Australian X disease See Murray Valley encephalitis
B. Headache characteristics as for post–lumbar puncture headache
C. Headache disappears within 14 days after effective treat-ment of fistula
(Reproduced with permission from Classification of headache disorders, cranial neuralgias and facial pains, and diagnostic criteria for primary headache disorders.
Cephalalgia 1988;8[suppl]:1–96).
cerebrospinal fluid hypovolemia See idiopathic in-tracranial hypotension
cerebrospinal fluid rhinorrhea Escape of CSF through the anterior cranial fossa to the nasal cavities.
The causes are divided between those which are trau-matic and those which are not, the latter group in-cluding cases of high- and normal-pressure leaks.4238 174 CEREBROMALACIA
cerebrotendinous xanthomatosis (cholestanolosis;
OMIM 213700) A rare autosomal recessive lipid stor-age disease characterized by the deposition of cholestanol (a cholesterol metabolite) in the body tis-sues (replacing cholesterol in the myelin of the central and peripheral nervous systems) and by raised plasma levels of cholestanol; the result of a defect in the activ-ity of sterol 27-hydroxylase, a mitochondrial enzyme responsible for the synthesis of bile acids.5708The ge-netic abnormality maps to 2q33–ter.
Clinically, the disease is manifest in early life with cataracts, xanthomatous swelling of tendons (particu-larly the Achilles tendons), mild mental slowing, ataxia and eventually cerebellar and pyramidal signs, and a sensorimotor peripheral neuropathy. Seizures, early atheroma, osteoporosis, bone fractures, pes cavus, hy-percholestanolemia, and myopathic facies are other fea-tures. The condition progresses to a state of spastic, dysarthric helplessness and death, usually before the age of 30 years.498,2439The condition was first described by van Bogaert in 1937.
cerebrovascular disease See strokes.
Cerebrovascular Diseases A neurological subspe-cialty journal. Address: S. Karger AG, P.O. Box 10, Allschwilerstrasse, CH-4009 Basel, Switzerland. Web site: http://www.karger.ch/journals/ced/ced_ jh.htm.
cerebrovascular insufficiency The term of Denny-Brown denoting episodes of focal reversible ischemia of the brain resulting from failure of the collateral blood supply, as occur during systemic hypotension in peo-ple with severe but previously subclinical carotid or ver-tebrobasilar disease, leading to a reduction in perfusion pressure in defined brain regions.6119
Cardiac abnormalities or mere postural change may be responsible for the hypotensive episodes. The clini-cal effects include brief, repetitive episodes of transient neurological dysfunction (amaurosis, clumsiness, weakness, paresthesias, etc.), frequently with involun-tary jerking of the affected limbs, accompanied by slow activity on the EEG.2057See also limb shaking.
cerebrovascular reserve 1. The capacity of the brain to withstand a limited period of ischemia without un-dergoing infarction. 2. The capacity of the cerebral cir-culation to protect the brain from the potentially dam-aging effects of ischemia by maintaining the delivery of glucose and oxygen despite reductions in local or global perfusion pressure.
cerebrovascular syphilis A form of tertiary syphilis in which arteritis leads to vascular occlusion, usually in the territory of the middle cerebral artery, after a pro-longed prodrome of nonspecific headaches, malaise, vertigo, insomnia, etc. The CSF cell count, protein, and serology findings are always abnormal.
cerebrum (Lat, the brain) Originally the whole thing, but the cerebellum was differentiated by Erasistratus in
the third century B.C. The word appeared in English in 1565.5204
ceroid An acid-fast, sudanophilic fluorescent sub-stance of unknown chemical composition which is found in human tissues such as liver, muscle, intestine, and nervous system, in association with several disease processes. See lipofuscin.
ceroid lipofuscinosis See neuronal ceroid lipofus-cinosis.
ceroid storage disease A disease resulting from an inborn metabolic error and once known as Landing-Oppenheim disease,3251but now subdivided according to the causative chemical deficits. See neuronal ceroid lipofuscinosis.
ceruleus See caeruleus. Lat, sky-blue.
ceruloplasmin deficiency with hemosiderosis A hereditary syndrome of progressive dementia, ex-trapyramidal disorders, cerebellar ataxia, and diabetes mellitus with low-serum cereloplasmin and raised fer-ritin levels, reported from Japan.3990
cerveau isolé A physiological preparation, with the brain (classically of a cat) being isolated from the re-mainder of the CNS by a cut at the intercollicular level, just beneath the third nerve nucleus.735Interruption of the activity of the ascending reticular activating system leads to permanent sleep, apart from the motor and sensory effects.
cervical (from Lat, the neck). Pertaining to the neck.
cervical acceleration injury See whiplash.
cervical band syndrome A form of thoracic outlet syndrome, supposed to be produced by pressure upon the lower fibers of the brachial plexus by a fibrous tis-sue remnant stretching from the transverse processes of the lowest cervical vertebra to the clavicle. The exis-tence of such bands, which are visible only to surgeons and which cannot be imaged, must be very uncommon.
cervical dystonia (spasmodic torticollis, idiopathic cervical dystonia [ICD]) The most common form of adult-onset focal dystonia characterized by sustained involuntary contractions of the neck muscles, which re-sult in abnormal movements and postures of the head and neck. Tremor may be associated.2810Dystonia in other regions and concomitant head or hand tremor are also present in a minority of cases. When the onset is in adult life the symptoms do not generalize, but seg-mental spread may occur and pain can increase inde-pendent of the dystonia.
Because the movements are not always spasmodic and because torticollis is not the invariable clinical re-sult of the muscle contractions (laterocollis, retrocol-lis, and antecollis also occur), the alternative term is now preferred to that used for over a century. The eti-ology is unknown; painful trauma may be involved in CERVICAL DYSTONIA 175
the pathogenesis of the condition but the underlying physiology is speculative. In inherited adult-onset cer-vical dystonia, linkage to chromosome 18p has been demonstrated.1300
cervical migraine (posterior cervical sympathetic syndrome of Barré, Bartschi-Rochaix syndrome, cervi-cal vertigo syndrome) A syndrome characterized by oc-cipital headache, scotomas, vertigo, tinnitus, vasomo-tor disorders, paresthesiae, facial spasm, and neck stiffness, ascribed by M.J. Barré373to irritation of the sympathetic plexus around the vertebral artery in pa-tients with cervical arthropathy.
Whether this represents migraine or vertebrobasi-lar insufficiency or arises as a result of structural pathology in the cervical spine is not determined.4161 See also cervicogenic headache.
cervical nystagmus (neck torsion nystagmus, verte-brobasilar insufficiency nystagmus) A form of jerk nys-tagmus with or without other vestibular signs, supposed to result from lesions of the proprioceptive mechanisms in the cervical cord, but which is commonly due either to toxicity or to positional changes.
cervical radiculopathy Compression or ischemia of the cervical nerve roots as a result of local degenerative or other disease.
The clinical features have been analyzed and com-prise pain in the neck, shoulders, arm, or forearm;
paresthesias, muscle weakness and atrophy, hypore-flexia, and objective disturbances of sensation in the appropriate dermatomal distributions.6084The follow-ing diagnostic criteria have been suggested:
1. Symptoms
A. Pain in the neck, the arm, or both
B. Paresthesias, dysesthesias, or hypesthesia in nerve root distribution
C. Muscle weakness 2. Signs
A. Sensory changes in dermatomal distribution
B. Weakness, atrophy, or fasciculation in myotomal dis-tribution
C. Unilaterally diminished muscle stretch reflexes 3. Diagnostic procedures
A. EMG evidence of acute denervation in cervical para-spinal muscles or in a myotome
B. Demonstrable abormality on myelography, CT-assisted myelography, or MRI, correlating with the clinical fea-tures
C. Identification of an affected cervical root at operation The diagnosis of definite radiculopathy can be made in the presence of criteria 3C; 3A; 2A, 2B, and 2C; or 3B and 1A and either 1B or 1C. The diagnosis of prob-able radiculopathy can be made in the presence of more than one of criteria 1A, 1B, and 1C with any one of cri-teria 2; or with any one of cricri-teria 1 with 3B; or 1A with any two of criteria 2.
Cervical radiculopathy is deemed not present if only one of criteria 1 is present; in the presence of an abnormal
im-aging test without evidence of a recent radiculopathy; or if the EMG changes suggest chronicity rather than a re-cent cervical root injury (amended from the criteria of Dr. William J. Litchy, with many thanks).
cervical rib A benign anomaly, first described by Naf-fziger4060and sometimes detected in patients who com-plain of neck or arm pain and who are then at risk of operation to remove the rib, which is thought to be causing a thoracic outlet syndrome.
cervical spinal muscular atrophy See hereditary motor neuropathy, type 1.
cervical spondylosis Degenerative changes in the vertebrae and their joints and in the annulus fibrosus of the disks with or without accompanying arthritis of the synovial joints of the cervical spine (cervical os-teoarthritis) leading to any combination of degenera-tion or prolapse of the intervertebral disks, thickening and buckling of the ligamentum flavum, and osteo-phyte formation.4375 The local fibrosis, hypertrophy, and distortion lead sometimes to ischemic or com-pressive effects upon the spinal cord and nerve roots at the affected levels.
The condition was recognized as a cause of paraple-gia by Strümpell in 1888, but only in 1926 was it shown (by Eliott) that spondylotic narrowing of the interver-tebral foramina could lead to radicular symptoms.710 cervical spondylotic myelopathy Ischemic-com-pressive disease of the spinal cord resulting from de-generative arthropathy of the cervical spine, in par-ticular affecting the intervertebral disks and the zy-gapophyseal joints and leading to hypertrophy of the ligamentum flavum. Disc protrusion compounds the clinical deficit.5487Clinically, compression leads to con-duction block in the cord, especially between C3 and C5, producing pyramidal signs and evidence of root compression. Compression of the vertebral arteries augmenting cord ischemia is another complication.
The condition had attracted little attention until the papers of Brain and Wilkinson demonstrated the abil-ity of such incursions upon the spinal canal to com-promise the functions of the cord at this site. The fur-ther studies of Yoss et al.6084confirmed the clinical and radiological picture, which is composed of the triad of reduced mobility of the neck, long tract signs below (of-ten far below) the level of the lesion(s), and local radicu-lopathic features.
Thoracic myelopathy is far less common; local tumors or disk herniation are the more common causes.
See also midcervical central cord syndrome.
cervical sympathetic irritation syndrome See Pourfour du Petit syndrome.
cervical vertigo Subjective hallucinations of rota-tional acceleration thought likely to arise as a result of lesions of the cervical proprioceptive mechanisms. The 176 CERVICAL MIGRAINE
existence of the phenomenon in humans is, however, controversial.726
cervical vertigo syndrome See cervical migraine.
cervicobrachial neuralgia Pain felt in the neck and referred to the regions of the arm supplied by the four lower cervical and the first thoracic roots.
cervicocollic reflex A muscle stretch reflex allowing compensatory adjustments to the position of the head in response to neck and truncal movements.
cervicogenic headache Recurrent, persistent, uni- or bilateral, moderate to severe pulsating head pain in any region, referred from a primary nociceptive source in the musculoskeletal tissues innervated by the cranial nerves, but usually associated with disease of the cervi-cal spine.
Consistent reproduction of the pain by neck move-ment, painful limitation of neck movemove-ment, accentua-tion of pain with sudden head movement, abnormal postures of the head and neck, abnormal mobility of the craniocervical junction, and sensory abnormalities implicating the upper cervical cord or lower medulla are features characteristically associated.1604Dizziness, conjunctival injection, phonophobia, photophobia, nausea, and vomiting are also described, which have led to persistent concerns since Liveing’s contention in 1873 that the differentiation of this condition from mi-graine is inappropriate.1603 Although neck structures play a role in the pathophysiology of some headaches, clinical patterns indicating a neck–headache relation-ship have still not been adequately defined. Further rig-orous studies are needed to confirm the validity of cer-vicogenic headache as an entity.3349
Diagnostic criteria have been suggested5198 (see Chart C–14). However, the validity of the syndrome is not universally accepted. See also posterior cervical sympathetic syndrome, cervical migraine.
Chart C–14. Diagnostic Criteria for Cervicogenic Headache
Following is a variant form of the diagnostic criteria proposed by Sjaastad et al. (1990).5198and reviewed by Antonaci et al.:189 1. Unilateral headache without side shift
2. Symptoms and signs of neck involvement
a. Pain triggered by neck movement and/or sustained awk-ward position and/or external pressure over the ipsilat-eral upper, posterior neck, or occipital region b. Ipsilateral neck, shoulder, and arm pain of a rather
vague, nonradicular nature
c. Reduced range of motion in the cervical spine 3. Pain episodes of varying duration or fluctuating,
continu-ous pain
4. Moderate, non-excruciating pain, usually of a non-throb-bing nature
5. Pain starting in the neck, eventually spreading to oculo-frontotemporal areas, where the maximum pain is often located
6. Anaesthetic blockades of the major occipital nerve and/or
the C2 root or other appropriate blockades on the symp-tomatic side abolish the pain transiently, provided a. Complete anesthesia is obtained or
b. A whiplash (neck trauma) is sustained a relatively short time prior to the onset
7. Various attack-related phenomena include autonomic symptoms and signs, nausea, vomiting (ipsilateral edema), and flushing mostly in the periocular area; dizziness; photo-and phonophobia; photo-and blurred vision on the eye ipsilateral to the pain.
cervicolinguomasticatory syndrome (Kulenkampff-Tarnow syndrome) Probably an earlier description of Meige syndrome. See tardive dyskinesia.
cervico-ocular reflex (neck-eye loop) A postural re-flex elicited by angular motion of the trunk relative to the fixed head or vice versa, holding still the position of the eyes and head to ensure gaze stability during movement of the trunk.771
cervico-oculoacoustic syndrome See Wildervanck syndrome.
cervico-osteofacial syndrome See Wildervanck syndrome.
CES-D Scale See Center for Epidemiologic Studies Depression Scale.
Céstan, Étienne Jacques-Marie-Raymond (1872–
1933) French neurologist and psychiatrist who worked at the Salpétrière for some years as chef de clinique to Raymond, before returning to his native Toulouse, where he practiced as professor of mental and nervous diseases or of clinical medicine.
His publications spanned numerous areas of neu-ropathology and clinical neurology without any one focus.
Céstan syndrome (Céstan-Chenais syndrome) Uni-lateral paralysis of the soft palate and vocal cords with Horner syndrome, ataxia, alternating hemiplegia and hemianesthesia for proprioception, and discriminative touch (medial lemniscus); the spinothalamic fibers, placed laterally, are spared.
The syndrome is due to infarction of the medial part of the medullary tegmentum as a result of vertebral ar-tery occlusion below the origin of the posterior inferior cerebellar artery.950It was first described by Dr. Céstan and by Dr. L.J. Chenais (1872–1950), who was also a French physician.
Céstan-Chenais syndrome See Céstan syndrome.
Céstan-Lejonne syndrome See Emery-Dreifuss syndrome.
cGy The unit of measurement of radiation.
Chaddock, Charles Gilbert (1861–1936) American neurologist and psychiatrist who trained in Michigan and later in Paris under Babin´ski at the Bicêtre. He be-came professor of neurological diseases at the St. Louis CHADDOCK, CHARLES GILBERT 177
University School of Medicine. He is remembered for the sign next following and for having translated Kraft-Ebing’s work Psychopathia Sexualis and some of Babin´ski’s papers into English.1750
Chaddock reflex 1. (external malleolar sign) A vari-ant method of eliciting the Babin´ski reflex (plvari-antar re-flex) by scratching the skin below the lateral malleolus from behind forward, its advantage being the lesser amount of withdrawal excited. There is evidence that this is the only method with greater sensitivity than the original.953The same method had been described by Kisaku Yoshimura, a Japanese physician, in 1906, 5 years before Chaddock’s description. The reversed Chaddock sign5494is the same thing done (obviously) backwards. 2. (wrist sign) Flexion of the wrist and ab-duction of the fingers when the ulnar side of the lower forearm is stimulated; seen in pyramidal tract dis-ease.4723
chaeromania (from Gr, to rejoice madness) Ma-nia or euphoria, a psychotic state characterized by un-wonted cheerfulness.
Chagas disease An arthropod-borne zoonosis due to infection with Trypanosoma cruzi, first described by Chagas in 1907. The clinical features are those of chronic meningitis with evidence of cortical inflamma-tion and demyelinainflamma-tion; late in the course of the dis-ease there is evidence of autonomic neuropathy.5288See South American trypanosomiasis.
chair tests 1. The production of nystagmus in the di-rection opposite rotation when the subjects revolve on a chair with their head flexed 30° forward (to bring the horizontal semicircular canal truly into a horizontal po-sition) and while wearing Frenzel glasses. The test was described by Bárány.
The normal response after rotation of the chair to the right is for horizontal jerk nystagmus to the left to persist for 10–30 seconds. See Bárány test.
2. Chair test If the chair on which the normal subject sits is suddenly tilted backward, the subject will reflexly sit forward. The subject with bilateral labyrinthine lesions or with Parkinson disease makes no such reaction.
chalasia (from Gr, chalasis; to relax or grow languid) 1. A term of Hippocrates for relaxation. 2. Failure of contraction of the cardiac sphincter of the stomach, leading to acid reflux.
chalastic fits (inhibitory epilepsy) Attacks of general limpness, resembling cataplexy but symptomatic of lesions affecting the medial aspects of both frontal lobes.1708
Chalfont Seizure Severity Scale A scale that assesses and scores the components of seizure attacks that cause patients the greatest disturbance. Factors such as the presence of falling, injuries, loss of awareness, and any
warning of seizures; the occurrence of dropping objects, incontinence, automatisms and convulsions; and dura-tion of the seizures and the time taken to regain nor-mal functioning are rated.1549See also National Hos-pital Seizure Severity Scale, which is derived from the Chalfont Scale.
Chamberlain line A line drawn on a true lateral X-ray of the skull from the back of the hard palate to the posterior border of the foramen magnum. The line nor-mally crosses above all parts of the atlas and axis; if basi-lar impression is present, part of the odontoid process and, in severe cases, other parts of the axis and of the atlas project above this line. However, the same is true in some normal subjects and a better measurement is that of the Bull angle.832
Chamberlain, W.E. (b. 1891) American radiologist in Philadelphia.
chameleon tongue Involuntary withdrawal of the tongue which is being protruded voluntarily; a sign commonly seen in patients with chorea.
champagne-cork papilledema Severe papilledema such that the optic nerve head bulges forward and ex-tends outward in all directions into the vitreous humor, thus resembling a champagne cork (or a mushroom).
chancre An indolent, painless skin or mucosal ulcer at the site of a primary infection with Treponema pal-lidum, which can be isolated from it.
channel The complete system for the detection, am-plification, and display of potential differences between a pair of electrodes.
channelopathies Genetically determined metabolic disturbances of the calcium, chloride, or potassium voltage-gated ion channels in the cell membrane, lead-ing to disparate manifestations. Many of the disorders recognized are listed below, some with notes on the sites of the chrosomal anomaly.
Site of Chromosomal
Disorder Anomaly
Familial periodic paralysis SCN4A, 17q23–25b hyperkalemic (sodium)
Familial periodic paralysis hypokalemic
(calcium or potassium channels) CACNLA3 Paramyotonia congenita (sodium) SCN4A 17q 23–25 Myotonia congenita (Thomsen, CLCN1; 7q
Becker) (chloride) Myotonia fluctuans (sodium) Myotonia permanens (sodium)
Episodic ataxia with myokymia CACNA1 12p13 Autosomal dominant nocturnal CHRNA4
frontal lobe epilepsy
Malignant hyperthermia (ryanodine) Central core disease
Andersen syndrome (potassium) Long Q-T syndrome 1–5 Startle
Benign familial neonatal convulsions 29q 13. 8q24?
178 CHADDOCK REFLEX
ADFNLE 20q13 Generalized epilepsy with febrile 19q13
seizures
Juvenile myoclonic epilepsy (Janz) 6p
Febrile convulsions 8q
Benign familial infantile convulsions 18q?
Partial epilepsy 10q22
X-linked infantile spasms Xp 11.4 Unverricht-Lundberg syndrome 21q22
Lafora body disease 6q 24
Cherry-red spot–myoclonus 6p 21.3 syndrome
MERRF mt DNA
Batten disease 16p 12.1
Juvenile Gaucher disease 1q 21 Familial hemiplegic migraine
(episodic ataxia 2 is closely linked) CACNL4 Calcium channelopathies
Familial adult myoclonic epilepsy 8q 24 chanson de parler See prosody.
Chapple syndrome Unilateral facial weakness with contralateral laryngeal palsy occurring in the newborn.
The latter problem is thought to be due to compres-sion of the superior laryngeal nerve by the thyroid car-tilage in utero as a consequence of excessive lateral flex-ion of the neck.968The syndrome was described by Dr.
Charles Chapple of Philadelphia in 1956.
Charcot artery The lenticulostriate branch of the middle cerebral artery, known as the “artery of cerebral hemorrhage.”
Charcot disease 1. See amyotrophic lateral sclero-sis.9802. Chorea in the elderly, of which Charcot gave the neat description: “the movements are gesticulatory;
they cover a wide area, are irregular, unrhythmical, con-tradictory, and render the accomplishment of any def-inite act a difficult matter.”974The status of the disor-der may be in some doubt.
Charcot edema (blue edema) Non-pitting stasis edema, coldness, and peripheral cyanosis, usually with sensory loss and weakness and occurring as a result of disuse of a limb—a manifestation of hysterical paraly-ses in many of Charcot’s patients but also sometimes seen following injury or stroke.2313
Charcot, Jean-Martin (1825–1893) French neurolo-gist, psychiatrist, and anatomist. After graduation from the University of Paris in 1853 he interned at the Salpétrière, an asylum in Paris for the mentally ill and for those suffering from various chronic diseases, for 4 years from 1858, seeing there a huge variety of previ-ously unclassified disease. He returned in 1862 as at-tending physician and over the next 30 years developed it into a school of neurology that became internation-ally famous. He was elected professor of pathological anatomy in 1872, succeeding Vulpian; 10 years after that, a special chair of nervous diseases was created for him, although his observations did not extend solely to neurology—as witness his accounts of Charcot-Leyden
crystals in the sputum of asthmatics, of intermittent claudication, and of biliary (Charcot) fever.
The starting point for this triumph was his meticu-lous recording of physical signs in all the 5000 patients housed in the hospital, signs that he later attributed to the specific neurological lesions found when the pa-tients came to autopsy. This was the clinico-anatomic method that became the hallmark of the Salpétrière and later of neurology in general. From 1866, Charcot offered lectures on neurological subjects that quickly became popular as a result of the inclusion of patho-physiology with the clinical demonstrations, his own dominating and self-assured manner, and the theatri-cality of the presentations. At these sessions (the Tues-day Lectures), symptoms, diagnoses, and pathology were discussed, focusing on a number of patients brought in for demonstration. The extent to which he actually examined (as opposed to observed) the patients is unclear.
Charcot’s private practice was large, his hospital practice even larger. Honored in his day, he may be considered in retrospect to be the father of modern neurology. His studies encompassed rheumatism and changes in abdominal organs and lungs in old age, or-ganic disease of the nervous system such as muscular atrophy, poliomyelitis, aneurysms, multiple sclerosis (differentiated for the first time in the Tuesday Lectures of 1868), and motor neuron disease (amyotrophic lat-eral sclerosis), in which he demonstrated the involve-ment of the anterior roots as well as the lateral columns in 1869, stroke syndromes, Ménière syndrome, tabetic arthropathy and lightning pains, encephalitis, cord compression, cerebral hemorrhage, hysteria and hyp-notism, and the psychoneuroses as well as art as applied to the nervous system. It was Charcot who differenti-ated tics from chorea. His complete works were pub-lished in nine volumes. The Tuesday Lectures were translated into English after 1877 by George Sigerson (New Sydenham Society).2154,5047
Charcot joint Painless, often gross deformity and de-struction of joints in tabes dorsalis and other condi-tions (e.g., syringomyelia, hereditary sensory neuropa-thy) in which the sensation of pain is lost.970 The absence of proprioceptive sense is also likely to be rel-evant by denying restraint to the muscular contractions and thus permitting abnormal joint positions to be as-sumed. [JF] See also neurogenic arthropathy.
Charcot sign Elevation of the eyebrow on the side of a peripheral facial palsy and its depression when the pa-tient attempts to close the eye.
The sign has also been interpreted as consisting of depression of the eyebrows when the eyes are closed, indicating blepharospasm; or their elevation, indicat-ing apraxia of eye closure.
Charcot syndrome See amyotrophic lateral sclero-sis.
CHARCOT SYNDROME 179