Australian X disease See Murray Valley encephalitis
8. The plantar push
B cells Type B lymphocytes which are the precursors of immunoglobulin-producing cells.
B waves Spontaneous oscillations of intracranial pressure of between 10 and 50 mmHg, occurring at 0.5-to 2-minute intervals during sleep and in patients with periodic (Cheyne-Stokes) respirations.
Baastrup disease The presence of lumbar pain due to soft tissue disease affecting the interspinous liga-ments and adjacent soft tissues.280
Babcock sentence “One thing that a nation must have to become rich and great is a large, secure supply of wood.” The inability to repeat exactly this rather pedestrian economic statement after three presenta-tions was once considered a strong indication of im-paired short-term memory, but three-item recall se-quences are now more fashionable.
Babes nodules Collections of microglia in the nerv-ous system, found in in rabies.
Babin´ski, Josef-Francois-Felix (1857–1932) French neurologist of Polish descent who became chef de clin-ique under Charcot, following Pierre Marie and pre-ceding Gilles de la Tourette. He did not succeed Char-cot as professor at the Salpétrière for internal political reasons, but instead led the neurology service at the Pitié, an adjacent Paris hospital, from 1895. He re-mained a bachelor, living with his older brother Henri, a gourmet of distinction.
Babin´ski regarded his work on spinal cord compres-sion to be his best, but today his most lasting legacy is considered to be the sign bearing his name, which he de-scribed in 1896 (in 28 lines). He is also remembered for his definition of many cerebellar signs and for the intro-duction of the terms dysdiadochokinesis and asynergia;
for the platysma, reinforcement, trunk-thigh, and prona-tor signs; and for the Babinski-Frölich (adiposo-geni-tal) syndrome, which he described in 1900, a year before Fröhlich. The Babinski-Nageotte and Anton-Babinski (anosognosia) syndromes also bear witness to his aston-ishing clinical perspicacity. It is also remembered that he pointed out that the hysterical symptoms of many pa-tients at the Salpétrière died with Charcot. He was an early proponent of the use of the reflex hammer.
Babinski attracted many important neurologists from abroad to his service, but his failure to gain ap-pointment at the Salpétrière reduced his influence within France, although he was a founder of the Société de Neurologie, edited the Revue Neurologique, and was elected to the Parisian Academy of Medicine in 1914.
In the end, dispassionately, from abroad, he was prop-erly regarded as the most outstanding of all the pupils of Charcot.2750Like Henry Head, he died from com-plications of Parkinson disease.
Babinski signs 1. Described by Babinski as “the phe-nomenon of the toes,” the sign consists of the upward (dorsiflexion or extension) movement of the hallux with uncomfortable or painful stimulation of the sole of the foot, while the normal response would be for the toes to move downwards. As he noted in his original283 or in later papers published between 1896 and 1903, flexion at the hip and knee, ankle dorsiflexion, and fan-ning of the toes (“signe de l’éventail”) may also occur.
The sign is present (upgoing toe) in the presence of a disturbance in the pyramidal system, regardless of its duration, intensity, or extent, and it represents a part of the flexion reflex in humans.
Marshall Hall, Vulpian, and Strumpell had previ-ously noted this phenomenon and Remak had de-scribed extension of the hallux on plantar stimulation in transverse myelitis 3 years before, but Babinski in-vestigated the phenomenon in depth, principally in an attempt to distinguish organic from hysterical weak-ness, and deserves the eponym. Some reports show him using a feather, but his words indicate that he elicited the sign by pricking the skin of the sole.283
Numerous other methods of eliciting the sign (Chaddock, Bing, Oppenheim, Gordon, Thomas, Stransky, etc.) have also been described, and are com-plementary but rather less useful.5729
2. Diminution or absence of the Achilles reflex in sciatica. Babinski introduced the preferred method of examining the reflex, with the patient kneeling.
3. Fanning of the toes on the side on which there is clinical evidence of a pyramidal lesion, when the supine patient attempts to sit up.
4. Greater contraction of the platysma on the healthy than on the affected side in hemiparesis, tested by getting the patient to open the mouth wide or to try to whistle.
5. (pronator sign) Increased pronation of the paretic arm in hemiparetic states, such that when the examiner places the patient’s hands in a position of supination, only the hand on the side affected returns to the pronated position.
6. Pupillary changes with aortic aneurysms.284See Horner syndrome.
7. Paradoxical raising of the eyebrow on the side of hemifacial spasm as a result of contraction of the frontalis muscle as well as the orbicularis oculi.286
• Only the normal leg is flexed in hysterical hemiparesis.
• Neither leg is flexed in hysterical paraparesis.
Thus an unusual situation obtains, namely that the re-tention of some movement denotes the presence of or-ganic disease.
Babinski-Fröhlich syndrome (pituitary tumor without acromegaly but with genital arrest) See Fröh-lich syndrome.
Babinski-Froment syndrome Post-traumatic paral-ysis with hypotonia and hyporeflexia but with excessive mechanical excitability. A syndrome of wartime, with an uncertain basis.288
Babinski-Nageotte syndrome (medullary tegmental paralysis, combined lateral and medial medullary syn-drome) A paramedian pontomedullary lesion causing homolateral Horner syndrome; ipsilateral weakness of the soft palate, pharynx, larynx, and tongue; loss of taste of the posterior third of the tongue; cerebellar ataxia with nystagmus; and contralateral hemiparesis and hemianesthesia.289
The Cestan-Chenais syndrome is almost identical.
Babinski-Weil test The blindfolded patient with a vestibular disorder, when asked to take 10 steps forward and 10 back, deviates to the right while walking forward and to the left going back, or vice versa. This gait pat-tern has been termed star walking.
Babkin reflex (hand–mouth reflex) Opening of the mouth and flexion of the forearm and the neck when the palm of a normal infant is pressed. The reflex is nor-mally present until the fourth month of life.
backaveraging Averaging a signal that occurs in a time epoch preceding a triggering event. It is often used to extract a time-locked EEG signal preceding volun-tary or involunvolun-tary movement, usually triggered by the onset of the EMG activity of the movement. An exam-ple is the Bereitschaftspotential. (From the 2001 Re-port of the Nomenclature Committee of the American Association of Electromyography and Electrodiagno-sis.10Used by permission, copyright © 2001, AAEM.) backfiring The discharge of an antidromically acti-vated motor neuron.
background activity 1. Any EEG activity represent-ing the settrepresent-ing in which a given normal or abnormal pattern appears and from which such pattern is distin-guished. This is not, however, a synonym for any indi-vidual rhythm such as the alpha rhythm. (Adapted from the report of the Committee on Terminology, IFCN,989
© 1974, with permission from Elsevier Science.) 2. In electromyography, no background activity is normally recorded unless the needle tip is close to a motor end plate. See end-plate noise.
back-handing The process of arising from the squat-ting position with the aid of the hands placed on the floor
beside and behind the feet to give a push upward; a sign of mild pelvic girdle weakness. See also Gowers signs.
back-kneeing Locking of the knee in hyperextension, a posture adopted by patients with quadriceps weak-ness in order to prevent sudden flexion at the joint while walking or standing.
back-up saccades See catch-up saccades.
Badal, Jules (1840–1929) French neuro-ophthalmol-ogist, the first holder of a chair in that subject in France (at Bordeaux). He was at first a soldier, being decorated in the field during the Franco-Prussian War, but left the army for medicine. In 1888 he published a de-scription of his patient Valerie, who developed a posteclamptic state of visual disorientation with fea-tures of what is now known as Gerstmann syndrome.
See also posterior leukoencephalopathy.
Badal-Gerstmann syndrome See Gerstmann syn-dromes.
BADS syndrome The association of a loss of the Bell phenomenon with corneal anesthesia and a dry eye in a patient with seventh cranial nerve palsy, leading to traumatic corneal damage.
Baer nystagmus See reflex sensory nystagmus.
Baetz-Greenwalt syndrome A congenital dysmor-phic syndrome characterized also by microcephaly, holoprosencephaly, or arhinencephaly and by congen-ital cardiac anomalies.337
bag fibers Intrafusal muscle fibers of two types, one reacting to the velocity and acceleration of stretch and the other to the acceleration of the stretching force.
Bailey, Pearce (1902–1976) American neurologist, who trained in medicine at Princeton and Columbia, receiving also a Ph.D. in psychology from the Sorbonne, before training in neurology at Bellevue Hospital in New York. His later clinical post was as professor at Georgetown University. He was the first director of the National Institute of Neurological and Communicative Disorders and Stroke, where he organized an intensive program of research into seizure disorders, multiple sclerosis, muscular disease, perinatal disease, and dis-eases of the eye and ear, training and assisting the ca-reers of many now-prominent scholar–neurologists.
Bailey was a founder of the World Federation of Neurology and both nationally and internationally was prominent in the organization of associations dedicated to the furtherance of social, research, or clinical goals within the neurological sciences. The Pearce Bailey Medal and Prize of the Epilepsy Foundation of Amer-ica were created in his honor.
Bailey, Perceval (1892–1973) American neurosur-geon, anatomist, and scholar who trained in medicine at Chicago, and Northwestern Universities and then studied with Cushing at Harvard, in Chicago and in 88 BABINSKI-FRÖHLICH SYNDROME
Paris. Returning to Harvard, he embarked upon a study of cerebral tumors, which culminated in his classifica-tion of gliomas, completed while working under Cush-ing, who, in the tradition of nineteenth century Euro-pean schools, published it himself in 1926. Bailey continued to collaborate with Cushing on tumor clas-sification but returned to the University of Chicago in 1928 where he became head of the Department of Neu-rology and Neurosurgery. His major work was In-tracranial Tumors of Infancy and Childhood (1939), published in collaboration. He transferred to the Uni-versity of Illinois in 1940, where he continued his work until retirement.7
Bailey-Cushing tumor See medulloblastoma.
Baillarger, Jules-Gabriel-Francois (1809–1890) An outstanding figure in French neurology and psychiatry in the nineteenth century. He worked at the Salpétrière from 1840, where he described the cycles of manic-depressive illness and melancholic stupor and wrote ex-tensively on hallucinations, syphilis, and brain anatomy until his retirement in 1870.297,298
Baillarger syndrome See Frey syndrome.
Baillarger-Jackson principle The ability of aphasic patients to enunciate words involuntarily that they can-not utter at will.68
The phenomenon puts one in mind of the words of Dr. Samuel Johnson: “Things too silly to be said may yet be sung.”
Baillie, Matthew (1761–1823) Scottish morbid anatomist who gave the first accounts of cerebral pathology.
Bainbridge, Francis Arthur (1874–1921) English physiologist who was professor of physiology succes-sively at Durham and at St. Bartholemew’s Hospital in London.1750His best-remembered work was that show-ing that cardiac acceleration occurs as a result of inhi-bition of vagal tone, as by an increase in blood pressure or distension of the right atrium.
Bainbridge reflex Increased sympathetic activity with tachycardia in response to an increase in venous and in right atrial pressure, due to vagal inhibition.
Bajonet posture Hyperpronation and hyperflexion of the hands and feet with permanent dorsiflexion of the hallux (pseudo-Babinski sign), seen in some basal ganglion disorders such as the later stages of parkin-sonism and pallidal atrophies.
balance The maintenance of the upright posture dur-ing sittdur-ing, standdur-ing, and locomotion through the de-tection and correction of displacement of the line of gravity beyond the base of support.
balanced amplifier An amplifier that consists of two identical single-ended amplifiers operated as a pair but in opposite phases.
Balci oculocerebral syndrome A congenital dys-morphic syndrome characterized by cloudy corneae or sclerocornea, microphthalmia, mental and develop-mental delay, seizures, microcephaly, and spasticity with aminoaciduria.337
balderdash syndrome A colloquial term for Ganser syndrome.
Balduzzi reflex Adduction and inward rotation of the contralateral leg in response to the vibration set up by a tap on the sole of the foot, present in normal sub-jects but increased in patients with pyramidal tract dis-ease.
The sign was described in 1926 by Ottorino Balduzzi, an Italian neurologist at the University of Rome.305 Balint, Rudolf (1874–1929) Hungarian neurologist, professor at the University of Budapest.
Balint syndrome 1. (Balint-Holmes syndrome, psy-chic paralysis of visual fixation, cortical paralysis of vi-sual fixation) A rare syndrome resulting from bilateral parietooccipital disease and characterized by inability to direct the eyes to a certain point in the visual field BALINT SYNDROME 89
Jules-Gabriel-Francois Baillarger
Baillarger lines The bands of fibers visible to the naked eye in sections of the occipital cortex. The striae Gennarii are such laminations. Six layers were descried by Baillarger, a finding later confirmed histologically by Remak.
Baillarger sign Unilateral dilatation of the pupil in tertiary syphilis, for which Baillarger could adduce no good explanation.297
despite the retention of intact eye movements (ocular apraxia, psychic paralysis of gaze); optic ataxia, a dis-order of visual reaching; inability to read; impaired depth perception and fluctuating visual inattention for any stimulus not exciting the macula (visual disorien-tation or simultagnosia.)3008
Balint’s patient had left-sided visual inattention but inability to perform directed movements with the right hand (his left handed movements were faultless), sug-gesting a disconnection syndrome. Fragments of re-ceptive aphasia, agraphia, and ideomotor apraxia may coexist.308,5671
2. Loss of all voluntary extraocular movements and pupillary paralysis with retention of reflex eye move-ments, seen in hyperthyroidism.5047
Balint-Holmes syndrome See Balint syndrome.
balking The increasingly angry refusal of demented patients to cooperate in the routine procedures of daily care.
Baller-Gerold syndrome (craniosynostosis and ra-dial aplasia) A recessively inherited congenital syn-drome characterized by craniosynostosis affecting the coronal suture, and by short stature, radial aplasia, and absent fingers.309
ballet dancer’s foot A focal occupational dystonia af-fecting the foot muscles.5335
Ballet, Gilbert (1853–1916) French neurologist and psychiatrist who trained in Limoges and Paris, became chef de clinique to Charcot, rising through the ranks to professor of the history of medicine and then of men-tal diseases in 1908.
Ballet sign Retention of the pupillary reflexes despite external ophthalmoplegia in dysthyroid ophthal-mopathy.
Ballet syndrome External ophthalmoplegia with paresis of the muscles supplied by the fifth, seventh, and twelfth cranial nerves and by the vagus in patients with hyperthyroidism.310
ballism (ballismus) (from Gr, to dance) Extensive, vigorous, fast, poorly patterned, nonadaptive move-ments of the facial, limb, or truncal muscles. These vi-olent, flinging, involuntary movements are due to le-sions of the subthalamic nucleus of Luys, most often following a lacunar stroke.
Hemiballism is the term applied when the move-ments are unilateral, as is more common. Paraballism denotes bilateral ballism.
ballismic cerebral palsy An ill-defined form of cere-bral palsy characterized by uni- or bilateral ballism af-fecting the arms or legs.
ballistic Not subject to modification after initiation.
ballistic movement overflow myoclonus A form of essential myoclonus, characterized by involuntary
movements occurring in 50- to 100-msec bursts of ac-tivity in the agonist muscle, accelerating a limb, fol-lowed by the same in the antagonist muscles (slowing it down) and then by further brief contraction in the agonist. The phenomenon has been described in pa-tients with familial essential myoclonus.
This pattern differs from that occurring in reticular and in cortical loop myoclonus, in which the duration of the burst is only 10–30 msec.2387
ballismus See ballism.
balloon cells Grossly distended neurons with dis-placement of the nucleus and Nissl substance to the pe-riphery, as in the gangliosidoses, in which the unde-graded gangliosides distend neuronal cell bodies.
Balo concentric sclerosis (encephalitis periaxialis concentrica) A rare subacute, progressive dysmyelinat-ing disease, perhaps a variant of multiple sclerosis, de-scribed by Balo in Hungary in 1927. Bands of intact myelin alternating with zones of demyelination are found in the white matter, especially of the frontal lobes, varying in size from 1 to 3 cm and arranged in irregular concentric rings, witnessing the patchy de-struction of myelin with reformation at the edge of each large plaque and thus explaining the laminated ap-pearance. The axons are intact within these foci.311
The clinical features include progressive spastic paralysis and other widely varying evidence of diffuse cerebral disease, with childhood onset.
Balo, Jozsef Matyas (1895–1979) Hungarian pathol-ogist who trained and worked at the University of Bu-dapest where he became chairman of the department of pathology and, for a period, dean of the medical school. He reported his case of concentric sclerosis in 1926.
Baltic myoclonus (Nordic myoclonus, Unverricht-Lundborg-Lafora syndrome) A recessively inherited form of progressive myoclonic epilepsy. The onset of the myoclonus and tonic-clonic seizures is between 8 and 13 years, while such neurological signs as gradual intellectual failure, dysarthria, ataxia, and intention tremor appear later. Absence and drop attacks some-times occur. The myoclonus may be precipitated by movement, stress, and sensory stimuli and repetitive morning myoclonus is common. Survival into adult life is usual. The gene locus has been mapped to chromo-some 21. The diagnostic feature of Lafora body disease is the PAS-positive inclusion body found in neurons throughout the gray matter of the brain, including the dentate nucleus of the cerebellum, and on biopsy of axillary skin, liver, or skeletal muscle.
The syndrome was first reported by Unverricht in 1891 and appears to occur mainly in the populations of eastern Sweden and Finland, determining the name.5689There is suggestive evidence that dyssyner-gia cerebellaris myoclonica is the same condition as 90 BALINT-HOLMES SYNDROME
this disease. See also Unverricht-Lundborg-Lafora syndrome (which differs only in a slightly older onset age and earlier onset of intellectual impairment).
Bamberger disease (palmus) The sudden onset of clonic spasms of the legs, producing a jumping or springing motion. It was sometimes a manifestation of hysteria, sometimes of psychosis. Guttmann (1867), Frey (1875), and Gowers2240 also wrote on the same condition, which they thought in at least some cases was organic. Bamberger also described allocheiria.
The author has seen one possible case but knows of no other report in the last century.2238See saltatory spasm.
Bamberger, H von (1822–1888) German physician who trained in Prague and Vienna, and later became professor of pathology first at Wurzburg University and then at Vienna. In 1872 he described the findings now regarded as typical of Pick disease, as well as the dis-ease entered above.
band A portion of the frequency spectrum of the EEG, including the delta, theta, alpha, and beta bands.
band heterotopia A form of gray matter heterotopia in which there is premature arrest of gray matter mi-gration resulting in a symmetric band of subcortical gray matter, referred to as the “double cortex sign.
There may be some associated cortical pachygyria. Clin-ically, patients manifest an age-related epileptogenic encephalopathy with multiple types of generalized seizures and progressive cognitive decline. Association with the Lennox-Gastaut syndrome has been re-ported.353See diffuse cortical dysplasia.
band keratopathy Horizontal ribbons of calcium deposition in the cornea and conjunctiva, occurring in hypercalcemic states.
bands of Büngner Chains of multiplying Schwann cells which act as templates for the regenerating axons following Wallerian degeneration.
bandwidth (EEG channel) The range of frequencies between which the response of an EEG channel is within stated limits. This is determined by the fre-quency response of the amplifier–writer combination and the frequency filters used.
The manner in which the EEG channel bandwidth is specified by different manufacturers is not standardized at present. For instance, in a given instrument, a band-width of 0.5–50 Hz may indicate that frequencies of 0.5 and 50 Hz are attenuated 30° (3 dB) or another stated percent, with intermediate frequencies being attenu-ated less. (Adapted from the report of the Committee on Terminology, IFCN989, © 1974, with permission from Elsevier Science).
Bannayan-Zonana syndrome A congenital dysmor-phic syndrome characterized by mental and develop-mental delay, macrocephaly, unusual frequency of
brain tumors such as capillary or cavernous heman-giomas, subcutaneous lipomas, and musculoskeletal maldevelopment.3872
Bannwarth syndrome (lymphocytic meningoradi-culitis) Subacute or chronic lymphocytic meningitis due to tick-borne infection with Borrelia burgdorferi and presenting with severe radicular pain and sensory and motor signs associated with cranial nerve palsies, especially facial paralysis.331 See Lyme disease (of which this is the European version).
Baraitser syndrome A craniofacial malformation syndrome characterized by cleft lip or palate, choroidal colobomas, seizures, mental retardation, short broad digits, and renal dysplasia.338
Bárány positional vertigo see benign positional vertigo.
BÁRÁNY, ROBERT 91
Robert Bárány
Bárány, Robert (1876–1936) Hungarian otologist born in Vienna who studied medicine, psychiatry, and neurology in Austria and Germany. He was awarded the Nobel prize in 1914 for his work on otovestibular physiology and medicine. Although originally working in Vienna, he was captured by the Russian forces while a physician in the Austro-Hungarian army during the World War I (although he was himself a pacifist) and was interned as a prisoner, being released by the Czar to work in Uppsala at the behest of Prince Carl of Sweden.
His publications on the genesis of vertigo and on caloric testing have not been disputed; he first noted that nystagmus followed aural lavage, and that the di-rection of the fast component was away from the cooled