Australian X disease See Murray Valley encephalitis
A. Fulfils criteria for migraine with aura
5. Functioning and self-care
1. No difficulty, either subjectively or objectively 2. Complains of forgetting location of objects. Subjective
work difficulties
3. Decreased job functioning evident to co-workers. Diffi-culty in traveling to new locations
4. Decreased ability to perform complex tasks (e.g., plan-ning dinner for guests, handling finances, marketing, etc.)
5. Needs help in choosing proper clothing
6. Needs help in feeding, toileting, bathing, and/or ambu-lating
7. Requires constant assistance in all activities of daily life From Reisberg, 1988.4649
Brief Psychiatric Rating Scale A relatively short ob-server-rated scale assessing 16 items including depres-sive symptoms, with the object of assessing changes after treatment.4281The scale concentrates on psycho-pathology, with less emphasis on the manifestations of dementia such as somatic and self-care factors.
A similar scale for the assessment of geriatric patients has also been constructed.4366
brief small abundant motor-unit action potentials A pattern of voluntary EMG activity which suggests, but is by no means diagnostic of, primary myopathy.1675 See BSAPPs.
Brief Symptom Inventory A self-report questionnaire used for individual screening of depressive symptoms.1415 Bright, Richard (1789–1858) English physician and pathologist born in Bristol. After training in Edinburgh, at Guy’s Hospital, and in Europe, he practiced in Lon-don where he was appointed physician to Guy’s Hos-pital in 1824. He is best remembered for his descrip-tions of chronic renal disease and of various neurological conditions, collected in the two volumes of his Reports of Medical Cases (1827–1831), in which inter alia he described the features of certain seizure types, recognizing the sensory march,745painful later-alized seizures, secondary generalization of focal seizures, ictal amaurosis, and olfactory auras.
His account of postictal paralysis preceded that of R.B. Todd. He described absence attacks as follows:
“Often it is the momentary absence of mind, the eye fixed in thought, yet gazing vacantly, no convulsion, no sound, the occupation of the hand ceases while the mind for the moment is annihilated; the cloud passes off, the intellect returns and often unconscious that its operation has been suspended, the patient resumes the occupation in which he was engaged.”
He obtained and himself performed autopsies when-ever possible, leading both to a superb pathological col-lection (retained at Guy’s) and to contemporary recog-nition of the value of understanding the pathology of disease. Thus he was able to show that focal epilepsy was as likely to originate from brain disease as was the generalized variety, at a time when Brown-Séquard, for example, was advocating amputations for the con-trol of focal seizures.6088
He was also a noted geologist; his collection, ex-panded by the purchase of the fossil section of that amassed by James Parkinson, passed eventually to the Institute of Geological Sciences.
Briquet, Paul (1796–1881) French physician whose two theses were on varicose veins and artificial illumi-nation, respectively, but who also published on tuber-culosis, cholera, and hysteria.
Briquet syndrome 1. Aphonia and dyspnea, consid-ered by Briquet (1859) to be due to hysterical paralysis of the diaphragm; this is almost certainly the hyper-BRIQUET SYNDROME 131
ventilation syndrome. 2. Hysteria, specifically hyster-ical anesthesia of the skin with consequent imprecision of movements (“ataxia”). Briquet regarded this as a
“neurosis of the brain” in which unpleasant environ-mental events acted upon the “affective part” of the brain in a susceptible and predisposed person. His book750finally disposed of the old concept that hyste-ria was in some way associated with wanderings of the uterus. 3. A chronic illness characterized by the occur-rence of multiple symptoms affecting multiple organ systems, resulting from conversion disorder.
His work and concepts of hysteria have been re-viewed.3577
Brissaud convolution The gyrus parietalis transversus.
Brissaud disease (chorea variabilis) See Tourette syndrome (which was in fact what he was describing).751 Brissaud, Edouard (1852–1909) French neuropsy-chiatrist. As a student he trained under Charcot, Mil-lard, and Lasègue and after graduation became chef de clinique at the Pitié. He later deputized for Charcot in his absences, filling the Chair at the Salpétrière tem-porarily after Charcot’s death.
He was elected professor of the history of medicine in the Faculté de Médicine in Paris, and later was pro-fessor of pathology. With Pierre Marie he founded the Revue Neurologique in 1893. Brissaud wrote extensively on such subjects as neuroanatomy, hysteria, asthma, acromegaly, parkinsonism, syphilis, involuntary move-ments, hypnosis, and medical history. He also gave a good account of tuberous sclerosis, but after Bourne-ville. Horsley operated on his brain tumor in Paris, but this did not prolong his life.
Brissaud infantilism A syndrome of the persistence of infantile characteristics beyond the age of puberty, with retarded growth and delayed mental development.
Round, chubby face, underdeveloped genitalia, dwarfism, lack of body hair, and delayed epiphyseal ossification are characteristic. The condition probably represents infantile myxedema.752
Brissaud reflex Contraction of the tensor fascia lata in response to scratching the sole of the foot; seen in pyramidal disease.
Brissaud syndrome 1. See Brissaud infantilism. 2.
Sinistrosis (“shell-shock”), but the term was used by him to describe the features of traumatic or compen-sation neurosis: “It is a pathological attitude in an in-jured person who refuses to recognise that he is cured as he genuinely believes that he has not obtained just legal compensation for the injury received. He is basi-cally a claimant who over-estimated his right to be in-demnified.”753
Brissaud-Lereboullet syndrome (hemicraniosis) Hemifacial and hemicranial hyperostosis associated with underlying meningiomas or sarcomas, raised in-tracranial pressure, visual disturbances, exophthalmos, and seizures.
The condition probably represents sarcomatous change in a long-standing meningioma.754
Brissaud-Marie syndrome Hysterical glossolabial paralysis in which the deviation of the face and of the tongue are unphysiological with respect to the limb weakness.755,757,4723
Brissaud-Meige syndrome See hemifacial spasm.
Brissaud-Sicard syndrome Facial hemispasm asso-ciated with contralateral hemiparesis due to a caudal-ventral pontine lesion, described in 1908.756
Bristowe, John Syer (fl. 1885) English neurologist and Fellow of the Royal Society who described the ef-fects of callosal tumors in Brain in 1885.
Bristowe syndrome (callosal syndrome) The pattern of symptoms associated with tumors of the corpus cal-losum, comprising negativism, disturbances of mem-ory, concentration, and personality, and hemipare-sis.121Tumors affecting the anterior part of the corpus callosum may lead to apraxias of the left hand as well.
British Journal of Neurosurgery A professional spe-cialist journal published for the Society of British Neurosurgeons. Web site: http://www.tandf.co.uk/
journals/titles/02688697.html.
British Medical Journal (BMJ) A general medical journal, also publishing items of neurological interest.
Access to the journal is free, on-line: http://bmj.com/.
Brixa-Koppens sign See Gowers signs.
broad A-band disease A benign congenital myopa-thy characterized clinically by neonatal hypotonia, with 132 BRISSAUD CONVOLUTION
Edouard Brissaud
or without congenital blindness due to retinal dystro-phy, and delay in speech amd development. Ultra-structural abnormalities include disorganization of myosin thick filaments leading to a loss of A band/I band demarcation and an impression of broadening of the A band. See also Leber congenital amaurosis.4028 Broadbent apoplexy Intracerebral hemorrhage which penetrates into the ventricles.762
Broadbent law With a lesion of the upper motor neuron above the facial colliculus in the pons, the hand and arm are most affected, the leg next so, and the lower face and tongue only slightly.
Broadbent, Sir William Henry (1835–1907) English neurologist and cardiologist who trained in Manches-ter and London, studying further under Trousseau in Paris after graduation. He practiced largely from St.
Mary’s Hospital, London, and was elected a fellow of the Royal Society in 1896. He was created a baronet as a result of his services as Physician to Queen Victoria and King Edward VII.
His neurological publications concerned hemiplegia, aphasia, and the preceding two entries.
Broca aphasia (Broca dysphasia, nonfluent aphasia, agrammatic aphasia). A classic nonfluent dysphasia characterized by awkward slow and effortful articula-tion, loss of prosody, impairment of repetition and naming, and restriction to simple grammatical forms in speech and writing (agrammatism) so that sponta-neous speech is of a telegraphic type.
Agrammatism leads to the use of single words, words with a high substantive content (nouns and verbs), but few articles, pronouns, prepositions, or conjunctions.
The normal syntactic relations are lost. Repetition and confrontation naming are also much affected but there is relative preservation of comprehension. Phonetic dis-integration (mainly shown as trouble in initiating speech and in selecting and blending words) and mispronunci-ation of phonemes (such as B for P) also occur.
The causative lesion involves the posterior part of the inferior frontal gyrus of the dominant hemisphere, the anterior parietal region, the insula, and both banks of the rolandic fissure with deep extension.763The condi-tion has been separated into two types:
Persisting Broca aphasia (big or true Broca aphasia) This type usually begins with a global aphasia and right-sided paresis with evolution to a state in which spoken language is comprehended but a nonfluent and agrammatical speech with poor repetition remains, which is due to ex-tensive damage affecting not only the inferior left frontal gyrus but also the surrounding cortex and the underly-ing white matter and basal ganglia, as above.
Broca area infarction syndrome (little, mild, or acute Broca aphasia) A nonfluent agrammatic output is present at the beginning of the disorder, comprehension is preserved, and the speech disturbance improves to a state of near normality except for some impairment in prosody. Any initial mutism is replaced by rapidly abating dyspraxic
and effortful articulation and there is significant perma-nent disturbance in language function. The lesion re-sponsible is a restricted infarction of the posterior por-tion of the dominant inferior frontal gyrus.3937 Broca dysphasia See Broca aphasia.
Broca, Pierre-Paul (1824–1880) French anthropolo-gist, anatomist, and professor of clinical surgery at the Bicêtre in Paris; later a politician. He described hemi-spheric dominance and determined that right-handed-ness was associated with the representation of speech in the left hemisphere, thus defining the anatomical correlate of nonfluent aphasia (1861).763He also de-scribed the major features of motor or expressive apha-sia in his patient Tan, and was the first to trephine for a cerebral abscess which he had localized correctly by clinical means (the only means available).
He reported in 1865 the cases of eight aphasic (“aphemic”) right-handers with left hemisphere lesions and stated that the left inferior frontal convolution was the center for articulate speech.764,2533 He also de-scribed muscular dystrophy before Duchenne, rickets, the treatment of cerebral aneurysms, clubfeet, fractures, and the use of hypnotism in surgery.
As an anthropologist he was interested in craniology (naming the inion, bregma, dacryon, lambda, meto-pion, obelion, and opisthion) and he was involved in the discovery and reports on Neanderthal man. He founded the French school of anthropology.
BROCA’S AREA 133
Pierre-Paul Broca
Broca’s area (Broca’s convolution) The motor speech area; the posterior part of the inferior (third) frontal convolution, on the left side in a large majority of people.
Broca’s area infarction syndrome See Broca apha-sia.
Broca’s convolution See Broca area.
Brodmann areas Fifty-two cortical areas, containing 11 regions distinguished by the structure and disposi-tion of their cellular layers (cytoarchitectonics) de-fined by Brodmann in 1903–1908.1478
Brodmann, Korbinian (1868–1918) German physi-cian who initially trained in psychiatry in Jena, Frank-furt, and Berlin but who later was appointed in suc-cession professor of anatomy in Tübingen, Halle, and Munich, where he pioneered the study of cytoarchi-tectonics.
Brody syndrome (Lambert-Brody syndrome) A re-cessive muscle disease of childhood mapped to chro-mosome 16p12; and due to functional deficiency of Ca2 ATPase in the sarcoplasmic reticulum of fast-twitch skeletal muscle. It is characterized clinically by impaired muscle relaxation after voluntary contraction, with cramps, stiffness and myalgia being induced by ex-ercise and cold. Progression is slow. The EMG shows that the cramps are electrically silent. Muscle biopsy re-veals only type II fiber atrophy.266,481,766Dominant in-heritance has also been reported.
bromide A halogen, introduced by Locock for the treatment of “hysterical” epilepsy in 1857,3451 on the bases that epilepsy was (at least in some cases) a man-ifestation of abnormal sexual tendencies, and that bro-mides had been found to cause impotence. As a result of Locock’s paper, Dr. C.B. Radcliffe and Sir Samuel Wilks both generalized the indications for its use to treatment of organic epilepsy.
bromide partition test A chemical test performed on CSF samples providing evidence for or against a diag-nosis of tuberculous meningitis.3591
bronx cheer A derogatory expression, a compound of eructation and retching. When involuntary, it is clas-sified as a complex vocal tic, a manifestation of Tourette syndrome; when voluntary, it is merely an expression of opinion.
Brossard, Jules (1855–1911) French physician.
Brossard scapulodistal syndrome A scapulopero-neal syndrome, probably recessively inherited.782 Brown ataxia See Sanger Brown ataxia.
Brown, Charles Leonard (b. 1899) American pathol-ogist, practicing in Boston and New York.
Brown, Jason W. American neurologist in Los An-geles.
Brown, Sanger (1852–1928) Canadian-born neurol-ogist who trained in medicine at Bellevue. He
intro-duced a new system of classification of patients at the mental hospital where he was first appointed which led to the discharge of a number of them. He achieved prominence in psychiatry, going on to be medical su-perintendent in other hospitals in the northeastern United States.
In 1886 he studied neurology with Sharpey-Shafer in London and established the role of the occipital lobe in vision. He settled in Chicago in 1890, be-coming professor of forensic medicine and hygiene at Rush and of clinical neurology at the University of Illinois.
134 BROCA’S AREA INFARCTION SYNDROME
Sanger Brown
Brown syndrome (a syndrome of the neural crest) Loss of deep pain sensitivity, retention of superficial pain, autonomic dysfunction, hypoplasia of dental enamel, meningeal thickening and cystic change, hy-poreflexia, mild mental retardation, and blond hair, blue-green eyes, and a fair complexion.795
The authors suggested that this association of signs indicated that there was a failure of differentiation of the neural crest as the underlying embryonic abnor-mality.
See superior oblique tendon syndrome. The con-dition was described by Dr. H.W. Brown in 1950.
Brown-Peterson paradigm A test used for the as-sessment of short-term memory. It typically consists of the presentation of a set of three items, after which the patient is asked to engage in some distracting activity such as counting backward from a three-digit number for between 2 and 30 seconds. Reproduction of the
orig-inally presented material is then requested. Between 10 and 20 trials are usually administered.4424
Brown-Séquard, Charles-Edouard (1817–1894) A neurologist of Eurasian and Irish-American parentage who was born in Mauritius but who trained in Paris, first for a literary and later for a med-ical career. Trousseau was one of his teachers, and af-ter graduation he worked with Baron Larrey. In his thesis on the physiology of the spinal cord, accepted in Paris in 1846; he described the sensory decussa-tion within the cord. Constrained by the Second Em-pire and short of money, he left France and visited New York and then Mauritius (where he was given a gold medal to commemorate his handling of a cholera epidemic) before settling to practice in Rich-mond, Virginia in 1855. He felt it proper to withdraw from this city following a tactless remark of his about slavery, and the resulting remarks of others upon his own mixed blood.
He returned to Paris in that year and published his findings on the functions of the adrenal glands. In 1858 he lectured in London, Glasgow, and Dublin, after which he was held in the highest esteem and was elected Fellow of the Royal Society and also Fellow of the Royal College of Physicians in 1860. At this time he was ap-pointed as the first physician to the National Hospi-tal, Queen Square, a position that he held until 1863 when he accepted the Chair of Nervous Pathology at Harvard. There he remained until the death of his wife in 1868, when he returned to Paris and accepted the Chair of Experimental Physiology at the Faculté de Médicine. In 1872 he returned to New York and re-married, but he went again to Paris on the death of his second wife 2 years later.
In 1877 he accepted the post of professor of physi-ology at Geneva, married for the third time, and re-mained in Geneva for a year before succeeding Claude Bernard as professor of experimental medicine at the Collège de France in Paris, a post that he retained un-til his death.
Although noted for many physiological observations, including that of caloric testing 45 years before Bárány, his name is chiefly remembered for his descriptions in 1850 and 1851 of the syndrome resulting from lateral hemisection of the spinal cord, popularly (but proba-bly incorrectly) thought to have been brought to his at-tention by the activities of the Parisian mafioso who set-tled their scores with stilettos so thin as to be able to penetrate into the spinal canal809and for his demon-stration that excision of the adrenal glands was followed by features of Addison disease.
His medical interests exceeded neurology,5672and he reported late in life on the effects he experienced from the self-injection of an extract of guinea pig testicles, which he regarded as entirely satisfactory.
BRUDZINSKI SIGNS 135
Charles-Edouard Brown-Séquard
Brown-Séquard spinal epilepsy Muscle spasms aris-ing because of the presence of an irritative lesion af-fecting the motor fibers in the spinal cord. Despite the name, the condition is not truly epileptic but rather a form of segmental myoclonus.574
Brown-Séquard syndrome Damage to the lateral half of the spinal cord, leading to ipsilateral loss of py-ramidal and posterior column function and contralat-eral spinothalamic loss, with evidence of a root lesion at the site of the cord injury. Direct trauma was once, and extrinsic compressive lesion is now, the most com-mon cause.809
Brown-Symmers disease (acute serous encephalitis) See acute disseminated encephalomyelitis.789 Brown–Vialetto–Van Laere syndrome See ponto-bulbar palsy with deafness.
Bruch membrane The structure supporting the pig-mented epithelial layer overlying the rods and cones in the retina.
Bruck–de Lange syndrome A fatal infantile syn-drome of psychomotor delay, extrapyramidal rigidity, movement disorder, and local or generalized muscular hypertrophy.1356See De Lange syndrome.
Brudzinski, Josef (1874–1917) Polish physician who graduated from Moscow and specialized in pediatrics.
After a tour of European schools he returned to War-saw and then was appointed head of a children’s hos-pital in Lodz, transferring to Warsaw again as rector of the university. The sign for which he is best remem-bered (described below) was described in 1909.810 Brudzinski signs 1. Neck phenomenon Flexion of the hips and knees (sometimes with extension of the
hal-lux and fanning of the toes) in response to passive neck flexion.
2. Contralateral leg sign The involuntary flexion or extension of one leg in response to forceful passive flex-ion of the other leg at the hip (straight leg raising).810 In each case it is a sign of meningeal irritation or in-flammation. The reciprocal contralateral leg sign is seen when one leg is flexed at the hip and knee and the other is extended. When the flexed limb is lowered to the bed, the other leg flexes.
3. (Cheek sign) Flexion of the elbows with upward jerking of both arms in response to pressure on the cheeks.811
4. (Symphysis sign) Flexion of the legs in response to suprapubic pressure.374
Most of these signs were described in patients with tuberculous meningitis.4723,5047
bruit (Fr, noise) An abnormal sound generated as a result of the turbulent flow of blood within arteries and heard through a stethoscope.
bruit de pôt fêlé See cracked-pot note.
Brumback syndrome A congenital dysmorphic syn-drome characterized by microcephaly, cerebral atro-phy, agenesis of the corpus callosum, and Brushfield spots on the iris.
Brunauer syndrome A dominantly inherited syn-drome of mental retardation, defective enamel forma-tion and hyperhidrosis with hyperkeratosis of the palms and soles.2221
Bruns ataxia See Bruns gait apraxia.
Bruns gait apraxia (Bruns ataxia, apractic or mag-netic gait) Inability to initiate the process of forward motion by alternating steps, although the power and coordination of the legs is normal in the lying or sit-ting positions. The maintenance of the feet on the floor, removed only with great effort, has led to the alterna-tive name magnetic gait.
This static ataxia in the presence of frontal lobe dis-orders is due to involvement of the descending efferent frontocerebellar fibres and was originally described in the setting of frontal lobe tumors but is now more com-monly seen in frontal ischemic disease. The constella-tion of abulia, psychomotor retardaconstella-tion, primitive re-flexes, and ideomotor apraxia with cerebellar findings is suggestive.816
The entity is of uncertain validity, and has been considered to have cerebellar, apraxic, agnosic, and labyrinthine components.818 See frontal dysequilib-rium.
Bruns, Ludwig (1858–1916) German neurologist, who studied at Munich and Göttingen Universities and trained in neurology with Hitzig and later in Paris. Af-ter some time in Berlin with Westphal and Oppen-heim, he left to direct the Department of Internal
Med-icine at Hanover University where he was later ap-pointed professor.
In 1892 he described the gait disorder named for him816and wrote on hysteria, callosal tumors, goiter, tetanus, parkinsonism, the optic nerve, birth injuries, and diabetes. He was also the author (in collaboration) of a large and influential Textbook of Pediatric Neurol-ogy.818
136 BRUIT
Ludwig Bruns
Bruns nystagmus The combination of small-ampli-tude, rapid-jerk nystagmus in the primary position, beating away from the side of an extraaxial mass lesion compressing the vestibular nerve and the brainstem, in association with slow, large-amplitude, gaze-evoked, or gaze-paretic nystagmus toward the side of the lesion.6075 The syndrome occurs typically with cerebellopontine angle tumors characterized by gaze paretic nystagmus evoked by gaze to the side of the tumor and by vestibu-lar nystagmus evoked by gaze to the opposite side.
Bruns syndrome Vertigo, vomiting headache, and visual disturbance during change in head posture; a sign of cerebral tumors, usually in the posterior fossa but also occurring with, for example, bilateral subdural hematomas.817
The original description was of a man with cysticer-cosis, who had no symptoms unless he moved his head quickly.122
Brushfield spots Mottled or speckled, white or light yellow pin-points regularly placed in a ring near the outer margin of the iris, found in 90% of people with Down syndrome and in about a quarter of normal sub-jects when they are known as Wolfflin nodules.
Brushfield could not find the spots in patients with brown irides.819