Australian X disease See Murray Valley encephalitis
A. Fulfils criteria for migraine with aura
2. Severe dementia (e.g, Mini-Mental State score of less than 10)
Adapted from Bennett DA, Wilson RS, Gilley DW et al. Clinical diagno-sis of Binswanger’s disease. J. Neurol. Neurosurg. Psychiatry 1990;
53:961–965, by kind permission.459
BINSWANGER, OTTO LUDWIG 113
Otto Ludwig Bingswanger
Binswanger, Otto Ludwig (1852–1929) Swiss physi-cian who qualified in Medicine at Zurich University and
who later studied psychiatry under Meynert in Vienna.
He became professor of psychiatry at Jena University and directed a military hospital during the World War I. He is best remembered for his paper “The Differentiation of Progressive General Paralysis,” published in 1894,577in which he suggested a vascular rather than a syphilitic cause for the dementing syndrome now named for him, but he also wrote extensively on epilepsy, hysteria, and delirium. He retired, following a severe attack of “Span-ish” influenza (encephalitis lethargica) in 1919.
biological clocks Pacemaking mechanisms in the brain controlling circadian rhythms, likely situated within the suprachiasmatic nuclei in the anterior hy-pothalamus.
biopsy The surgical removal of tissue (such as mus-cle, nerve or brain) for microscopic or bacteriological study.
biopterin deficiency See phenylketonuria.
Biot breathing A pattern of respiration marked by irregular grouping of the breaths that vary in depth and between which there are apneic pauses of different lengths. These occur in patients with dorsolateral medullary lesions and in such diffuse diseases as menin-gitis and uremia. The pattern is not (as has sometimes been suggested) regular, rapid, or rhythmical.
Dr. C. Biot (b. 1848) was a French physician who pub-lished his description in 1876 while an intern in Lyon.578 biotin-responsive encephalopathy (OMIM 252260) A recessively inherited, progressive neurological syn-drome of early childhood due to deficiency of bio-tinidase.6023It is mapped to 3p25.
Clinically, the disease is phenotypically diverse, and may be characterized by alopecia, skin rash, encepha-lopathy, ataxia, hypotonia, hearing loss, myoclonus, neonatal seizures, developmental delay, lactic acidosis, and/or aminoaciduria.4885
The condition is one cause of the Ramsay-Hunt syn-drome of seizures, myoclonus, and ataxia. See multi-ple carboxylase deficiency.
biotin-responsive multiple carboxylase deficiency See holocarboxylase deficiency.
biphasic action potential An action potential that crosses the baseline only once, producing two phases (one above and the other below the baseline).
biphasic end-plate activity See end-plate activity.
biphasic motor reaction The occurrence of a flexor reflex followed by extension of the legs in patients with incomplete spinal cord lesions, after any form of stim-ulation given to sites below the level of the lesion.
biphasic wave See diphasic wave.
bipolar With two opposite poles or states. Hence bipolar depression, bipolar cells (neurons with two ax-ons), and the following entries.
bipolar derivation A recording from a pair of ex-ploring electrodes. See also exex-ploring electrode, bipo-lar montage.
bipolar montage Multiple bipolar derivations in which no electrode is common to all derivations. In most cases, bipolar derivations are linked, i.e., adjacent derivations from electrodes along the same array have one electrode in common, connected to the input ter-minal 2 of one amplifier and to the input terter-minal 1 of the following amplifier. See also referential montage.
(Adapted from the report of the Committee on Ter-minology, IFCN,989© 1974, with permission from El-sevier Science.)
bipolar needle electrode A recording electrode that measures the voltage between two insulated wires ce-mented side-by-side in a steel cannula. The bare tips of the electrodes are flush with the level of the cannula which may serve as a ground. (From the 2001 Report of the Nomenclature Committee of the American As-sociation of Electromyography and Electrodiagnosis.10 Used by permission, copyright © 2001, AAEM.) bird-like face syndrome See Seckel bird-headed dwarfism.
Birnbaum syndrome A syndrome characterized by chronic progressive chorea with cerebellar atrophy, de-scribed in 1941.587
bisynchronous A tautologous contraction the equally tautologous term “bilaterally synchronous.”
bitemporal hemianopia Loss or reduction in effi-cacy of the visual field on the temporal side in each eye.
The first complete analytic description was in 1915 by Cushing and Walker,1233 who pointed out that the usual cause was a hypophyseal tumor and that the de-fects were rarely symmetrical.
They divided such visual defects into eight stages ac-cording to their severity, among which stage 1 repre-sented a slant in the upper boundary of the temporal field and a corresponding quadrantic defect in the color peripheries; stage 4 represented hemianopsia, often with macular sparing; and stage 8 indicated complete blindness.
The development of the deficit was charted by Traquair, who showed that it characteristically begins in the upper outer quadrant, proceeding clockwise in the right and counterclockwise in the left field, so that the upper nasal quadrant remains intact longest. The central defect behaves in the same way.5625
See also hemianopia, Traquair scotoma.
bizarre high-frequency discharge See complex repetitive discharge.
bizarre repetitive discharge See complex repetitive discharge.
bizarre repetitive potential See complex repetitive discharge.
114 BIOLOGICAL CLOCKS
Bjerrum, Jamik Peterson (1851–1926) Danish pro-fessor of ophthalmology in Copenhagen, who described arcuate scotomas in patients with glaucoma in 1890.
He also developed the tangent screen, which is still in general use, employing a cloth pinned to the door of his office.1829
Bjerrum screen (tangent screen) A dark felt cloth 1 meter square marked with a central point, radii, and concentric rings, set vertically in a darkened room, upon which the subject gazes while a lighted object is brought into the field of vision from the periphery as a test of the peripheral visual fields and the extent of the blind spot.
Björnstadt syndrome See pili torti.
black widow spider An arachid, the venom of which induces the rapid release of transmitter from nerve ter-minals, causing initial muscular contractions followed by paralysis. This may be fatal in young children and the frail elderly.
The spider (so called because the female eats her mate) is recognized by the characteristic red “hour-glass” pattern on her belly.
bladder disorders See automatic bladder, deaffer-ented bladder, hypertonic bladder, incontinence, motor paralytic bladder.
Blanchet syndrome Unilateral lumbar meningopoly-radiculoneuritis associated with inflammation of the lumbar sympathetic chain.599Further reports of this syndrome are not available and the nature of the con-dition is unknown.
blast (Gr, bud) a suffix denoting development.
blepharismus (from Gr, the eyelid) Winking.
blepharoclonus Repetitive blinking, sometimes heralding the later development of blepharospasm. See spasmus nictitans.
blepharocolysis (Gr, an eyelid inhibition) A supra-nuclear movement disorder characterized by a transient atonic inability to initiate eyelid opening in the absence of a definable lesion of the supranuclear or infranuclear oculomotor pathways, described most often in patients with pyramidal or extrapyramidal diseases. The disor-der is thus not actually an apraxia at all. See apraxia of lid opening.
blepharonasofacial syndrome A dominantly inher-ited dysmorphic syndrome characterized by stenosis of the lacrimal puncta, syndactyly, pyramidal and cere-bellar signs, dystonia, and mental retardation.4352 blepharophimosis A dominantly inherited condi-tion characterized by shortness and narrowing of the palpebral fissures, shortening of the upper eyelid (with deficiency of the levator palpebrae superioris also con-tributing to ptosis), hypoplasia of the orbicularis oculi, and flattening of the root of the nose.4
blepharophimosis syndrome A heterogeneous dom-inantly inherited, congenital, dysmorphic syndrome characterized by blepharophimosis and, variously, pto-sis, telecanthus, nystagmus, strabismus, reversible joint contractures, cleft palate, dolichocephaly, arachn-odactyly, skeletal and cerebellar dysgeneses, hypotonia, and mandibular hypoplasia.1877 See also Marden-Walker syndrome, Schwartz-Jampel syndrome.
Congenital blepharophimosis is a variant character-ized by general diminution of the palpebral fissure in all its dimensions but with the eyelids normally differ-entiated. Associated eye defects include nystagmus, mi-crophthalmos, anophthalmos, ptosis, epicanthus inver-sus, and hypermetropia. Facial dysmorphisms are also frequently associated. The condition may be domi-nantly inherited as uncomplicated blepharophimosis, in association with microphthalmos and anophthal-mos; with rigidity and dysfunction of the extraocular muscles, or as part of the Waardenburg syndrome.
The following criteria for the diagnosis of congeni-tal blepharophimosis have been suggested:5437
1. Diminution of the palpebral fissure in the range of 10–15 mm long and 2–4 mm wide on opening of the lids. The measurements remain constant throughout life.
2. The distance between the internal canthi often equals double the length of the palpebral fissure.
3. A flattened nasal bridge is present.
4. The aplasia of the palpebral levators and the tautness of the skin cause stiffness of the lids and obliteration of the tarsal folds.
5. The eyelid descends over the cornea and covers the pupil.
For improvement the head is usually tilted back and there is continued overaction of the occipitofrontalis muscle.
6. The eyelids are defectively developed with thin, smooth atrophic skin and rudimentary tarsal plates.
7. The eyelashes are underdeveloped and grow irregularly.
8. There is lateral displacement of the lachrymal puncta and elongation of the lateral.
9. The lack of contact within the bulb and the lids espe-cially nasally results in epiphera.
blepharoplegia (Gr, eyelid weakness) Ptosis due to paresis of the levator palpebrae.
blepharoptosis (Gr, eyelid a fall) Drooping of the upper eyelid. See ptosis.
blepharoschisis A malformation affecting the eyelids which may be a simple cleft up to complete absence (ablepharon).4See also coloboma.
blepharospasm (facial paraspasm, bilateral facial spasm, benign essential blepharospasm) Symmetrical, repetitive, involuntary, sustained, tight closure of the eyes as a result of involuntary contractions of the orbiculari oculi, in the absence of ocular pathol-ogy,2256,2808although the R2 component of the blink reflex is abnormally prolonged.
The condition represents a focal dystonic syndrome, most common in adult women. The muscular con-tractions are often increased by bright light and reduced BLEPHAROSPASM 115
by other voluntary activity, despite which they may be so intractable as to produce functional blindness.1649,2811 See also Meige syndrome.
Rating scales for blepharospasm have been con-structed.1729
In a variant form, patients with Parkinson disease or progressive supranuclear palsy are unable to initi-ate or sustain eye opening in the absence of overt spasm of the orbicularis muscles.1648
blepharospasm-oromandibular dystonia See Brue-ghel syndrome.
Blessed-Roth dementia instruments (Newcastle De-mentia Scale, Information-Memory-Concentration Test) A two-part measure designed to quantify the cognitive and behavioral symptoms of dementia, comparing these manifestations with measures of underlying brain neu-ropathology. Some of the elements have been included in the CAMDEX4814and the CERAD Instrument.3997
The Blessed Dementia Scale is a behavioral rating scale evaluating functional and emotional impairment and consisting of 22 items measuring changes in perform-ance of everyday activities, self-care habits, and per-sonality interests and drives. The Blessed Information-Memory-Concentration Test603 is a well-validated measure of cognitive impairment. Dr G. Blessed is a neuropsychologist in Newcastle, UK. See Information-Memory-Concentration Test.
blind headache A once-popular term for migraine.
blind spot A physiological defect in the visual fields due to the absence of rod and cone receptors on the head of the optic nerve. Its presence was first remarked in 1688 by E. Mariotte (1620–1684), a French physicist, who also described the red reflex.
blindisms (digito-ocular maneuver) The repetitive rubbing of the eyes, an action performed frequently by some people with congenital pigmentary retinal de-generations.
blindsight The capacity to detect (or at least make saccadic movements in the direction of) visual stimuli presented within the area of a field defect in the absence of acknowledged awareness. This may occur when the blindness results from damage to the striate cortex.5901 blink rate The frequency of involuntary repetitive blinking. In the normal subject this is 24 15 per minute.
The rate is decreased in depressive illnesses, Parkinson disease, and progressive supranuclear palsy.4851 blink reflex
1. Clinically, closure of the eyes with sudden visual stimu-lation or with tactile stimustimu-lation of the upper part of the face. The reflex was known to Descartes and to Jean Fer-nel (1506–1588). See also palpebral reflex, orbicularis oculi reflex.
2. In electromyography, compound muscle action potentials