A rare phenotype of GM 2 gangliosidosis 5554

Australian X disease See Murray Valley encephalitis

A. Fulfils criteria for migraine with aura

3. A rare phenotype of GM 2 gangliosidosis 5554

4. Familial bulbospinal neuronopathy with optic atrophy⁴³²⁷ bulbospinal system Pathways that originate in the brainstem, descend to spinal levels, and are concerned in the control of movement and of posture.

bulimia (Gr, a particle hunger) Morbid hunger;

excessive appetite with decreased satiety after eating.

bulimia nervosa (Gr, a particle hunger) (binge eating) 1. Morbid hunger; excessive appetite with de-creased satiety after eating. 2. A chronic but self-limit-ing eatself-limit-ing disorder characterized by an episodic desire to eat large amounts of food, accompanied by a sense of loss of control and often of depression; each episode is followed by purging, starvation, and fasting.

As with anorexia, the patients manifest a fear of fat-ness, a desire for thinfat-ness, a distorted body image, and a denial of illness.⁴⁸⁵⁵The American Psychiatric Associ-ation criteria (DSM-IV-TR)¹⁵⁰amplify this description.

Bull angle The angle between the plane of the hard palate and the plane of the atlas vertebra on a true

lat-eral skull X-ray film. The normal angle is 5°, while patients with basilar impression often have an angle measuring over 13°.⁸³²

Although a popular measure, it has been criticized because the first reference point is not part of the base of the skull, and is therefore unsuitable when defining the relationship of different parts thereof, such as the rim of the foramen magnum.

bulldog reflex Involuntary clenching of the teeth in response to the sensation of any object placed between them; shown by some patients with frontal lobe tu-mors.⁵⁷⁴

Bumke, Oswald Conrad Edward (1877–1950) Ger-man neurologist and psychiatrist who studied medicine in various German universities, graduating from Kiel University. He rose through the ranks in Freyberg, Halle, Rostock, Breslau, Leipzig, and Munich, succeed-ing Kraepelin at Munich University. He is best re-membered for describing the pupillary sign, but he also published extensively on various psychiatric topics.⁵⁰⁴⁷ He collaborated with Foerster in the production of the Handbuch der Neurologie (1935–1937), which be-came the most influential book (in 17 volumes) on the subject in European neurology. He attended Lenin in his last illness (a stroke).

Bumke (anxiety) pupils Dilatation of the normal pupil in response to a stressful psychic stimulus, with an impaired light response. The sign was described by Bumke in his book Die Pupillenstörungen (Jena, 1903), and is said to be absent in schizophrenia.

bundle of Turck A fiber pathway running from the posterior temporal region to the pontine nuclei and from there to the cerebellar vermis. It is probably re-sponsible for the retention of whole-body movements in cases of apraxia.

bungarotoxin (a-BTX) The lethal factor in the venom of the banded krait, which binds so completely and specifically to acetylcholine receptor sites that it supplies a method for their quantification.

Büngner bands Basal laminae which assist in the ori-entation of sprouting axons.

Bunina bodies Eosinophilic, rounded, or elongated in-tracytoplasmic inclusion bodies with clear haloes found in the motor neurons of the spinal cord, brainstem, and motor cortex in amyotrophic lateral sclerosis.

Burdach column (fasciculus cuneatus) The lateral part of the posterior (dorsal) columns of the spinal cord, named by Burdach in 1819. The medial part (fas-ciculus gracilis) is named for Goll. The Burdach nu-cleus is the cuneate nunu-cleus. See also cuneate fascicu-lus and nucleus.

Burdach, Karl Friedrich (1776–1847) German physi-cian, anatomist, and physiologist, and professor in these 138 BULBOMIMIC REFLEX

disciplines in Dorpat and then Königsberg. He was a member of the Naturphilosophen group, concerned with speculative generalizations on brain functions.

He introduced the terms biology and morphology to medicine and named the globus pallidus (1819), inter-nal capsule, putamen, lenticular nucleus, red nucleus, cingulum, cuneus, and amygdaloid nucleus and classi-fied the thalamic nuclei, as well as writing widely in other areas of pathology and physiology.³⁸⁵⁶He named the fasciculus cuneatus, and discovered the red nucleus before Stilling. He was also a neuroanatomical histo-rian; in his Vom Baue und Leben des Gehirns (1819–

1826) he attributed to Willis the first description of the corpus striatum, pyramids, olives, and the anterior commissure, and the description of the anatomy of the connections of the cerebellar peduncles, the stria medullaris, and the eleventh cranial nerve.

Burkitt lymphoma A multicentric tumor composed of poorly differentiated lymphoid cells, common in East Africa and described by Sir Denis Burkitt from Uganda in 1958. Clinically, swellings of the jaw, orbit, and face and ovarian infiltration are the usual features.

Neurological complications include direct or is-chemic myelopathy, multiple cranial neuropathies af-fecting especially the oculomotor and seventh cranial nerves, malignant meningitis, seizures, and delirium.

burn encephalopathy The acute onset of reduction in the level of consciousness, often with confusion, seizures, or neurological signs, or any of these in com-bination, in patients (especially children) suffering from severe burns.

The syndrome is not homogeneous. It affects some 5% of children at risk (usually with over 30% of the body surface burned) within a few days of the injury, but is usually reversible. Multiple metabolic problems are considered to be responsible.³⁹³²The syndrome was first noted by Dupuytren in 1832.

burn neuropathy Multiple mononeuropathies oc-curring in severely burned patients, often with associ-ated complications of sepsis. Vascular occlusion of the vasa nervorum, direct thermal injury, and a dissemi-nated neurotoxin are suggested as mechanisms.³⁶³⁷ Burnett syndrome (milk-alkali syndrome) The oc-currence of weakness, depression, nausea, headache, and mental confusion in patients with hypercalcemia and alkalosis, due to excessive intake of milk and solu-ble alkalis.⁸⁴⁵

Burnier, Louis-Rene (1880–1952) French dermatol-ogist practicing in Paris.

Burnier syndrome (hypophysial nanism) A syn-drome of short stature, optic atrophy, and adiposo-genital dystrophy, with or without obesity, due to a pi-tuitary tumor destroying the anterior lobe in childhood.⁸⁴⁷

burning feet syndrome (Gopalan syndrome, hot feet, happy feet, painful feet, jittery legs) A sensory neu-ropathy causing aching, burning, numbness, tingling, and aching in the legs with vasodilation of the feet, worse with heat and better with cold. It is generally due to deficiency in B-complex vitamins, as occurs in mal-nutrition states and uremia,²²⁰¹but a dominantly in-herited form has been reported.⁵³⁶²

burning hands syndrome A forme fruste of the cen-tral cord syndrome in which limited injury has spared many of the fibers usually affected in that condition but has led to reduced input into the spinothalamic tract from the cervical segments.³⁶³³

burning mouth syndrome (glossodynia, glossopyro-sis, oral dysesthesias) A syndrome of persistent aware-ness of a painful, burning sensation in the mouth and/or tongue seen mainly in mature adult women.

The causes are multifactorial; ill-fitting dentures, vi-tamin B deficiency, anxiety, depression, and cancer-phobia are all cited as etiological factors.³²³³

Burns syndrome See oculoauriculocutaneous syn-drome.

burst A group of waves that appear and disappear abruptly and are distinguished from background activ-ity by differences in frequency for and/or amplitude.

The term does not imply abnormality and is not a syn-onym for paroxysm. (Adapted from the report of the Committee on Terminology, IFCN,⁹⁸⁹ © 1974, with permission from Elsevier Science.)

burst lobe The occurrence of an intracerebral or in-tracerebellar hematoma in continuity with a subdural hematoma, following cerebral trauma.

burst neurons Neurons which, with pause neurons, are found in the paramedian pontine reticular forma-tion (PPRF) and which generate the high-frequency burst discharges that act as the command for saccades to occur.

burst suppression An EEG pattern characterized by bursts of theta and/or delta waves, at times intermixed with faster waves and with intervening periods of rela-tive quiescence.

The term should be used to describe the EEG effects of some anesthetic drugs at certain levels of anesthesia.

(Adapted from the report of the Committee on Ter-minology, IFCN,⁹⁸⁹© 1974, with permission from El-sevier Science.)

The pattern is typically seen in hypoxic brain dam-age and in prolonged status epilepticus, and it indicates significant compromise of the brain’s metabolic in-tegrity, with a poor prognosis.

Burton sign The blue line in the gums just below their junction with the teeth in chronic lead poisoning, described by Dr. H. Burton (1799–1849), a physician at St. Thomas’ Hospital in London.

BURTON SIGN 139

Buschke Cued Recall Test A test of memory (using confrontation naming) which coordinates the encod-ing and retrieval of new information for cued recall with an initial search strategy. The subject is required to name the objects shown in a series of pictures (e.g,

“Which one is a piece of clothing?”), is prompted if he fails to name it, and finally is told the answer. Follow-ing a distractFollow-ing task, the subject is required to recall as many of the 12 pictures shown as possible.^852,853 Buschke-Fuld selective reminding procedure A test of recent memory in which the subject learns 12 words over a dozen trials, being reminded only of those words that he failed to recall on the preceding trial. After 12 tri-als, the subject is required to note from a multiple-choice list those words with which he had difficulty.⁸⁵³The test is very sensitive to the presence of cognitive change.

Busse-Buschke disease See cryptococcal meningi-tis.

butterfly area The nasal and adjacent malar regions of the face.

butterfly glioma A glioma that has spread from one hemisphere to the other directly through the corpus

cal-losum. These lesions may have started within the cor-pus callosum and, by infiltrating both frontal lobes, lead either to increasing stupor or to emotional lability, ir-ritability, or delusional states.

butterfly infarct The pattern of infarction with oc-clusion of the precommunal part of the posterior cere-bral artery, and thus of the penetrating arteries spring-ing from it; and involvspring-ing the medial part of the midbrain tegmentum and the medial part of the thal-amus bilaterally.

Pupillary disorders and disturbances of eye move-ment result.

Buzzard, E.F. (1871–1945) English physician, Regius Professor of Medicine at Oxford.

Buzzard reflex A variant of the knee jerk, in this case obtained as the patient sits with the toes pressing on the floor as a facilitatory maneuver.⁴⁷²³See nociocep-tive reflex.

Bywaters syndrome Traumatic/ischemic myositis with myoglobinemia and myoglobinuria and shock, sometimes leading to renal failure and usually described after extensive crush injuries.⁸⁶⁴

140 BUSCHKE CUED RECALL TEST

C fibers Unmyelinated sensory nerve fibers in the pe-ripheral nervous system, feeding in from receptors that respond to temperature, chemical, and high-threshold mechanical stimulation.

C reflex An abnormal reflex response representing the electrophysiologic correlate of sensory evoked mvo-clonus. The term C was chosen to indicate that the re-flex might be mediated in the cerebral cortex. This is sometimes, but not always, true.¹⁰

This enhanced long-latency EMG response is found in a stimulated muscle (and sometimes in the homol-ogous muscle on the opposite side), particularly in pa-tients with myoclonic disorders, time locked to a pe-ripheral nerve electrical stimulus and equating a myoclonic jerk.

C syndrome (Opitz trigonocephaly syndrome) A re-cessively inherited craniofacial malformation syndrome characterized by trigonocephaly and microcephaly with ridging in the metopic region due to synostosis of the metopic suture, hypotelorism, Mongoloid slant to the eyes, epicanthic folds, strabismus, short neck, congen-ital cardiac malformations, and skeletal anomalies in-cluding syndactyly.^4247,4256

C waves (Traub-Hering waves) Low-amplitude fluc-tuations of the CSF pressure occurring at 4–8/minute, normally present but enhanced in states of raised in-tracranial pressure. The waves correspond with changes in arterial blood pressure.

cacacusis Frightening echoes of mechanical sounds or of words heard or said, symptomatic of right hemi-sphere lesions.⁴⁸⁹⁴See also palinacousis.

CACH (childhood ataxia with diffuse CNS hy-pomyelination; vanishing white matter disease) A het-erogeneous leukodystrophic condition of childhood with variable course resulting from maldevelopments of myelin formation, (dys- or hypomyelination) rather than demyelination.^4960,5719 See fatal infantile leukodystrophy.

cachectic myopathy (neuromyopathy) A syndrome of proximal atrophy with relative retention of power.

Both neuropathic and myopathic causes are suspected, while the underlying problem is considered to be nu-tritional.

cachexia (Gr; bad a habit) Severe loss of subcuta-neous tissue and muscle bulk, the latter without com-mensurate weakness; muscle biopsy shows the presence of scattered atrophic fibers. The condition is the result of severe malnutrition, malignancy, or other systemic illness.

Cachexia mercurialis was the term applied to

wide-spread ulceration occurring in syphilitic patients treated with mercury; cachexia saturnia is an old term for chronic lead poisoning.

cachodia (Gr, a bad smell) Having a bad smell.

cacogenesis (Gr, bad origin) A morbid malforma-tion, such as a monster.

cacogeusia (Gr, bad taste) The perception that all food in the mouth has a foul taste; a symptom of local disease, partial damage to the olfactory nerve, or de-pressive illness.

cacophonia (Gr, bad lack of  voice) Hoarseness of the voice.

cacosmia (Gr, bad smell) Perception of all food as having a foul smell. The causes are similar to those of cacogeusia.

CADASIL See cerebral autosomal dominant arteri-pathy with subcortical infarcts and leukoencepha-lopathy.

cadiva insania (Lat, falling senseless) An ancient term for epilepsy.

caducus (Lat, falling) A term indicating falling—

hence caducus morbus, another ancient term for epilepsy.

Cadwalader, Thomas (1708–1779) American physi-cian who first described lead palsy and lead colic in his Essay on the West Indian Dry-Gripes, published in Philadelphia by Benjamin Franklin in 1746.⁴⁷²³ caeruleus (ceruleus) (from Lat, dark blue) Blue, azure.

café-au-lait spots Brown skin macules, possessed by many healthy people but a diagnostic requirement for von Recklinghausen disease, in which there must be more than six present. Of these, at least one should measure over 5 mm in its longest axis in prepubertal people; after puberty, at least one should measure more than 15 mm.

caffeine withdrawal headache A variety of rebound headache for which the following diagnostic criteria are suggested:¹⁰⁵¹

A. Patient has consumed caffeine daily or at a rate of 15 g monthly

B. Occurs within 24 hours after last caffeine intake C. Is relieved within 1 hour by taking 100 mg of caffeine Caffey-Silverman syndrome See infantile cortical hyperostosis.

cage paralysis Vitamin B12deficiency in captive pri-mates fed a fruit diet deficient in that vitamin.

caida de mollera See fallen fontanelle.

C

141

Cairns, Sir Hugh William Bell (1896–1952) Aus-tralian neurosurgeon, born and educated in Adelaide and seeing service in World War I before returning to Australia to graduate in 1917. He was then elected to a Rhodes scholarship at Balliol College, Oxford, and, fol-lowing that, worked at the London Hospital. After studying with Harvey Cushing on a Rockefeller fel-lowship, he turned to neurosurgery and later joined the staff first of the London Hospital and the National Hospitals for Nervous Diseases in Queen Square and Maida Vale and subsequently of the Radcliffe Infirmary as the first Regius Professor of Surgery at the Univer-sity of Oxford.

During the World War II he established and ran a head injury base hospital in Oxford and organized mo-bile neurosurgical teams for the front. He designed a crash helmet and leg guard for motor cyclists, which saved many lives and prevented many fractures. His nu-merous publications include studies of gliomas and subdural hematomas, the use of antibiotics, problems of consciousness, and neuro-ophthalmology.^2824,5014

due to a disorder of cortical function secondary to a di-encephalic lesion.⁸⁷⁰2. Hydrocephalus due to blockage of the CSF pathways by meningitic exudate.⁸⁶⁹ caisson disease See decompression sickness.

Cajal cells Astrocytes.

Cajal, Santiago Ramon y (1852–1934) Spanish neu-rohistologist and anatomist who trained in Saragossa. He was appointed professor of anatomy at Valencia Uni-versity at the age of 29 but later transferred to Bologna and then returned to Spain as professor of histology and morbid anatomy in Madrid. His major work, Histologie du système nerveuse de l’homme and des vertèbres (Paris, 1909–1911), established him as the father of neurohis-tology, although some of the credit belongs to Camillo Golgi, a modification of whose silver stain Cajal used, and with whom he shared the Nobel prize for 1906. His textbook of general pathology⁴⁵⁷⁸ became a standard source. Cajal also introduced a silver nitrate stain to show the content of the neuronal cytoplasm; this stain was em-ployed by Alzheimer to demonstrate neurofibrillary tan-gles. Among his students or collaborators were Lorente de No, Lafora, and Wilder Penfield.

Cajal enunciated his neuronal theory in three parts.

First, nerve cells are independent elements that do not anastomose (as in a syncytium) but make contact only at specific points. Second, nervous impulses are always transmitted from the cell body out to the axon; and third, the axon conducts away from the cell body. His autobiography has recently been published.⁴⁵⁷⁹ Cajal stain A metallic impregnation method of stain-ing astrocytes, usstain-ing gold chloride sublimate.

calamus scriptorius (Lat, a quill or reed of a writer) The inverted triangle, somewhat resembling the point of a pen, on the dorsum of the medulla in the floor of the fourth ventricle; so named by Herophilus, a Greek physician and anatomist in Athens (335–280 B.C.) calcification of the basal ganglia 1. An asympto-matic condition discovered fortuitously on X-ray stud-ies, CT scans, or MRI scans of the head. 2. Calcium dep-osition in the basal ganglia as occurs in numerous progressive white matter degenerations, true and pseudohypoparathyroidism, hyperparathyroidism, mi-tochondrial cytopathies, some infections including HIV infection and toxoplasmosis, tuberous sclerosis, Down syndrome, and systemic lupus erythematosus, as well as following many forms of toxic, radiation, or other dam-age to neural tissue. See striatopallidal calcification.

3. A specific fatal syndrome in infants who present with microcephaly, bilateral spasticity, dystonia, and CSF lymphocytosis.⁶¹See also choroidocerebral calci-fication syndrome.

calcinosis universalis See Profichet syndrome.

calcitonin myopathy A myopathic syndrome charac-terized by medullary carcinoma of the thyroid, neuromas 142 CAIRNS, SIR HUGH WILLIAM BELL

Sir Hugh William Bell Cairns

Cairns syndrome 1. persistent vegetative state with near-complete loss of all but ocular movements and of emotional response. “Oft-repeated commands may be carried out in a feeble, slow and incomplete manner but usually there are no movements of a voluntary charac-ter.”⁸⁷⁰ Voluntary extraocular movements, chewing, swallowing, and breathing are maintained. There is in-continence and, in some cases, decorticate rigidity.

Fluctuations may occur.

The condition is produced by lesions in and around the third ventricle. Cairns considered this state to be

of the tongue and eyelid, prominence of the lips and stig-mata of Marfan syndrome, and skin pigmentation.¹²²⁰ calcium ATPase deficiency A rare disorder of muscle characterized by exertional muscle contractures with rhabdomyolysis and impaired muscle relaxation, due to a deficiency in sarcoplasmic reticulum-ATPase.⁴⁴⁷³ calcium gene-related peptide A neurotransmitter normally found at cholinergic sites in the motor system but also released in the walls of cranial vessels in mi-graine attacks, at which sites it has a local inflamma-tory action.

Calderon syndrome (kinky hair–photosensitiv-ity–mental retardation) A congenital dysmorphic syn-drome characterized by sparse, brittle, and kinky hair, absent eyebrows and eyelashes, skin photosensitivity, and mental and developmental delay.³³⁷ See also Menkes disease.

calibration 1. The testing and recording of the re-sponses of EEG channels to voltage differences applied to the input terminals of their respective amplifiers. DC (usually) or AC voltages of magnitude comparable to the amplitudes of EEG waves are used. 2. The testing of the accuracy of paper speed regulation by means of a time marker. (Adapted from the report of the Com-mittee on Terminology, IFCN,⁹⁸⁹ © 1974, with per-mission from Elsevier Science.)

California encephalitis Direct viral infection of brain due to bunyavirus members of the arbovirus group. Clinically, the disease typically affects children in the central and eastern parts of the United States and southern Canada and occurs seasonally, mainly in early fall, presenting with typical features of encephalitis with focal neurological signs and stupor but with a low mor-tality and few residua.²⁸⁶⁴

California Verbal Learning Test A recently developed test of aural learning, recall, and recognition memory for a list of words over five trials and allowing assessment of different components of memory as well as of retrieval strategy and other executive functions.¹³⁶³

Call-Fleming syndrome The occurrence of va-sospasm following subarachnoid hemorrhage or hem-orrhagic stroke. The clinical presentation is with seizures, and initially fluctuating neurological deficits but permanent neurological disability ensues. (Call GK.

et al. Stroke 1988;19:1159–70.)

callosal agenesis Failure of formation of the corpus callosum due to a developmental failure during em-bryogenesis. See agenesis of the corpus callosum.

callosal anomia See anomia.

callosal apraxia Apraxia of the left hand resulting from infarction of the corpus callosum.⁵⁸⁷³See apraxia.

callosal disconnection The association of tactile anomia, agraphia, and apraxia affecting the left hand,

described in multiple sclerosis.⁴⁹⁸⁵Left-ear suppres-sion on dichotic listening tasks is another feature of the syndrome.

callosal fissure The space between the dorsum of the corpus callosum and the overlying hemisphere.

callosal syndrome See Bristowe syndrome.

callosotomy Section of the corpus callosum: a surgi-cal therapy for intractable generalized and akinetic seizure disorders for half a century, now more widely accepted than previously on account of reduced mor-bidity from the use of microsurgical techniques.⁴²⁰⁶ Calmeil, Louis-Florentin (1798–1895) French physician who described general paralysis in a book in 1826, immediately after Bayle’s description of the same condition. He also introduced the term absence into medicine.

calmodulin The main calcium-binding protein in cells. Ca^2ATPase is the main enzyme responsible for its removal. With depletion of high-energy phosphates resulting from ischemia, the Ca^2ATPase system quits and Ca^2levels increase within cells. High Ca^2levels are cytotoxic, and cell death only occurs in the presence of Ca^2.

caloric after-nystagmus See vestibular nystagmus.

caloric nystagmus Nystagmus of the type seen in pe-ripheral vestibular lesions and induced by warm or cold stimulation of the labyrinths. Cold stimulation leads to nystagmus with its fast component away from the irri-gated side, and warm stimulation produces nystagmus in the other direction. See vestibular nystagmus.

caloric paresis A relative decrease in the responsive-ness of one side compared with the other on caloric testing. See canal paresis.

caloric tests (caloric irrigation tests) The introduc-tion of hot or cold (44°C or 30°C) water or air into the external auditory meatus to test eighth (vestibular) cra-nial nerve function through stimulation of the recep-tors in the semicircular canals. The change in ambient temperature sets up convection currents in the en-dolymph of the horizontal semicircular canal when the subject lies supine with the head elevated 30° from the horizontal. These induce vertigo and nystagmus (with the fast component away from the canal when irrigated with cold, and toward the canal when irrigated with warm) in the normal subject, the maximal velocity of the slow component being recorded using electronys-tagmography.

See also canal paresis, directional preponderance, Barany test.

calvaria, calvarium (from Lat, baldness) The vault of the cranium.

calvarial hyperostosis (Morgagni syndrome) See hyperostosis frontalis interna.

CALVARIAL HYPEROSTOSIS 143