the ability to recognize the familiar objects by palpating them with closed eyes. The disorder of stereognosis with full conservation of common sensation types is called astereognosis. A two- dimensional space sense is studied by drawing different numbers, letters and shapes on the patient's skin with a blunt object, which he or she must determine. Kinds of sensitive disorders Quantitative and qualitative kinds of sensitive disorders are distinguished in clinic. Quantitative kinds include: anesthesia, hypesthesia and hyperesthesia. Anesthesia is total loss of this or another sensitive kind. There are anesthe- sia of pain (ana/gesia), temperature (termanesthesia), proprioceptive sense (bathyanesthesia). When all sensation kinds disappear, total anesthesia is de- termined. Hypesthesia means reducing of sensation. Hyperesthesia means increasing perception of sensation. Dissociation or split of sensation is called an isolated losing of one sensa- tion kind while other kinds of it in the same area are kept. Dissociation occurs when the dorsal horns, tr. spinothalamicus and front white commissures of the spinal cord are affected. Quality disorders appear as hyperpathia, dysaesthesia, polyesthesia, and synesthesia. Hyperpathia is the raising of the excitability threshold. Mild ir- ritations are not perceived, and strong are felt inadequately, painfully and non-locally. Hyperpathia is strongly marked, when the thalamus is impaired; median and tibial nerves’ trunks are traumatically and partially damaged (in the case of causalgia). Dysaesthesia is the abnormality of irritation perception when, for example, thermal irritation is felt as painful or touching provokes a feeling of pain. Poly- esthesia occurs when single irritations are perceived as multiple. Synesthesia is a sense of irritation not only in the place where the irritator acts, but also in any other area, Sensation disorders may also arise independently, without external irri- tants. First of all, it is paraesthesia and pain. Paresthesia is called a sense of numbness, formication, burning, cold or pricking sensations that occur without outside influences. 16. | PART 1: General NeurologyPain. Perception of pain can be caused by lesions of afferent system dif- ferent levels. A particularly intense pain occurs when peripheral nerves, dorsal roots of the spinal nerve, ganglion, sensitive roots of the cerebral nerve and the thalamus are damaged. Causalgia (burning pain) occurs, when median and tibial nerves’ trunks are traumatically and partially damaged as a result of irritation of sympathetic fi- bers of the autonomous nervous system being a part of these nerves. Types of sensitive disorders Peripheral, segmental and conductive types of sensitive disorder (Fig. 1.3) are distinguished. The peripheral type of sensation disorder occurs when peripheral nerves are damaged and is divided into neural and polyneuritic. Fig. 1.3. Types of sensitive disorder: 1 — conductive — hemianesthesia; 2 — conductive — alternative hemianesthesia; 3 — conductive; 4 — segmental; 5— peripheral y pai ar BMLMiA HaBYanbHHA aaKn i i f aaKnan «KMSBCEK HA MET ye Hi YHIBEPCHTET yar Mee Waerrndiacuidinns o s a involved in the pathological process. The segmental dissociated anesthe- sia of pain and temperature on the face arises on the side of the focus, and conductive hemianesthesia of superficial sensitivity kinds occurs on the opposite side. Syndrome of medial lemniscus disorder within the cerebral pons and pe- duncle is characterized by a loss of all kinds of sensitivity on the opposite side by the conductive type, i.e. the syndrome of the “two hemi” occurs: hemianesthesia and sensitive hemiataxia . Syndrome of thalamus disorder appears on the opposite side as hemi- anesthesia of all sensitivity kinds and sensitive ataxia due to propriocep- tive sensory disorder, and hemianopsia — falling out of the opposite halves of the vision field — takes place. Thus, the syndrome of the “three hemi" occurs: hemianesthesia, hemiataxia and hemianopsia. Moreover, when the thalamus is affected, thalamic pain (hemialgia) in the opposite half of the body is typical: burning, unbearable pain rises as a result of affecting any irritants (emotions, acute sound, or simple touch). |. Syndrome of internal capsule disorder due to posterior limb damaging where sensitive fibers pass from the thalamus to the cortex. There het- erolaterally is a syndrome of the “three hemi”: hemianesthesia, hemi- ataxia and hemianopsia. If the pyramid tract is involved in the process, another syndrome of the “three hemi” appears on the opposite side: hemiplegia, hemianesthesia and hemianopsia. Cerebral syndrome occurs when the postcentral gyrus is affected. There are its function loss syndrome and the syndrome of gyrus irritation. Due to the large extent of the cortical sensitivity centers its disorders are only limited to one part of the body (the upper, lower limb or the face), i.e. conductive anesthesia or hypesthesia appear by monotype on the op- posite side. These are the cortical symptoms of falling out of sensitive function. Irritation of a postcentral gyrus by the pathological process provokes paresthesia on the opposite side in the form of seizures in the relevant localization of the pathological focus on body's areas (the sensor type of Jackson epilepsy). Paresthesia can be extended to the total half of the body and ends up with common epileptic seizure. 1. Sensibve function andéits abnormality | 21Tests and typical tasks 22 Where is the body of the first sensory neuron of all types of sensitivity localized? a) spinal ganglion ») Burdach’s and Goll’s nuclei ) spinal cord lateral horn d) spinal cord posterior horn ) thalamus 2. Where is the end of the first sensitivity neuron of deep sensitivity localized? a) postcentral gyrus b) Burdach’s and Goll’s nuclei ¢) thalamus d) spinal cord posterior horn ) spinal cord lateral horn 3. In what part of the brain cortex the projection of a hand sensitive innervation is presented? a) postcentral gyrus upper part b) precentral gyrus upper part c) precentral gyrus middle part d) postcentral gyrus middle part e) postcentral gyrus lower part 4. What kinds of sensitivity will be impaired if peripheral nerves are damaged multiply? a) only pain and temperature sense b) only touch sense ©) allkinds d) only kinesthesia e) only vibration sense 5, What structures damage can cause sensitive disorders by the segmental type? a) spinal cord posterior horn, spinal ganglion b) peripheral nerve, anterior root PART 1: General Neurology©) postcentral gyrus, internal capsule d) thalamus, medial loop e) spinal cord lateral and posterior funicles 6, Patient feels vibration for 3 seconds in all joints of the lower extremities, muscle- joint sensation is impaired. There are no palsies or other sensitivity disturbances. What structures are damaged? 7. Sensation of pain and temperature sense are absent on the right upper limb and ‘on the right side of the patient's trunk, the so called "semi-jacket’, but sense of touch is preserved in this region. Kinesthesia and vibration sense are also present. How is such a type of sensitive disorder called? 8. A patient complains of the inability to find necessary things in his pocket with the help of his right upper limb. Superficial and deep sensitivity are present. What is the kind of sensitive disorder in this case? 9. A patient complains of pain, paresthesia, hypesthesia in the left elbow and in the 4 and 5 fingers of the hand, which appeared after trauma of the left elbow joint. What type of sensitive disorders Is there? What is damaged? 10. A patient has lesion of the spinal cord cross-section. What sensitive disorders will this patient have? Tests and typical tasks | 232. REFLEX-MOTOR FUNCTION OF THE NERVOUS SYSTEM. SYNDROMES OF MOVEMENT DISORDERS Reflex actions are the simplest form of movement. A reflex action is a ste- reotyped response to a specific sensory stimulus. The reflex elicited depends on the site of the stimulus and the strength of the stimulus determines the amplitude of the response. Reflex responses are used by higher motor centers to generate more complex movements and behaviors. The neural circuitry re- sponsible for reflex actions is present at different levels of the motor system and disturbances in these reflexes are important for localizing lesions in the motor system. All reflexes are divided into unconditioned and conditioned ones. Uncondi- tioned (instinctive, inborn) reflexes are an inborn motor reaction, phylogenetic old, under cortex regulative influence and are the basis of conditioned reflexes. Inborn reflexes are closed in the spinal cord, brainstem and basal ganglia. Conditioned reflexes are closed in the brain cortex and lay the foundation to higher nervous functions. Unconditioned reflexes are classified into superficial and deep ones Superficial: cutaneous from mucosa ~ abdominal corneal ~ plantar conjunctival - cremasteric - pharyngeal ~ anal - palatal Deep: tendon periosteal flexor ulnar ~ supraorbital ~ extensor ulnar ~ mandibular (jaw) ~ knee - brachioradial ~ Achilles (ankle) ~ scapulohumoral 24 | PART 4: General NeurologyThe monosynaptic stretch reflex is the simplest spinal reflex. As the name implies, the reflex muscle contraction is elicited by lengthening or stretch- ing the muscle. To evoke this reflex, the muscle must be stretched rapidly, which produces a short phasic contraction. Thus, this reflex is termed a phasic stretch reflex. In the human subject, sudden stretch is produced by tapping a tendon with a reflex hammer. Another cutaneous reflex of clinical significance is the superficial abdomi- nal reflex. This reflex is elicited by stroking the skin of the abdomen, which causes a reflex contraction of the abdominal muscles beneath the stimulus. Thus, stroking the upper abdomen causes contraction of the upper abdominal muscles, whereas stimulation of the lower abdomen causes contraction of the lower abdominal muscles. Reflexes arc Afferent nerve fibers conduct the impulses generated by activated receptors to neurons in the central nervous system, which fire impulses that are then transmitted through efferent nerve fibers to the cells, muscles, or organs that carry out the reflex response. The pathway as a whole is known as the reflex arch. Reflex arch functions are: reflex carrying-out, muscle trophism supplying, muscle tone supporting. The scheme of reflex arch are sketched out on Fig. 2.1. Fig. 2.1. The reflex arch of the knee-jerk reflex: 1 — sensory fibers of n. femoralls; 2— ganglion; 3 — posterior root; 4 — posterior horn; 5 — intermedium neuron; 6 — anterior horn; 7 — anterior root; 8 — motor fibers of n. femoralis 2. Reflex-motor function of (he nervous system. Syndromes of movement disorders | 25The spinal cord is divided into segments, each giving rise to dorsal and ventral roots, which are combined to form the spinal nerve. The axons of all the motor neurons located in one spinal segment leave the spinal cord through one ventral root and continue into the spinal nerves. These spinal nerves con- form to the embryological myotomes. This myotomal organization can still be seen in the rostral-caudal distribution of innervation from the cervical and lum- bar cord segments innervating the upper and lower extremities respectively. The levels of the spinal reflex arches locking » Biceps — flexor ulnar (C5-C6 segments), » Brachioradialis — carporadial (C5-C8 segments), » Triceps — extensor ulnar (C7-C8 segments), » Abdominal (T7-T12 segments), » Cremasteric (L1-L2 segments), » Quadriceps — knee (-jerk) (L2/3-L4 segments), » Plantar (L5-S1 segments), » Achilles (S1-S2 segments), » Anal wink (S3-S5 segments). Methods of the inborn reflexes study To master the methods of the inborn reflexes study fulfill the reflexes study in the following sequence. The hammer strikes are performed with equal force. Pay attention if the below mentioned normal reaction is achieved: » corneal reflex — a carefully touch of the cornea above the iris (not above the pupil) with a soft paper stripe leads to lids closing » pharyngeal reflex — a touch of the posterior pharyngeal wall with a spat- ula leads to swallowing or coughing movements occur, » palatal reflex— touch the soft palate with a spatula leads to the soft pal- ate elevation, + mandibular (jaw) reflex — strike your index finger put on the patient's mandible with a hammer (mouth is half-open) leads to the mandible el- evation, » flexor ulnar (Biceps) reflex — half-bended arms are placed on the pa- tient’s abdomen, Press the arm biceps muscle tendon with your left pol- 26 | PART 1 General Neurologylex. Strike your pollex nail with a hammer — as a result forearm flexion will appear, » extensor ulnar (Triceps) reflex — a patient's arm is bent under an obtuse angle. Strike the arm triceps muscle tendon (2 cm above the ulnar process) with a hammer — as a result forearm extension will appear, » brachioradial (carporadial) reflex — patient's arms are bent in the ulnar joint under an obtuse angle, they are half-proned and placed on the abdo- men. Strike the radius styloid process with a hammer and arm flexion in the ulnar joint, fingers pronation and flexion will occur, » abdominal reflexes — make a quick hatched irritation of the abdominal skin with a pointed object from the peripheral to the middle lower the costal arches (superior), on the umbilical level (middle), above the fal- lopian ligament and abdominal wall muscles contraction will occur, » knee (Quadriceps) reflex — a patient’s legs are half-bent in his knee joints. Place your left arm under the patient’s joints. Strike the thigh quadrate muscle tendon under the kneecap with a hammer — as a result legs extension in the knee joint will appear, » Achilles reflex —a patient's leg is bent in the hip and knee joints. Strike the Achilles tendon with a hammer and foot plantar extension will occur, » plantar reflex — perform a hatched skin irritation of the sole external edge with a blunt object and toes plantar flexion will occur. An interruption of the reflex arches at any point weakens or abolishes the reflex. Reflexes changes: » areflexia — absence of reflex, » hyporeflexia — decrease of reflex, » hyperreflexia — increase of reflex, » anisoreflexia — different expression of symmetric reflexes. Pathological reflexes Some reflexes, especially spinal and brainstem reflexes, are normally Observed or elicited only in the developing nervous system. As the ner- vous system and higher motor centers get mature, these reflexes are sup- pressed, only to reemerge if damage of the higher motor centers modulates 2. Reflex-motor function of the nervous system. Syndromes of movement disorders | 27the reflex. Reflexes that can be elicited only in the diseased state are called pathological reflexes. \t indicates dysfunction of the pyramidal (corticospi- nal) tract. Pathological reflexes on feet extensor flexor = Oppenheim ~ Zhukovski’s ~ Gordon ~ Rossolimo ~ Babinski's ~ Bechterew ~ Shaffer ~ Chaddock Oral automatism reflexes: » nasal-lip (nasolabial), » lip, » palmar-chin (palmomental), » distant-oral. Pathologie synkinesises: » global, » imitative, » coordinative. Defense (protective) reflexes (withdrawal leg). Methods of pathological reflexes study Exploring the extensor group foot pathological reflexes: » Babinski reflex — make a hatched skin irritation of the plantar external edge, slow hallux (great toe) extension with a flaccid separation of other toes will occur, » Oppenheim reflex — same response to a downward stroke of the exam- iner’s thumb on the patient's shin, » Gordon reflex— the same response to squeezing of the calf muscles, » Sheffer’s reflex — press the Achilles tendon, slow hallux extension with a flaccid separation of other toes will occur. 28 | PART 4, General NeurologyExploring the flexor group foot pathological reflexes: » Rossolimo reflex — strike easily with your fingers on the plantar surface of the terminal phalanges of the patient's II-IV toes, quick plantar flexion of the toes will occur, » Bechterew reflex — strike with a hammer on the dorsal foot above III-IV metatarsal bones, quick plantar flexion of toes will occur, » Zhukovski reflex — strike with a hammer on the sole under the toes, quick plantar flexion of the toes will occur. Exploring the oral automatism: » nasal-lip reflex — strike easily with a hammer on the nose root, the lips are stretched ahead, » lip reflex — strike easily with a hammer on the lips, the lips are stretched ahead, » palmar-chin reflex — make a hatched irritation of the palmar skin over the thenar, chin muscles at the same side are contracted. Voluntary motor functions are realized through the pyramidal system — a complex of cells which connect primarily to the cortical motor areas cortex and segmental apparatus of the spinal cord and brainstem. The functions of this system are: » movements ensuring; » segmental apparatus regulation (activation of o-motoneurones and breaking the activity of reflex arches), » breaking reflex automatism of subcortical, brainstem and spinal levels. The cortex motor centers are located anterior to the central sulcus, in the frontal lobe and include precentral gyrus, paracentral lobule, premotor area and prefrontal area (the frontal pole). The last one supports the integration of all informational signals and forms a motor act model. The premotor area establishes connection with the extrapyramidal system and cerebellum. The precentral gyrus (Brodmann area 4) is the primary motor area. Ihe corticospi- nal tract is composed of axons of pyramidal neurons located in layer 5 of the cerebral cortex mostly in the precentral gyrus. Layer 5 in the primary motor cortex contains distinctive giant pyramidal neurons known as Betz cells — the upper motor neuron (the central neuron). The axons of these cells become 2. Rellex-motor function of the nervous system, Syndromes of movement disorders | 20the corticonuclear or pyramidal tract and represent one of several descending influences on the motor neurons of the brainstem and spinal cord. The mo- tor neurons in the primary motor area have a somatotopic organization. The homunculus was mapped by Penfield and colleagues (Fig.2.2). Fig, 2.2. A somatotopic organization of the motor cortex The axons of the corticospinal tract gather together, forming the corona fadiata, and descend through the posterior limb of the internal capsule into the cerebral peduncles of the midbrain (Fig. 2.3). In the brainstem corticonuclear tract comes to the end. In the pons the corticospinal fibers form bundles inter- spersed with a variety of other descending and crossing white matter tracts. When the fibers enter the medulla, they form a very discrete, easily recogniz- able bundle on the ventral surface of the medulla known as the pyramids, therefore the corticospinal tract is often referred to as the pyramidal tract. At the bottom of the medulla, most of the corticospinal fibers cross the midline in the decussation of pyramids and continue in the lateral funiculus of the spinal cord as the lateral corticospinal tract. Most of the minority of fibers that do not cross in the pyramidal decussation continue in the ipsilateral ante- 30 | PART 4: General NourlogyFig. 2.3. Pyramidal system 1 — precentral gyrus; 2 — internal capsule; 3 — cranial nerves nucleuses; 4 — cervical intumescence; 5 — anterior horns; 6 — lumbar intumescence; 7 — motoneurons of anterior horns rior corticospinal tract. Some of them cross the midline in the anterior spinal commissure only once they reach the level of their target motor neurons. The lateral and ventral corticospinal axons terminate in motor neurons of anterior horn, where the /ower motor neuron (peripheral neuron) is presented. The lateral corticospinal tract is involved in the movement of the contralateral ex- tremities, whereas the anterior corticospinal one innervates the paravertebral muscles involved in the posture: the neck, trunk and perineum on two (both) sides. Corticonuclear fibers destined for the motor nuclei of the cranial nerves leave the corticospinal tract in the brainstem. Muscles of the head, except for the lower facial muscles (VII cranial nerve) and tongue (XII cranial nerve), re- ceive both crossed and uncrossed corticonuclear fibers. Therefore, as a rule, 2, Retlexmotor function ofthe nervous system. Syrdomes of movement disorders | 31in a patient with a lesion of the corticonuclear tract on one side, one seldom sees significant weakness of the jaw, pharynx, or larynx. The motor nuclei of cranial nerves VII (lower portion) and XI! receive contralateral cortical innerva- tion only. Methods of motor function study A doctor carries out an examination in such a sequence. At first it is neces- sary to exam the volume of active movements in the limbs, including Barre’s test (upper and lower), the “Budda” posture, the examination should be start- ed with big joints to small ones. The muscle strength of the limbs is estimated by five point score: 5 — normal, 0 — absence of the muscle strength. When examination of passive movements in limbs is performed the doctor estimates the muscular tonus of the limbs, defines the presence of atrophy and fibrillar twitches. Methods of the physiological and pathological reflexes exploration were de- scribed above. Remember that pathological reflexes of the extension group are physiological for children under one. After examination the character of paralyses on the basis of the revealed pathological symptoms the topical di- agnosis established. Impairment of motor function The interruption of the pyramidal tract causes paralysis of the correspond- ing muscles. Paralysis (palsy, plegia) means the absence of movement. The partial interruption of the pyramidal tract causes paresis — limitation of move- ment, incomplete paralysis. Paralysis of one limb is called monoplegia, two limbs’ paralysis on one side — hemiplegia, two symmetric limbs’ paralysis — upper or lower paraplegia, four limbs’ paralysis — tetraplegia. Depending on damaged motoneurons there are classified central or pe- ripheral paralysis. Central palsy occurs when the upper motor neuron (cen- tral neuron) is affected. Peripheral — if the lower motor neuron (peripheral neuron) is damaged. The upper motor neuron syndrome is marked by weak- ness, an increased muscle tone — spasticity, hyperactivity of the tendon re- flexes — hyperreflexia, and the presence of the pathological reflex (Babinski sign). This pattern of motor disturbance occurs when there is an interruption 32 | PART 1: General Neurologyof descending projections from the motor neurons in the cerebral cortex and brainstem that modulate excitation of the alpha and gamma motor neurons. “The higher nervous system arrangements inhibits (or controls) the lower, and thus, when the higher are suddenly rendered functionless, the lower rise in activity” J. H. Jackson. ‘With hyperreflexia of the periosteal and tendon reflexes there will be en- largement of reflex zones, the knee and foot clonuses appear, abdominal and plantar reflexes are diminished or absent. The difference in tone in the arm and the leg extensors can also give rise to spastic seizure, or the clasped knife phenomenon, the process that has been compared to opening the blade of a clasped knife. The upper motor neuron syndrome may lead to gait changes: paraspastic gait, leg circumduction, spastic-ataxic gait, Wernicke — Mann gait. Diseases of the lower motor neuron may affect the cell body in the anterior spinal horn itself or its axon in the spinal root and peripheral nerve. Signs of disease of the lower motor neuron — the peripheral palsy features — include muscular weakness, diminition of muscle tone (flaccidity), atrophy, fascicula- tions, and loss of tendon reflexes. Fasciculations or fibrillar muscular twitching are a feature of disorders of the anterior horn cell or root compression; therefore fasciculations are often viewed as an ominous sign. Forcible contraction or percussion of the muscle may increase the frequency of fasciculations. In thin elderly men with sus- pected amyotrophic lateral sclerosis, the shoulder girdle and pectoral muscles are often a good place to look for fasciculations or fibrillation. In patients with more subcutaneous adipose tissue, the first dorsal interosseous muscle of the hand is better. The lower motor neuron syndrome may lead to gait changes such as step- page gait (foot-drop gait). Syndromes of motor tract damage in different levels Motor tract cerebral lesions Precentral gyrus lesion: » function loss syndrome — central monoparesis on the opposite lesion’s side, » irritation syndrome — motor jacksonian epilepsy. 2. Rellex-motor function of the nervous system. Syndromes of movement disorders | 33Internal capsule lesion gives central hemiparesis with central mimic mus- cles and tongue paresis, hemianesthesia of all sensitivity kinds, hemianopsia on the opposite lesion’s side. Very often Wernicke — Mann's position will occur: the arm is flexed at the elbow and wrist and adducted against the chest while the leg is stiffly extended and the foot is inverted and flexed in a plantar direction. Spasticity in the adductors of the leg leads to the tendency of the hemiplegic leg to “scissor” over the healthy leg. Brainstem lesion leads to crossed paralysis (alternated): ipsilateral symp- toms of cranial nerve nucleus lesion and central hemiparesis of contralateral extremities. Spinal cord lesions Over cervical intumescence (C1-C4) lesion leads to central tetraparesis, conductive impairment of all sensitivity kinds and pelvic disorders. Cervical intumescence (C5-Th1) lesion gives peripheral paraparalysis in arms, central paraparalysis in legs with conductive impairments of all sensitiv- ity kinds and pelvic disorders. Thoracic part of the spinal cord (Th3-Th12) lesion leads to inferior central paraparalysis with conductive impairments of all sensitivity kinds and pelvic disorders. Lumbar intumescence (L1-S2) lesion \eads to inferior peripheral parapa- resis with conductive impairments of all sensitivity kinds and pelvic disor- ders. Half of the spinal cord diameter lesion gives Brown-Sequard syndrome: movement impairments and deep sensitivity disorders on the lesion side, con- ductive impairments of superficial sensitivity on the opposite side. Spinal cord anterior corn lesion \eads to segmental peripheral paresises with muscles fibrillation without sensitive disorders. Medullary cone (S3-S5) lesion does not give paresises, sensitivity is im- paired in the perineum, true enuresis will occur. Peripheral nervous system lesions Anterior radix lesion leads to segmental peripheral paresises without sensi- tive impairments, fascicular twitches can be observed. 34. | PART 1: General NeurologyPeripheral nerve Jesion leads to peripheral paresises with sensitive disor- ders in the nerve innervation area. Tests and typical tasks 1. Where are unconditioned reflexes locked? a) in the limbic system b) in the cerebellum 6) In the segmental part of the spinal cord d) in the cerebral cortex e) In the thalamus 2 Indicate the deep periosteal reflex: a) knee b) corneal ©) plantar d) biceps e) supraorbital 3. Indicate the pathological foot reflexes of the extensor group a) Zhukovski’s reflex ©) palmar-chin reflex ©) Oppenheim reflex d) Bechterew reflex 2) Rossolimo’s reflex 4, Point to the signs of the central palsies: a) areflexia, muscle atonia b) muscle hypertonus, pathological reflexes ©) muscle atrophy, deep reflexes hyperreflexia 4d) deep reflexes areflexia, muscle atrophy ©) hyperesthesia, hyperpathia 5. Point to signs of the peripheral palsies a) pathological reflexes ») areflexia, muscle atonia 2, Retex-motor funovon ofthe nervous system. Syndromes of movement disorders | 36©) deep reflexes hyperreflexia d) muscle hypertonus, oral automatism e) hypoesthesia, anesthesia 6. A patient after bis arm trauma doesn’t have right extensor ulnar reflex. What nerve is damaged? 7. A patient with hyporeflexion has weakness of the right hand and low muscular tonus. What pathology has the patient got? 8. A patient who has had stroke doesn't have active movements in the left limbs, The muscular tonus and reflexes are increased, What is this dysfunction? 9. A patient has peripheral paresis of the arms and central paresis of the legs. Indi- cate the location of the process. 10. A patient suffers from attacks of clonic convulsions in the right foot, which last during a minute, How is the attack mentioned called? What is affected? 36 | PART 1: General Neurology3. THE EXTRAPYRAMIDAL SYSTEM AND SYNDROMES OF ITS LESION The movements are formed as a result of consequent functioning of sepa- rate neurons of the pyramidal tract and of a great complex of nervous struc- tures outside the pyramidal system that are united into the extrapyramidal system. This functioning coordinates the strength and duration of movements. When an action is performed voluntarily, a person doesn't keep in his con- scious memory the work scheme of the consequence of motor acts. A change of some muscular contractions with others is automatic. These motor automa- tions are provided by the activity of the extrapyramidal system (EPS). The main functions of the extrapyramidal system are: regulation of the muscular tonus, preparing muscles for action, creation preliminary conditions for performing motor acts, maintaining the posture, providing motor expres- sions of emotions, creation an individual expression of movements, responsi- bility of automatic, stereotypic and reflector protecting movements. The main structural anatomical levels are: 1. Cortical — the premotoric gyrus of the cortex (field 5.6 on Brodman); 2. Subcortical » the nucleus caudatus, » the nucleus lenticularis ( putamen, globus pallidus) 3. Brainstem » the substatia nigra, red nucleus, Dareshevich's nuclei, inferior olivas, vestibular nuclei, formatia reticularis, Lues’ body (subthalamic nucle- us), lower olives. 4. The spinal level descending tracts and structures of the spinal cord: » the tractus reticulo-spinalis, tractus vestibulo-spinalis, tractus rubro- spinalis, tractus tecto-spinalis, tractus olivo-spinalis, The spinal cord: gamma-motor neurons and «-small motor neurons, which are located in the anterior horns of the spinal cord. There are two parts of the extrapyramidal system: » the pallidal system — the globus pallidus, substantia nigra, red nu- cleus, Darkschevich’s nuclei, inferior olivas, vestibular nuclei, formatia reticularis, Lues’ body (subthalamic nucleus), lower olives; » the striatic system — the cerebral cortex, caudate nucleus, putamen. 3. The extrapyramidal system and syndromes ofits lesion | 37The striatic and pallidal systems coordinate functioning and they are united into the striatic-pallidal system. The pallidal structures are responsible for the increase of movements (activating), the striatic structures — for their de- crease inhibition (Fig. 3.1) The biochemistry of neurotransmission is basic to a pathophysiological understanding of extrapyramidal disease. Dopamine is the neurotransmitter used by nigrostriatal neurons, which exerts an inhibitory influence on the cho- linergic interneurons of the striatum. Gamma-aminobutyric acid (GABA) is the Neurotransmitter of the inhibitory striatopallidal pathway. The pars compacta of the substatia nigra contains about 80 % of the total dopamine content of the brain in healthy individuals, but is severely depleted of dopamine in patients with Parkinson's disease. There are two main syndromes of the extrapyramidal system’s lesion: 1. Hypertonic-hypokinetic syndrome or syndrome of parkinsonism; 2. Hypotonic-hyperkinetic syndrome or syndrome of involuntary move- ments (hyperkynesis). In 1817 the English doctor James Parkinson was the first who described the major manifestation of this syndrome and this disease was called Parkinson's disease. In 1920 Tretiakov noticed that the greater is a cell loss in the substan- tia nigra, the lower concentration of dopamine is in the striatum. Now there are two forms of parkinsonism: primary and secondary. Primary parkinsonism (94-96 %) is named Parkinson's disease (idiopatic parkinsonism). Fig. 3.1. The structures of the extrapyramidal system: 41 putamen; 2 — globus pallidus dorsalis; 3 — globus pallidus ventralis; 4 — nucleus caudatus; 5 — substatia nigra; 6 — Lues’ body; 7 — upper hill; 8 — vestibular nucleus; 9— thalamus 38 | PART 1; General NeurologySecondary parkinsonism: postencephalitic, vascular, toxic, post-traumatic, drug-induced, oncologic are seldom. Pathology Aloss of melanin-containing neurons in the substatia nigra with glial prolif- eration is the pathological cause of Parkinson’s disease. Lesion of the substantia nigra and degeneration of the nigrostriatal pathway cause the reduction of dopamine’s synthesis and its amount. As the result the influence of dopamine on the nucleus caudatus is decreased that leads to the increasing of cholinergic activity, counterinhibition of the nucleus caudatus and increase of its inhibitory influence on the motor activity. Clinically it is manifested as hypertonic-hypokinetic syndrome (Parkinson's desease). Hypertonic-hypokinetic (parkinsonism syndrome) It arises due to lesion of the substantia nigra and nigrostriatal tract The typical triad of symptoms of Parkinson’s disease: » muscular rigidity » hypokinesia (akinesia) » tremor The main pathogenetic mechanisms of parkinsonism are: 1. The degeneration of dopamine neurons is in the substantia nigra, 70- 80 % cell loss in it, a low concentration of dopamine in the striatum, the influence of the striatum on pallidum. As a result akinesia occurs. 2. The rigidity is a result of increasing of the tonic reflex on muscles tension. 3. In the ventrolateral nucleus of the thalamus were detected neurons with frequency of firing that corresponds to the rhythm of tremor. Muscular rigidity Hypertonia of the extrapyramidal type manifests itself mainly as parkinso- nian rigidity, as exaggerated, viscous, waxy resistance to passive movement that can be felt by the examiner during the entire movement. Testing of the muscle tone often reveals the so-called cogwheel phenomenon: the examiner, 3. The extraoyramidal system and syndromes ofits lesion | 39