Diseases Causing Chorea
The neuropathological basis of chorea consists of degeneration of small neurons, mainly in the putamen and caudate nucleus. This lesion is particularly evident in hereditary chorea see below . Clinical manifestations. Chorea consists of irregular, sudden, involuntary movements that are usually more pronounced at the distal end of the limbs. In some patients, these movements are of low amplitude and look almost normal, resembling nonpathological fidgetiness in others, they are massive and highly...
Intracranial Pressure and Brain Tumors
Intracranial masses rapidly elevate the intracranial pressure because the skull is a closed compartment. Intracranial hypertension is most commonly caused by tumor, hemorrhage, extensive stroke, trauma and the edema accompanying these conditions , and hydrocephalus. It can also be caused by a variety of other conditions cf. Table 6.7 . Intracranial hypertension impairs cerebral perfusion and CSF circulation and may also result in compression of intracranial structures e. g., compression of...
Spinal Radicular Syndromes
Radicular lesions are usually due to mechanical compression less commonly, they may be infectious inflammatory or traumatic. Their main clinical manifestation is pain, usually accompanied by a sensory deficit in the dermatome of the affected nerve root. Depending on the severity of the lesion, there may also be flaccid weakness and areflexia in the muscle s innervated by the nerve root. Preliminary anatomical remarks. The spinal nerve roots constitute the initial segment of the peripheral...
Hemifacial Spasm
This condition is characterized by synchronous, irregular, rapid, brief contractions of all of the muscles of facial expression supplied by the facial n. on one side of the face, particularly including the platysma. On close observation, hemifacial spasm is readily distinguishable from a facial tic Fig. 11.19 . It rarely arises in the aftermath of a peripheral facial nerve palsy. The usual cause is irritation of the facial nerve root by a looping blood vessel just distal to its point of exit...
Neurological Disturbances of Hearing
The differentiation of sensorineural from conductive hearing loss helps determine whether the underlying cause is located in the middle ear or external auditory canal more common sites, conductive hearing loss or in the sensory cells of the inner ear or the neural apparatus of hearing less common sites, sensorineural hearing loss . The method of examination and typical findings are summarized above on p. 22. The diagnosis and treatment of conductive hearing loss and of disorders of the cochlea...
Blood Supply of the Spinal Cord
The spinal cord receives arterial blood from three vessels the unpaired anterior spinal a., which runs down the anterior median fissure of the cord and supplies the anterior two-thirds of its cross-sectional area, and the paired posterolateral spinal aa. Each of these spinal arteries is made up of a series of individual segments that are linked with one another along the longitudinal axis and receive arterial blood from various sources Fig. 7.9 . At cervical levels, the anterior spinal a....
Examination of the Trunk
The back and spine are examined with the patient standing. Inspection may reveal scoliosis or an alteration of the normal lordosis or kyphosis of particular segments of the vertebral column. Protruding ribs on one side often visible only when the patient bends forward are a sign of torsional scoliosis. As one looks at the patient from behind, there is a triangular gap to either side of the patient's waist, formed by the dependent arm, the rib cage, and the upper border of the pelvis asymmetry...
Episodic Disturbances with Transient Loss of Consciousness and Falling
Table 9.9 provides a quick overview of nonepileptic disturbances of this type and, for completeness, also includes some that are of epileptic origin . Only the more important ones are described in greater detail in this section. Table 9.9 Clinical features of various conditions causing brief loss of consciousness and falls orthostatic hypotension vasomotor collapse chronic sympathetic autonomic Shy-Drager, tabes dorsalis I diuretics, antihypertensives, 1 -blockers L-Dopa possibly due to anemia...
Other Types of Viral Encephalitis
Herpes zoster encephalitis is accompanied by a segmental vesicular rash in the territory of a peripheral nerve cranial nerve . CSF examination reveals lympho-cytic pleocytosis up to 200 cells mm3. The disease may appear in particularly severe form after a generalized herpes zoster infection. Rarer types. Other, rarer viruses causing meningoen-cephalitis, some of which are specific to particular regions, are listed in Table 6.16 in addition to those already discussed. Fig. 6.29 concerns one such...
Internuclear Ophthalmoplegia
This condition is caused by a lesion of the medial longitudinal fasciculus MLF . When the patient attempts to look away from the side of the lesion, the ipsilateral ad-ducting eye cannot fully adduct, and the contralateral abducting eye exhibits end-gaze nystagmus. The inability of the ipsilateral eye to adduct is not due to a lesion of the oculomotor n. nucleus, as is demonstrated by a preserved ability to adduct converge in the near reflex. Internuclear ophthalmoplegia INO can also be...
Info Gzk
Fig. 5.4 The cortical representation of different parts of the body in the primary somatosensory cortex of the postcentral gyrus left and the primary motor cortex of the precentral gyrus right in the human being. After Penfield, W., H. Jasper Epilepsy and the Functional Anatomy of the Human Brain. Little, Brown, Boston 1954. Fig. 5.4 The cortical representation of different parts of the body in the primary somatosensory cortex of the postcentral gyrus left and the primary motor cortex of the...
Info Gsv
Fig. 11.17 Mass innervation of the face after right peripheral facial nerve palsy. Because of faulty redirection of regenerating motor axons to their muscular targets, the active contraction of a muscle group in the face can be accompanied by involuntary contraction of other muscle groups. In the patient shown, whistling is accompanied by involuntary eye closure. From Mumenthaler, M. Didaktischer Atlas der klinischen Neurologie. 2nd edn, Springer, Heidelberg 1986 Fig. 11.17 Mass innervation of...
Hypoglossal Nerve Palsy
The anatomy and technique of examination of the hypoglossal n. are described above on p. 27. Typical deficits. The ipsilateral half of the tongue is paretic and, in the course of time, becomes atrophic. When the tongue is protruded, it deviates to the paretic side. This condition is illustrated in Fig. 3.16, p. 27. Causes. Unilateral hypoglossal nerve palsy is usually due to a bony fracture or a mass lesion rarely, a congenital malformation in the posterior cranial fossa. Carotid dissection is...
Info Kai
unsteady stance and gait spinal ataxia, see below due to the lack of proprioceptive feedback regarding the posture and movements of the head, trunk, and limbs. Anterolateral column system. The centripetal processes of the pseudounipolar spinal ganglion cells first sensory neuron that subserve protopathic sensation pain, temperature sensation, coarse touch and pressure form synapses onto second sensory neurons in the posterior horn of the spinal cord. The axons of these cells cross the midline...
Disorders of Lipid Metabolism
Lipid storage diseases are caused by faulty enzymatic degradation of individual lipid substances, leading to deposition of the intermediate products of lipid metabolism in various internal organs liver, spleen, bone marrow and in the nervous system. Disorders in which these nondegradable metabolites accumulate mainly in the neurons of the brain are characterized by degeneration of the cerebral cortex or of the subcortical nuclear areas lipidoses disorders in which they accumulate mainly in...
Cryptogenic Peripheral Facial Nerve Palsy
Epidemiology. This condition Bell palsy accounts for three-quarters of all cases of facial palsy and has an annual incidence of ca. 25 per 100 000 individuals. Etiology. The cause is unknown, but it is probably a viral infection. Swelling of the facial nerve trunk in the narrow confines of the facial canal leads to local com-pressive ischemia, which, in turn, leads to further swelling a vicious circle . The result is a local interruption of the blood supply to the facial n. through the vasa...
Special Considerations in the Neurological Examination of Infants and Young
Most of the techniques presented above for the neurological examination of adults cannot be used in infants or small children. In this age group, the important clinical indicators of nervous system function are body posture, spontaneous motor behavior, and the reflex motor responses induced by certain specific stimuli. In taking the clinical history, the examiner must inquire about any problems that may have occurred during the child's gestation and delivery. In the physical examination,...
The Main Spinal Cord Syndromes and Their Anatomical Localization
Diseases of the spinal cord, like those of the brain, can be of traumatic, vascular, neoplastic, paraneoplastic, infectious,inflammatory, metabolic, endocrine, toxic, or hereditary degenerative origin. The clinical manifestations of spinal cord lesions depend on their level and extent and are largely independent of etiology. Thus, the first step in the diagnostic evaluation of spinal cord diseases, as of brain diseases, is topographical localization, i.e., the deduction of the level of the...
Lesions of the Facial Nerve
This mainly motor cranial nerve innervates the muscles of facial expression. It also provides taste to the anterior two-thirds of the tongue and innervates the lacrimal and salivatory glands. Lesions of the facial n. usually lie in the peripheral nerve trunk and are clinically evident mainly as facial palsy. Peripheral facial nerve palsy often arises without any apparent cause i. e., it is often crypto-genic . It must always be carefully differentiated from symptomatic weakness of the facial...
Anatomical Fundamentals
The spinal cord is the component of the central nervous system that connects the brain to the peripheral nerves. It contains in the white matter, fiber pathways leading from the brain to the periphery and vice versa in the gray matter, an intrinsic neuronal system consisting of i-1 cervical roots C1-C8 thoracic roots T1-T12 lumbar roots L1-L5 I-4 sacral roots S1-S5 coccygeal root spinous processes C1-C7 spinous processes T1-T12 spinous processes L1-L5 HBE1 spinous processes S1-S5 HBM coccyx...
Cataplexy
Cataplexy, a component of the narcolepsy-cataplexy syndrome, may present with the clinical picture of an unexplained, atonic fall. Directed history taking then reveals some or all of the five cardinal symptoms of cataplexy disturbance of wakefulness, usually with brief and restorative naps during the day in sleep-promoting situations falls, due to sudden loss of muscle tone, precipitated by fright or other emotions affective loss of muscle tone , and perhaps brief local loss of tone in...
Other Demyelinating Diseases of Unknown Pathogenesis
In addition to the genetic, immune-mediated, and metabolic myelinopathies, there are a number of other demyelinating diseases whose causes remain unknown. These diseases are briefly listed and characterized in Table 8.3. Table 8.3 Rarer demyelinating diseases Concentric sclerosis Bal sclerosis, periaxial encephalitis Acute disseminated encephalomyelitis ADEM Subacute myelo-optic neuropathy SMON onset at any age, slow progression, associated with dementia acute multiple sclerosis simultaneous...
Lesions of the Trigeminal Nerve
The trigeminal n. is responsible for the soma-tosensory innervation of the skin of the face and forehead and of many of the mucous membranes of the face and head. It also carries motor fibers innervating the muscles of mastication. Lesions of this nerve thus produce sensory deficits and paralysis of the muscles of mastication. The anatomical course and distribution of the trigemi-nal n. are shown in Fig. 3.10, p. 23 and the technique of clinical examination is presented on p. 22. Clinical...
Info Rhu
oculomotor nerve lesion and ciliary ganglionitis ocular motility disturbed only in oculomotor nerve lesion contraction in response to miotic agent normal ocular motility, tonic dilatation after convergence reaction, normal response to mydriatic agents pupils often misshapen, no response to weak mydriatic agents, enhanced contraction with physostigmine, mild dilatation with atropine early optic nerve lesion afferent pupillary defect normal ocular motility, no contraction in response to miotic...
Fundamentals of Eye Movements
The anatomical substrate of eye movements consists cortical areas in the frontal, occipital, and temporal lobes, in which the impulses for voluntary, conjugate eye movements and ocular pursuit movements are generated a number of important gaze centers in the brainstem particularly the paramedian pontine reticular formation, PPRF, and midbrain nuclei that relay the cortical impulses onward to the motor nuclei innervating the eye muscles in such a way that coordinated movements of the eyes can...
Spinal Muscular Atrophies
These diseases are due to a genetic defect on chromosome 5 that causes isolated degeneration of the second lower motor neurons, i. e., the motor neurons of the anterior horn cells and the cranial nerve nuclei. The result is the typical clinical syndrome of anterior horn degeneration described above flaccid weakness, muscle atrophy, loss of reflexes, fasciculations . The main clinical types of spinal muscular atrophy are classified according to their age of onset and the pattern of motor...
Disorders of Copper Metabolism
A disturbance of copper metabolism causes hepatolen-ticular degeneration Wilson disease , an autosomal recessive disorder whose genetic locus lies on the long arm of chromosome 13. The concentration of the copper transport protein ceruloplasmin is abnormally low and, as a result, the serum free copper concentration is high and an abnormally large amount of copper is eliminated in the urine. Free copper is deposited in the liver, the edge of the cornea producing the typical KayserFleischer ring...
Examination of the Head and Cranial Nerves
The examiner should first note the general appearance of the head and cervical spine e.g., sunken temples in Steinert myotonic dystrophy and the patient's facial expressions e.g., paucity of facial expression in Parkinson disease . Young, healthy persons should be able to turn the neck and head almost 90 in either direction, so that the eye that is farther from the examiner disappears behind the root of the patient's nose. Further, the patient should be able to incline the head laterally 45 in...
Classic Polyradiculitis LandryGuillainBarrStrohl Syndrome
Acute polyradiculitis is characterized by rapidly ascending paresis, accompanied by at most mild sensory disturbances. In severe cases, the cranial nerves and autonomic system can be involved. Weakness usually improves spontaneously in all involved muscles those that became weak first recover last . The prognosis is favorable. Epidemiology. This illness, usually called Guillain-Barre syndrome for short, can appear at any age. Its annual incidence is between 0.5 and 2 cases per 100 000 persons....
SAEAssociated Dementia and Multiinfarct Dementia
Etiology. Vascular dementia, the second most common etiologic category of dementia, is caused either by multiple subcortical lacunar infarcts due to cerebral micro-angiopathy subcortical arteriosclerotic en-cephalopathy, SAE more common type or by multiple cortical and subcortical infarcts due to macroangiopathy or recurrent embolic stroke multi-infarct dementia less common type . The two types often appear together. The sites and extent of the infarcts determine the severity and progression of...
Ballism
The neuropathological substrate of ballism is a lesion of the contralateral subthalamic nucleus corpus Luysii and or its fiber connections to the thalamus. Etiology. Ballism is usually due to a focal ischemic process, less commonly to a space-occupying lesion. It may also be the result of severe neonatal jaundice or of a hereditary degenerative disease it is bilateral in such patients. Clinical manifestations. Rapid, lightning-like, large-amplitude, unbraked flinging movements of the limbs are...
Examination of the Lower Limbs
The procedure here is essentially the same as in the upper limbs cf. Examination of the Upper Limbs, pp. 27 ff. . Particular attention should be paid to the examination of the peripheral pulses, because pathological processes frequently affect the circulation of the lower limbs. The pedal and popliteal pulses should be palpated the pulses in the abdominal vessels should be examined by auscultation, as should those of the femoral a., both in the groin and in the proximal adductor canal. The...
Funicular myelosis is caused by vitamin B12 deficiency
The latter, in turn, may be due either to inadequate dietary intake or to impaired resorption, owing to a lack of sufficient gastric intrinsic factor e. g., in atrophic gastritis, or after gastrectomy . The pathological findings include demyelination of the posterior columns, posterior roots, and pyramidal tracts in later stages of the disease, other tracts of the spinal cord, and the cerebral white matter, can be affected as well. There is often, but by no means always, an accompanying...
Examination of the Upper Limbs
General aspects. The examiner should ask the patient which hand he or she mostly uses, right or left. Only persons who use a pair of scissors, a knife, or a sewing needle with their left hand, or write with the left hand, are true left handers. Any abnormalities of muscle bulk should be noted, in particular isolated atrophy of muscle groups. Fasciculations must be deliberately sought in our experience, these involuntary contractions of groups of muscle fibers, which induce no movement, can be...
Muscle Weakness and Other Motor Disturbances
Anatomical Substrate of Motor Function It is a useful simplification to consider the motor system as consisting of the following components Fig. 5.1 First central motor neuron neurons in the precen-tral gyrus . The axons travel in the corticobulbar and corticospinal tracts through the internal capsule and cerebral peduncle and terminate either in the cranial nerve nuclei of the pons and medulla corticobulbar pathway or on the anterior horn cells of the spinal cord pyramidal pathway . Lesions of...
Microscopic Anatomy of the Nervous System
Neurons are the structural and functional building blocks of the nervous system. This type of cell is specialized for the reception, integration, and transmission of electrical impulses. Neurons. The cell body soma of the neuron is enclosed by the cell membrane and contains the cell nucleus, mitochondria, endoplasmic reticulum, neurotubules, and neurofilaments Fig. 1.1 . Dendrites are short, more or less extensively branched, cellular processes that conduct afferent impulses toward the cell...
Stance and Gait
General remarks. Though stance and gait are listed at the bottom of Table 3.1, we in fact recommend testing these functions as the first step in the examination of the undressed patient. Mere observation of the patient in a standing position can reveal evidence of a disease process, e.g., muscle atrophy, spinal deformities, and winging of the scapula. The patient's posture at rest may be abnormal, e. g., the exaggerated lumbar lordosis of muscular dystrophy cf. Fig. 14.3, p. 266 or the stooped,...
Heinrich Mattle MD
Library of Congress Cataloging-in-Publication Data Mumenthaler, Marco, 1925- Grundkurs Neurologie. English Neurology an illustrated guide Mark Mumenthaler, Heinrich Mattle translated by Ethan Taub. Revised translation of the German edition Grundkurs Neurologie. Illustriertes Basiswissen f r das Studium - T.p. verso. ISBN 1-58890-450-4 GTV alk. paper -ISBN 3-13-136451-3 TNY alk. paper 1. Neurology Outlines, syllabi, etc. I. Mattle, Heinrich. II. Title. DNLM 1. Nervous System Diseases Handbooks....