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Patients with depression may also complain of impaired sense of smell. Loss of olfactory acuity may be a feature of normal ageing.
The neurology of olfaction. Cross References Age-related signs; Ageusia; Cacosmia; Dysgeusia; Mirror movements; Parosmia Anosodiaphoria Babinski used the term anosodiaphoria to describe a disorder of body schema in which patients verbally acknowledge a clinical problem e.
Anosodiaphoria usually follows a stage of anosognosia. References Babinski JM. Observations on anosodiaphoria. Some authorities would question whether this unawareness is a true agnosia or rather a defect of higher-level cognitive integration i.
Anosognosia with hemiplegia most commonly follows right hemisphere injury parietal and temporal lobes and may be associated with left hemineglect and left-sided hemianopia; it is also described with right thalamic and basal ganglia lesions.
Many patients with posterior aphasia Wernicke type are unaware that their output is incomprehensible or jargon, possibly through a failure to monitor their own output.
The neuropsychological mechanisms of anosognosia are unclear: the hypothesis that it might be accounted for by personal neglect asomatognosia , which is also more frequently observed after right hemisphere lesions, would seem to have been disproved experimentally by studies using selective hemisphere anaesthesia in which the two may be dissociated, a dissociation which may also be observed clinically.
Temporary resolution of anosognosia has been reported following vestibular stimulation e. Babinski JM.
Hemianopic anosognosia. Unawareness of deficits in neuropsychological syndromes. Morris RG, Hannesdottir K. Anosognosia in patients with cerebrovascular lesions: a study of causative factors.
Loss of anserina may be a feature of some autonomic disorders. Antecollis Antecollis anterocollis is forward flexion of the neck.
It may be a feature of multiple system atrophy cf. Reference Quinn N. Disproportionate antecollis in multiple system atrophy. Lancet ; 1: The syndrome most usually results from bilateral posterior cerebral artery territory lesions causing occipital or occipitoparietal infarctions but has occasionally been described with anterior visual pathway lesions associated with frontal lobe lesions.
It may also occur in the context of dementing disorders or delirium. Gassel M, Williams D. Visual function in patients with homonymous hemianopia.
The completion phenomenon: insight and attitude to the defect: and visual function efficiency. Cross References Agnosia, Anosognosia, Confabulation, Cortical blindness Anwesenheit A vivid sensation of the presence of somebody either somewhere in the room or behind the patient has been labelled as anwesenheit German: presence , presence hallucination, minor hallucination, or extracampine hallucination.
Hence, listlessness, paucity of spontaneous movement akinesia or speech mutism , and lack of initiative, spontaneity, and drive may be features of apathy These are also all features of the abulic state, and it has been suggested that apathy and abulia represent different points on a continuum of motivational and emotional deficit, abulia being at the more severe end.
Apathy is a specific neuropsychiatric syndrome, distinct from depression. Apathy is also described following amphetamine or cocaine withdrawal, in neuroleptic-induced akinesia and in psychotic depression.
Selective serotonin-reuptake inhibitors may sometimes be helpful in the treatment of apathy. Apathy is not depression. Marin RS.
Differential diagnosis and classification of apathy. Cross References Abulia; Akinetic mutism; Dementia; Frontal lobe syndromes Aphasia Aphasia, or dysphasia, is an acquired loss or impairment of language function.
Non-linguistic components of language emotion, inflection, cadence , collectively known as prosody, may require contributions from both hemispheres.
Language is distinguished from speech oral communication , disorders of which are termed dysarthria or anarthria.
Dysarthria and aphasia may coexist but are usually separable. Comprehension: spared or impaired? Repetition: preserved or impaired?
Naming: preserved or impaired? Reading: evidence of alexia? Writing: evidence of agraphia? Conduction aphasia is marked by relatively normal spontaneous speech perhaps with some paraphasic errors , but a profound deficit of repetition.
In transcortical motor aphasia spontaneous output is impaired but repetition is intact. Aphasia may also occur with space-occupying lesions and in neurodegenerative disorders, often with other cognitive impairments e.
References Basso A. Aphasia and its therapy. Oxford: Oxford University Press, Benson DF, Ardila A.
Caplan D. Aphasic syndromes. Approach to the patient with aphasia. Spreen O, Risser AH. Assessment of aphasia. Willmes K, Poeck K.
To what extent can aphasic syndromes be localized? The term is now used to describe a motor disorder of speech production with preserved comprehension of spoken and written language.
This syndrome has also been called phonetic disintegration cf. Such conditions may stand between pure disorders of speech i.
Aphemia: an isolated disorder of articulation. Henderson VW. Alalia, aphemia, and aphasia. Aphemia: clinicalanatomic correlations.
Cross References Anarthria; Aphasia; Aprosodia, Aprosody; Dysarthria; Phonemic disintegration; Speech apraxia Aphonia Aphonia is loss of the sound of the voice, necessitating mouthing or whispering of words.
As for dysphonia, this most frequently follows laryngeal inflammation, although it may follow bilateral recurrent laryngeal nerve palsy.
Dystonia of the abductor muscles of the larynx can result in aphonic segments of speech spasmodic aphonia or abductor laryngeal dystonia ; this may be diagnosed by - 37 - A Applause Sign hearing the voice fade away to nothing when asking the patient to keep talking; patients may comment that they cannot hold any prolonged conversation.
Aphonia of functional or hysterical origin is also recognized. Aphonia should be differentiated from mutism, in which patients make no effort to speak, and anarthria in which there is a failure of articulation.
Cross References Anarthria; Dysphonia; Mutism Applause Sign To elicit the applause sign, also known as the clapping test or three clap test, the patient is asked to clap the hands three times.
Diagnostic accuracy of the clapping test in Parkinsonian disorders. Aposiopesis Critchely used this term to denote a sentence which is started but not finished, as in the aphasia associated with dementia.
Reference Critchley M. The divine banquet of the brain and other essays. Cross Reference Aphasia Apraxia Apraxia or dyspraxia is a disorder of movement characterized by the inability to perform a voluntary motor act despite an intact motor system i.
Ideomotor apraxia IMA : A disturbance in the selection of elements that constitute a movement e.
This may be associated with the presence of a grasp reflex and alien limb phenomena limb-kinetic type of apraxia. Apraxia is more common and severe with left hemisphere lesions.
Difficulties with the clinical definition of apraxia persist, as for the agnosias. Likewise, some cases labelled as eyelid apraxia or gait apraxia are not true ideational apraxias.
The exact nosological status of speech apraxia also remains tendentious. References Binkofski F, Reetz K.
Cognitive neurology: a clinical textbook. Crutch S. Advances in Clinical Neuroscience and Rehabilitation ; 5 1 : 16, Grafton S.
Apraxia: a disorder of motor control. Limb apraxias. Higher-order disorders of sensorimotor integration. Liepmann H. Berlin: Karger, Cross References Alien hand, Alien limb; Body part as object; Crossed apraxia; Dysdiadochokinesia; Eyelid apraxia; Forced groping; Frontal lobe syndromes; Gait apraxia; Grasp reflex; Optic ataxia; Speech apraxia - 39 - A Aprosexia Aprosexia Aprosexia is a syndrome of psychomotor inefficiency, characterized by complaints of easy forgetting, for example, of conversations as soon as they are finished, material just read, or instructions just given.
There is difficulty keeping the mind on a specific task, which is forgotten if the patient happens to be distracted by another task.
These difficulties, into which the patient has insight and often bitterly complains of, are commonly encountered in the memory clinic.
They probably represent a disturbance of attention or concentration, rather than being a harbinger of dementia. These patients generally achieve normal scores on formal psychometric tests and indeed may complain that these assessments do not test the function they are having difficulty with.
Concurrent sleep disturbance, irritability, and low mood are common and may reflect an underlying affective disorder anxiety, depression which may merit specific treatment.
Cross References Attention; Dementia Aprosodia, Aprosody Aprosodia or aprosody dysprosodia, dysprosody is a defect in or absence of the ability to produce or comprehend speech melody, intonation, cadence, rhythm, and accentuations, in other words the non-linguistic aspects of language which convey or imply emotion and attitude.
Progressive affective aprosodia and prosoplegia. Monrad-Krohn GH. Ross ED. The aprosodias: functional-anatomic organization of the affective components of language in the right hemisphere.
Cross References Retinopathy; Scotoma Areflexia Areflexia is an absence or a loss of tendon reflexes.
This may be physiological, in that some individuals never demonstrate tendon reflexes; or pathological, reflecting an anatomical interruption or physiological dysfunction at any point along the monosynaptic reflex pathway which is the neuroanatomical substrate of phasic stretch reflexes.
Areflexia is most often encountered in disorders of lower motor neurones, specifically radiculopathies, plexopathies, and neuropathies axonal and demyelinating.
It fails to react to light reflex iridoplegia , but does constrict to accommodation when the eyes converge.
Since the light reflex is lost, testing for the accommodation reaction may be performed with the pupil directly illuminated: this can make it easier to see the response to accommodation, which is often difficult to observe when the pupil is small or in individuals with a dark iris.
There may be an incomplete response to mydriatic drugs. Although pupil involvement is usually bilateral, it is often asymmetric, causing anisocoria.
The Argyll Robertson pupil was originally described in the context of neurosyphilis, especially tabes dorsalis.
The neuroanatomical substrate of the Argyll Robertson pupil is uncertain. A lesion in the tectum of the rostral midbrain proximal to the oculomotor nuclei has been suggested.
Some authorities think that a partial oculomotor III nerve palsy or a lesion of the ciliary ganglion is a more likely cause.
References Argyll Robertson D. Four cases of spinal myosis [sic]: with remarks on the action of light on the pupil.
The Argyll Robertson pupil. Significance of the Argyll Robertson pupil in clinical medicine. It is said that in organic weakness the hand will hit the face, whereas patients with functional weakness avoid this consequence.
Functional weakness and sensory disturbance. The term was invented in the nineteenth century Hamilton as an alternative to aphasia, since in many cases of the latter there is more than a loss of speech, including impaired pantomime apraxia and in symbolizing the relationships of things.
Hughlings Jackson approved of the term but feared it was too late to displace the word aphasia. Father of English neurology.
Cross References Aphasia, Apraxia Asomatognosia Asomatognosia is a lack of regard for a part, or parts, of the body, most typically failure to acknowledge the existence of a hemiplegic left arm.
Asomatognosia may be verbal denial of limb ownership or non-verbal failure to dress or wash limb. All patients with asomatognosia have hemispatial neglect usually left , hence this would seem to be a precondition for the development of asomatognosia; indeed, for some authorities asomatognosia is synonymous with personal neglect.
Attribution of the neglected limb to another person is known as somatoparaphrenia. The neuroanatomical correlate of asomatognosia is damage to the right supramarginal gyrus and posterior corona radiata, most commonly due to a cerebrovascular event.
Cases with right thalamic lesions have also been reported. The predilection of asomatognosia for the left side of the body may simply be a reflection of the aphasic problems associated with leftsided lesions that might be expected to produce asomatognosia for the right side.
Asomatognosia is related to anosognosia unawareness or denial of illness but the two are dissociable on clinical and experimental grounds.
Some authorities consider asomatognosia as a form of confabulation. The neuroanatomy of asomatognosia and somatoparaphrenia.
The term has no standardized definition and hence may mean different things to different observers; it has also been used to describe a disorder characterized by inability to stand or walk despite normal leg strength when lying or sitting, believed to be psychogenic although gait apraxia may have similar features.
A transient inability to sit or stand despite normal limb strength may be seen after an acute thalamic lesion thalamic astasia.
Thalamic astasia: inability to stand after unilateral thalamic lesions. Human walking and higher-level gait disorders, particularly in the elderly.
Cross Reference Gait apraxia Astereognosis Astereognosis is the failure to recognize a familiar object, such as a key or a coin, palpated in the hand with the eyes closed, despite intact primary sensory modalities.
Description of qualities such as the size, shape, and texture of the object may be possible. Hence, this is a failure of higher-order i.
There may be associated impairments of two-point discrimination and graphaesthesia cortical sensory syndrome. Astereognosis was said to be invariably present in the original description of the thalamic syndrome by Dejerine and Roussy.
Some authorities recommend the terms stereoanaesthesia or stereohypaesthesia as more appropriate descriptors of this phenomenon, to emphasize that this may be a disorder of perception rather than a true agnosia for a similar debate in the visual domain, see Dysmorphopsia.
Cross References Agnosia; Dysmorphopsia; Graphaesthesia; Two-point discrimination Asterixis Asterixis is a sudden, brief, arrhythmic lapse of sustained posture due to involuntary interruption in muscle contraction.
It is most easily demonstrated by observing the dorsiflexed hands with arms outstretched i. These features distinguish asterixis from tremor and myoclonus; the phenomenon has previously been described as negative myoclonus or negative tremor.
Asterixis may be bilateral or unilateral. Unilateral asterixis has been described in the context of stroke, contralateral to lesions of the midbrain involving corticospinal fibres, medial lemniscus , thalamus ventroposterolateral nucleus , primary motor cortex, and parietal lobe; and ipsilateral to lesions of the pons or medulla.
Unilateral asterixis and stroke in 13 patients: localization of the lesions matching the CT scan images to an atlas.
European Journal of Neurology ; 11 suppl 2 : 56 abstract P Unilateral asterixis: motor integrative dysfunction in focal vascular disease.
Such refractive errors are sometimes blamed for headache. Asynergia Asynergia or dyssynergia is lack or impairment of synergy of sequential muscular contraction in the performance of complex movements, such that they seem to become broken up into their constituent parts, so-called decomposition of movement.
This may be evident when performing rapid alternating hand movements. Dyssynergy of speech may also occur, a phenomenon sometimes termed scanning speech or scanning dysarthria.
This is typically seen in cerebellar syndromes, most often those affecting the cerebellar hemispheres, and may coexist with other signs of cerebellar disease such as ataxia, dysmetria, and dysdiadochokinesia.
Cross References Ataxia; Cerebellar syndromes; Dysarthria; Dysdiadochokinesia; Dysmetria; Scanning speech Ataxia Ataxia or dystaxia refers to a lack of coordination of voluntary motor acts, impairing their smooth performance.
The rate, range, timing, direction, and force of movement may be affected. Ataxia is used most frequently to refer to a cerebellar problem, but sensory ataxia, optic ataxia, and frontal ataxia are also described, so it is probably best to qualify ataxia rather than to use the word in isolation.
There may be concurrent limb hypotonia. An International Cooperative Ataxia Rating Scale has been developed to assess the efficacy of treatments for cerebellar ataxia.
It is markedly exacerbated by removal of visual cues e. These fibres run in the corticopontocerebellar tract, synapsing in the pons before passing through the middle cerebellar peduncle to the contralateral cerebellar hemisphere.
References Klockgether T. Sporadic ataxia with adult onset: classification and diagnostic criteria. International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome.
Wardle M, Robertson N. Progressive late-onset cerebellar ataxia. There may be additional dysarthria, nystagmus, paraesthesia, and pain.
This syndrome is caused by lacunar small deep infarcts in the contralateral basis pons at the junction of the upper third and lower two-thirds.
It may also be seen with infarcts in the contralateral thalamocapsular region, posterior limb of the internal capsule anterior choroidal artery syndrome , red nucleus, and the paracentral region anterior cerebral artery territory.
Sensory loss is an indicator of capsular involvement; pain in the absence of other sensory features is an indicator of thalamic involvement.
Painful ataxic hemiparesis. Ataxic hemiparesis. A pathologic study. Ataxic hemiparesis: critical appraisal of a lacunar syndrome.
Athetosis often coexists with the more flowing, dance-like movements of chorea, in which case the movement disorder may be described as choreoathetosis.
Athetoid-like movements of the outstretched hands may also been seen in the presence of sensory ataxia impaired proprioception and are known as pseudoathetosis or pseudochoreoathetosis.
Choreoathetoid movements result from disorders of the basal ganglia. Athetosis I: historical considerations. Athetosis or dystonia? Cross References Chorea, Choreoathetosis; Pseudoathetosis; Pseudochoreoathetosis Athymhormia Athym h ormia, also known as the robot syndrome, is a name given to a form of abulia or akinetic mutism in which there is loss of self-autoactivation.
Clinically there is a marked discrepancy between heteroactivation, behaviour under the influence of exogenous stimulation, which is normal or almost normal, and autoactivation.
Left alone, patients are akinetic and mute, a state also known as loss of psychic self-activation or pure psychic akinesia.
It is associated with bilateral deep lesions of the frontal white matter or of the basal ganglia, especially the globus pallidus.
Athymhormia is thus environment-dependent, patients normalizing initiation and cognition when stimulated, an important differentiation from apathy and akinetic mutism.
Isolated athymhormia following hypoxic bilateral pallidal lesions. The term is often applied to wasted muscles, usually in the context of lower motor neurone pathology in which case it may be synonymous with amyotrophy , but also with disuse.
Atrophy develops more quickly after lower, as opposed to upper, motor neurone lesions. It may also be applied to other tissues, such as subcutaneous tissue as in hemifacial atrophy.
Atrophy may sometimes be remote from the affected part of the neuraxis, hence a false-localizing sign, for example, wasting of intrinsic hand muscles with foramen magnum lesions.
Reference Larner AJ. Cross Reference Dementia Attention Attention is a distributed cognitive function, important for the operation of many other cognitive domains; the terms concentration, vigilance, and persistence may be used synonymously with attention.
It is generally accepted that attention is effortful, selective, and closely linked to intention. Impairment of attentional mechanisms may lead to distractability with a resulting complaint of poor memory, perhaps better termed aprosexia , disorientation in time and place, perceptual problems, and behavioural problems e.
The neuroanatomical substrates of attention encompass the ascending reticular activating system of the brainstem, the thalamus, and the prefrontal multimodal association cerebral cortex especially on the right.
Damage to any of these areas may cause impaired attention. In the presence of severe attentional disorder as in delirium it is difficult to make any meaningful assessment of other cognitive domains e.
References Parasuraman R. Scholey A. Neurochemistry of consciousness: neurotransmitters in mind. Cross References Aprosexia; Delirium; Dementia; Disinhibition; Dysexecutive syndrome; Frontal lobe syndromes; Pseudodementia Auditory Agnosia Auditory agnosia refers to an inability to appreciate the meaning of sounds despite normal perception of pure tones as assessed by audiological examination.
This agnosia may be for either verbal material pure word deafness or nonverbal material, either sounds bells, whistles, animal noises or music amusia, of receptive or sensory type.
This may be equivalent to noiseinduced visual phosphenes or sound-induced photisms. It is not certain that this phenomenon meets suggested criteria for synaesthesia.
Augmentation also refers to the paradoxical worsening of the symptoms of restless legs syndrome with dopaminergic treatment, manifesting with earlier onset of symptoms in the evening or afternoon, shorter periods of rest to provoke symptoms, greater intensity of symptoms when they occur, spread of symptoms to other body parts such as the arms, and decreased duration of benefit from medication.
An aura indicates the focal onset of neurological dysfunction. Auras are exclusively subjective, and may be entirely sensory, such as the fortification spectra teichopsia of migraine, or more complex, labelled psychosensory or experiential, as in certain seizures.
Localizing value of epileptic visual auras. Semiological seizure classification. Palmini A, Gloor P. The localising value of auras in partial seizures.
These are sometimes known as the startle-automatic obedience syndromes. Although initially classified by Gilles de la Tourette with tic syndromes, there are clear clinical and pathophysiological differences.
Gilles de la Tourette and the discovery of Tourette syndrome. Includes a translation of his article. It has been suggested that it should refer specifically to a permanently present or elicitable, compulsive, iterative and not necessarily complete, written reproduction of visually or orally perceived messages cf.
This is characterized as a particular, sometimes isolated, form of utilization behaviour in which the inhibitory functions of the frontal lobes are suppressed.
Increased writing activity in neurological conditions: a review and clinical study. Cross References Hypergraphia; Utilization behaviour Automatism Automatisms are complex motor movements occurring in complex motor seizures, which resemble natural movements but occur in an inappropriate setting.
These may occur during a state of impaired consciousness during or shortly after an epileptic seizure. There is usually amnesia for the event.
Automatisms occur in about one-third of patients with complex partial seizures, most commonly those of temporal or frontal lobe origin.
Although - 52 - Autoscopy A there are qualitative differences between the automatisms seen in seizures arising from these sites, they are not of sufficient specificity to be of reliable diagnostic value; bizarre automatisms are more likely to be frontal.
Automatic behaviour and fugue-like states may also occur in the context of narcolepsy and must be differentiated from the automatisms of complex partial seizures on the basis of history, examination, and EEG.
Complex partial seizures on closed circuit television and EEGs: a study of attacks in 79 patients. Cross References Absence; Aura; Pelvic thrusting; Poriomania; Seizure Autophony The perception of the reverberation of ones own voice, which occurs with external or middle, but not inner, ear disease.
The hallucinated image is a mirror image, i. Unlike heautoscopy, there is a coincidence of egocentric and body-centred perspectives.
Autoscopy may be associated with parieto-occipital space-occupying lesions, epilepsy, and migraine.
Out-of-body experience and autoscopy of neurological origin. Brugger P. Reflective mirrors: perspective taking in autoscopic phenomena.
Semiologic value of ictal autoscopy. Cross References Hallucination; Heautoscopy Autotopagnosia Autotopagnosia, or somatotopagnosia, is a rare disorder of body schema characterized by inability to identify parts of the body, either to verbal command or by imitation; this is sometimes localized but at worst involves all parts of the body.
This may be a form of category-specific anomia with maximum difficulty for naming body parts or one feature of anosognosia.
Reference Ogden JA. Autotopagnosia: occurrence in a patient without nominal aphasia and with an intact ability to point to parts of animals and objects.
The plantar response is most commonly performed by stroking the sole of the foot, although many other variants are described e.
References Critchley M. Lance JW. The Babinski sign. Van Gijn J. The Babinski sign: a centenary. Utrecht: Universiteit Utrecht, This observation indicated to Babinski the peripheral facial nerve origin of hemifacial spasm.
It may assist in differentiating hemifacial spasm from other craniofacial movement disorders. Reference Devoize JL. Journal of Neurology, Neurosurgery and Psychiatry ; The recumbent patient is asked to sit up with the arms folded on the front of the chest.
In organic hemiplegia there is involuntary flexion of the paretic leg, which may automatically rise higher than the normal leg; in paraplegia both legs are involuntarily raised.
In functional paraplegic weakness neither leg is raised, and in functional hemiplegia only the normal leg is raised. This pattern of facial sensory impairment may also be known as onion peel or onion skin.
Accurate eye movements may be programmed by sound or touch. Loss of spontaneous blinking has also been reported. Optic ataxia: A failure to grasp or touch an object under visual guidance.
Not all elements may be present; there may also be coexisting visual field defects, hemispatial neglect, visual agnosia, or prosopagnosia.
References Husein M, Stein J. Rezso Balint and his most celebrated case. Rafal R. Cross References Apraxia; Blinking; Ocular apraxia; Optic ataxia; Simultanagnosia Ballism, Ballismus Ballism or ballismus is a hyperkinetic involuntary movement disorder characterized by wild, flinging, throwing movements of a limb.
These movements most usually involve one-half of the body hemiballismus , although they may sometimes involve a single extremity monoballismus or both halves of the body paraballismus.
The movements are often continuous during wakefulness but cease during sleep. Hemiballismus may be associated with limb hypotonia.
Clinical and pathophysiological studies suggest that ballism is a severe form of chorea. It is most commonly associated with lesions of the contralateral subthalamic nucleus.
It indicates a lesion causing rectus abdominis muscle weakness below the umbilicus. This may occur with a spinal lesion e. Lower cutaneous abdominal reflexes are also absent, having the same localizing value.
Hilton-Jones D. Tashiro K. In: Rose FC ed. Patients with neuropathological lesions may also demonstrate a lack of concern for their disabilities, either due to a disorder of body schema anosodiaphoria or due to incongruence of mood typically in frontal lobe syndromes, sometimes seen in multiple sclerosis.
It is thought to result from viral inflammation of the facial VII nerve. Poorer prognosis is associated with older age over 40 years and if no recovery is seen within 4 weeks of onset.
Meta-analyses suggest that steroids are associated with better outcome than no treatment, but that acyclovir alone has no benefit.
Combined corticosteroid and antiviral treatment for Bell palsy: a systematic review and meta-analysis. This is a synkinesis of central origin involving superior rectus and inferior oblique muscles.
The reflex indicates intact nuclear and infranuclear mechanisms of upward gaze, and hence that any defect of upgaze is supranuclear.
Reference Bell C. On the motions of the eye, in illustration of the use of the muscles and nerves of the orbit.
The intorsion of the unaffected eye brought about by the head tilt compensates for the double vision caused by the unopposed extorsion of the affected eye.
Very occasionally, head tilt is paradoxical, i. The test is usually negative in a skew deviation causing vertical divergence of the eyes.
This test may also be used as part of the assessment of vertical diplopia to see whether hypertropia changes with head tilt to left or right; increased hypertropia on left head tilt suggests a weak intortor of the left eye superior rectus ; increased hypertropia on right head tilt suggests a weak intortor of the right eye superior oblique.
Cross References Diplopia; Hypertropia; Skew deviation Binasal Hemianopia Of the hemianopic defects, binasal hemianopia, suggesting lateral compression of the chiasm, is less common than bitemporal hemianopia.
Various causes are recorded including syphilis, glaucoma, drusen, and chronically raised intracranial pressure. Progressive binasal hemianopia.
Lancet ; Cross Reference Hemianopia Bitemporal Hemianopia Bitemporal hemianopia due to chiasmal compression, for example, by a pituitary lesion or craniopharyngioma, is probably the most common cause of a heteronymous hemianopia.
Conditions mimicking bitemporal hemianopia include congenitally tilted discs, nasal sector retinitis pigmentosa, and papilloedema with greatly enlarged blind spots.
Usually bilateral in origin, it may be sufficiently severe to result in functional blindness. The condition typically begins in the sixth decade of life and is more common in women than in men.
Like other forms of dystonia, blepharospasm may be relieved by sensory tricks geste antagoniste , such as talking, yawning, singing, humming, or touching the eyelid.
This feature is helpful in diagnosis. Blepharospasm may be aggravated by reading, watching television, and exposure to wind or bright light.
Blepharospasm is usually idiopathic but may be associated with lesions usually infarction of the rostral brainstem, diencephalon, and striatum; it has been occasionally reported with thalamic lesions.
The pathophysiological mechanisms underlying blepharospasm are not understood, but may reflect dopaminergic pathway disruption causing disinhibition of brainstem reflexes.
Local injections of botulinum toxin into orbicularis oculi are the treatment of choice, the majority of patients deriving benefit and requesting further injection.
Failure to respond to botulinum toxin may be due to concurrent eyelid apraxia or dopaminergic therapy with levodopa.
Blepharospasm: a review of patients. Hallett M, Daroff RB. Blepharospasm: report of a workshop. Reference Weiskrantz L.
A case study and implications. Oxford: Clarendon, Minor enlargement of the blind spot is difficult to identify clinically, formal perimetry is needed in this situation.
Enlargement of the blind spot peripapillary scotoma is observed with raised intracranial pressure causing papilloedema: this may be helpful in differentiating papilloedema from other causes of disc swelling such as optic neuritis, in which a central scotoma is the most common field defect.
Enlargement of the blind spot may also be a feature of peripapillary retinal disorders including big blind spot syndrome.
In contrast, blink rate is normal in multiple system atrophy and dopa-responsive dystonia, and increased in schizophrenia and postencephalitic parkinsonism.
These disparate observations are not easily reconciled with the suggestion that blinking might be a marker of central dopaminergic activity.
In patients with impaired consciousness, the presence of involuntary blinking implies an intact pontine reticular formation; absence suggests structural or metabolic dysfunction of the reticular formation.
Blinking decreases in coma. Functional disorders may be accompanied by an increase in blinking. Reference Karson CN. Spontaneous eye-blink rates and dopaminergic systems.
Care should be taken to avoid generating air currents with the hand movement as this may stimulate the corneal reflex which may simulate the visuopalpebral reflex.
It is probable that this reflex requires cortical processing: it is lost in persistent vegetative states. Acoustic stimulus: Sudden loud sounds acousticopalpebral reflex.
The final common efferent pathway for these responses is the facial nerve nucleus and facial VII nerve, the afferent limbs being the trigeminal V , optic II , and auditory VIII nerves, respectively.
Electrophysiological study of the blink reflex may demonstrate peripheral or central lesions of the trigeminal V nerve or facial VII nerve afferent and efferent pathways, respectively.
It has been reported that in the evaluation of sensory neuronopathy the finding of an abnormal blink reflex favours a non-paraneoplastic aetiology, since the blink reflex is normal in paraneoplastic sensory neuronopathies.
Role of the blink reflex in the evaluation of sensory neuronopathy. Liu GT, Ronthal M. Reflex blink to visual threat.
References Goodglass H, Kaplan E. Disturbance of gesture and pantomime in aphasia. These signs may help to distinguish tardive dyskinesia from chorea, although periodic protrusion of the tongue flycatcher, trombone tongue is common to both.
Cross References Buccolingual syndrome; Chorea, Choreoathetosis; Trombone tongue Bouche de Tapir Patients with facioscapulohumeral FSH muscular dystrophy have a peculiar and characteristic facies, with puckering of the lips when attempting to whistle.
The pouting quality of the mouth, unlike that seen with other types of bilateral neurogenic facial weakness, has been likened to the face of the tapir Tapirus sp.
Cross Reference Facial paresis Bovine Cough A bovine cough lacks the explosive character of a normal voluntary cough.
It may result from injury to the distal part of the vagus nerve, particularly the recurrent laryngeal branches which innervate all the muscles of the larynx with the exception of cricothyroid with resultant vocal cord paresis.
Because of its longer intrathoracic course, the left recurrent laryngeal nerve is more often involved. A bovine cough may be heard in patients with tumours of the upper lobes of the lung Pancoast tumour due to recurrent laryngeal nerve palsy.
Cross References Bulbar palsy; Diplophonia; Signe de rideau Bradykinesia Bradykinesia is a slowness in the initiation and performance of voluntary movements in the absence of weakness and is one of the typical signs of parkinsonian syndromes, in which situation it is often accompanied by difficulty in the initiation of movement akinesia, hypokinesia and reduced amplitude of movement hypometria which may increase with rapid repetitive movements fatigue.
It may be overcome by reflexive movements or in moments of intense emotion kinesis paradoxica. Bradykinesia in parkinsonian syndromes reflects dopamine depletion in the basal ganglia.
It may be improved by levodopa and dopaminergic agonists, less so by anticholinergic agents. Such patients typically answer questions correctly but with long response times.
Silent reading may also be impaired deep dyslexia as reflected by poor text comprehension; Writing: similarly affected.
The term alalia was also once used. Depression may be a concurrent feature. More commonly there is infarction in the perisylvian region affecting the insula and operculum Brodmann areas 44 and 45 , which may include underlying white matter and the basal ganglia territory of the superior branch of the middle cerebral artery.
Grodzinsky Y, Amunts K eds. Sensory: A dissociated sensory loss, i. Spinal cord lesions producing this syndrome may be either extramedullary e.
A visionary of science. Engelhardt P, Trostdorf E. Tattersall R, Turner B. Passive flexion of the neck to bring the head onto the chest is accompanied by flexion of the thighs and legs.
Reference Pearce JMS. Kernig and Brudzinski. In: Fragments of neurological history. Additional features may include paroxysmal hyperpnoea and upbeating nystagmus.
Reference Gilbert GJ. Brueghel syndrome: its distinction from Meige syndrome. Cross References Blepharospasm; Dystonia Bruit Bruits arise from turbulent blood flow causing arterial wall vibrations which are audible at the body surface with the unassisted ear or with a stethoscope diaphragm rather than bell, better for detecting higher frequency sounds.
They are associated with stenotic vessels or with fistulae where there is arteriovenous shunting of blood. Dependent on the clinical indication, various sites may be auscultated: eye for orbital bruit in carotico-cavernous fistula; head for bruit of AV fistula; but probably the most frequently auscultated region is the carotid bifurcation, high up under the angle of the jaw, in individuals thought to have had a transient ischaemic attack or ischaemic stroke.
Examination for carotid bruits in asymptomatic individuals is probably best avoided, other than in the clinical trial - 67 - B Brushfield Spots setting, since the optimal management of asymptomatic carotid artery stenosis has yet to be fully defined.
The carotid bruit. Bruxism Bruxism is forcible grinding or gnashing of the teeth. Masseter hypertrophy may become apparent in persistent grinders.
Bruxism may also occur in encephalopathic disorders e. If necessary, a rubber gum shield or bite may be worn in the mouth to protect the teeth.
Botulinum toxin injections have also been tried. Bruxism: a critical review. This may be differentiated clinically from bulbar weakness of upper motor neurone origin pseudobulbar palsy.
Recognized causes include Brainstem disorders affecting cranial nerve motor nuclei intrinsic : Motor neurone disease which may also cause a pseudobulbar palsy ; Poliomyelitis; Glioma; Syringobulbia.
A myogenic bulbar palsy may be seen in oculopharyngeal muscular dystrophy, inclusion body myositis, and polymyositis.
The reflex may be abolished in lesions of the cauda equina. Cross References Cauda equina syndrome; Reflexes Buphthalmos Buphthalmos, literally ox-eye, consists of a large and bulging eye caused by raised intraocular pressure due to congenital or secondary glaucoma.
Diamond on quadriceps sign may also be seen in dysferlinopathies. Reference Pradhan S. Calf-head sign in Miyoshi myopathy.
Cross Reference Diamond on quadriceps sign Calf Hypertrophy Calf enlargement has many causes; it may reflect true hypertrophy enlargement of muscle fibres or, more commonly, pseudohypertrophy, due to infiltration with tissue elements other than muscle.
Calf and other muscle hypertrophy is also a feature of limb girdle muscular dystrophy type 2I. Unilateral calf hypertrophy.
Calf hypertrophy following paralytic poliomyelitis. Each labyrinth may be separately assessed by irrigating each outer ear.
Induced nystagmus is then timed both with and without visual fixation in the dark, Frenzel glasses. This method is cheap but has poor patient acceptability.
Normally, the eyes show conjugate deviation towards the ear irrigated with cold water, with corrective nystagmus in the opposite direction; with warm water the opposite pattern is seen.
The direction of nystagmus may thus be recalled by the mnemonic COWS: cold opposite, warm same. Dysconjugate responses suggest brainstem damage or depression.
A reduced duration of induced nystagmus is seen with canal paresis; enhancement of the nystagmus with removal of visual fixation suggests this is peripheral in origin labyrinthine, vestibulocochlear nerve , whereas no enhancement suggests a central lesion.
As coma deepens even the caloric reflexes are lost as brainstem involvement progresses. Investigations of disorders of balance.
In: Hughes RAC ed. Neurological Investigations. Cases with associated lenticular putaminal lesions have also been described. Camptocormia: pathogenesis, classification, and response to therapy.
Camptocormia: the bent spine syndrome, an update. The term streblomicrodactyly has sometimes been used to designate isolated crooked little fingers.
Camptodactyly is not accompanied by any sensory or motor signs. The condition may be familial and is more common in women.
Camptodactyly may occur as part of a developmental disorder with other dysmorphic features or in isolation.
Awareness of the condition is important to avoid unnecessary neurological investigation. Camptodactyly in a neurology outpatient clinic.
Initially described in patients with psychiatric disorders, it may also occur in traumatic, metabolic, and neurodegenerative disorders e.
Neurologists have encompassed this phenomenon under the term reduplicative paramnesia. Capgras syndrome may be envisaged as a Geschwindian disconnection syndrome, in which the visual recognition system is disconnected from the limbic system, hence faces can be recognized but no emotional significance ascribed to them.
Behavioral neurology. The neurology of Capgras syndrome. Ramachandran VS, Blakeslee S. Phantoms in the brain.
Human nature and the architecture of the mind. Some have characterized carphologia as a form of akathisia.
Carphologia, or floccillation. Advances in Clinical Neuroscience and Rehabilitation ; 7 4 : Clearly, this term is cognate with or overlaps with waxy flexibility which is a feature of catatonic syndromes.
Catalepsy should not be confused with the term cataplexy, a syndrome in which muscle tone is transiently lost. Reference Perkin GD.
Cross Reference Cataplexy; Catatonia Cataplexy Cataplexy is a sudden loss of limb tone which may lead to falls drop attacks without loss of consciousness, usually lasting less than 1 min.
Attacks may be precipitated by strong emotion laughter, anger, embarrassment, surprise. Sagging of the jaw and face may occur, as may twitching around the face or eyelids.
During an attack there is electrical silence in antigravity muscles, which are consequently hypotonic, and transient areflexia.
Rarely status cataplecticus may develop, particularly after withdrawal of tricyclic antidepressant medication. Therapeutic options for cataplexy include tricyclic antidepressants such as protriptyline, imipramine, and clomipramine; serotonin-reuptake inhibitors such as fluoxetine; and noradrenaline and serotonin-reuptake inhibitors such as venlafaxine.
Pharmacotherapy for cataplexy. Cross References Areflexia; Hypersomnolence; Hypotonia, Hypotonus Catathrenia Catathrenia is expiratory groaning during sleep, especially its later stages.
Although sufferers are unaware of the condition, it does alarm relatives and bed partners. There are no associated neurological abnormalities and no identified neurological or otorhinolaryngological cause.
Catathrenia nocturnal groaning : a new type of parasomnia. Catatonia Catatonia is a clinical syndrome, first described by Kahlbaum , characterized by a state of unresponsiveness but with maintained, immobile, body posture sitting, standing; cf.
After recovery patients are often able to recall events which occurred during the catatonic state cf.
Catatonia may be confused clinically with abulia. Kraepelin classified catatonia as a subtype of schizophrenia but most catatonic patients in fact suffer a mood or affective disorder.
Neurological disorders: Cerebrovascular disease posterior circulation ; Tumours especially around third ventricle, corpus callosum ; Head trauma; Encephalitis; Neurosyphilis; Extrapyramidal disorders; Epilepsy.
Catatonia of psychiatric origin often responds to lorazepam; there are also advocates of ECT. Kahlbaum K. Levij Y, Pridan T trans. Moore DP.
Textbook of clinical neuropsychiatry 2nd edition. Catatonia or abulia? A difficult differential diagnosis. Weakness of hip flexion L1 does not occur, and - 76 - Central Scotoma, Centrocaecal Scotoma C this may be useful in differentiating a cauda equina syndrome from a conus lesion which may otherwise produce similar features.
The syndrome needs to be considered in any patient with acute or acute-onchronic low back pain, radiation of pain to the legs, altered perineal sensation, and altered bladder function.
Missed diagnosis of acute lumbar disc herniation may be costly, from the point of view of both clinical outcome and resultant litigation.
Cauda equina syndrome secondary to lumbar disc herniation: a meta-analysis of surgical outcomes. Markham DE. Cauda equina syndrome: diagnosis, delay and litigation risk.
Cauda equina syndrome. Cross References Bulbocavernosus reflex; Foot drop; Incontinence; Radiculopathy; Urinary retention Central Scotoma, Centrocaecal Scotoma These visual field defects are typical of retinal or optic nerve pathology.
They may be mapped by confrontation testing or automatically. Examination for a concurrent contralateral superior temporal defect should be undertaken: such junctional scotomas may be seen with lesions at the anterior angle of the chiasm.
Broadly speaking, a midline cerebellar syndrome involving the vermis may be distinguished from a hemispheric cerebellar syndrome involving the hemispheres.
Reference Holmes G. The Croonian lectures on the clinical symptoms of cerebellar disease and their interpretation. References Chaddock CG.
A preliminary communication concerning a new diagnostic nervous sign. There is trophic change, with progressive destruction of articular surfaces with disintegration and reorganization of joint structure.
Although the destruction is painless, the Charcot joint itself may be painful. There may be concurrent skin ulceration. Charcot joints.
Cross References Analgesia; Main succulente Charles Bonnet Syndrome Described by the Swiss naturalist and philosopher Charles Bonnet in , this syndrome consists of well-formed complex , elaborated, and often stereotyped visual hallucinations, of variable frequency and duration, in a partially sighted usually elderly individual who has insight into their unreality.
Hallucinations may disappear on eye closure. Predisposing visual disorders include cataract, macular degeneration, and glaucoma.
There are no other features of psychosis or neurological disease such as dementia. The pathogenesis of the visual hallucinations is uncertain.
Reduced stimulation of the visual system leading to increased cortical hyperexcitability is one possible explanation the deafferentation hypothesis , although the syndrome may occasionally occur in people with normal vision.
Functional magnetic resonance imaging suggests ongoing cerebral activity in ventral extrastriate visual cortex.
Treatment consists primarily of reassurance. Pharmacological treatment with atypical antipsychotics or anticonvulsants may be tried but there is no secure evidence base.
The anatomy of conscious vision: an fMRI study of visual hallucinations. Flournoy T. Le cas de Charles Bonnet. Complex visual hallucinations in the visually impaired: the Charles Bonnet syndrome.
The Charles Bonnet syndrome: a large prospective study in the Netherlands. Storage of sphingolipids or other substances in ganglion cells in the perimacular region gives rise to the appearance.
Cross Reference Winging of the scapula Chorea, Choreoathetosis Chorea is an involuntary movement disorder characterized by jerky, restless, purposeless movements literally dance-like which tend to flit from one part of the body to another in a rather unpredictable way, giving rise to a fidgety appearance.
There may also be athetoid movements slow, sinuous, writhing , jointly referred to as choreoathetosis. There may be concurrent abnormal muscle tone, - 80 - Chorea, Choreoathetosis C either hypotonia or rigidity.
Hyperpronation of the upper extremity may be seen when attempting to maintain an extended posture. The pathophysiology of chorea as for ballismus is unknown; movements may be associated with lesions of the contralateral subthalamic nucleus, caudate nucleus, putamen, and thalamus.
One model of basal ganglia function suggests that reduced basal ganglia output to the thalamus disinhibits thalamic relay nuclei leading to increased excitability in thalamocortical pathways which passes to descending motor pathways resulting in involuntary movements.
Hypernatraemia or hyponatraemia, hypomagnesaemia, hypocalcaemia; hyperosmolality; Hyperglycaemia or hypoglycaemia; Non-Wilsonian acquired hepatocerebral degeneration; Nutritional.
Tumours: Primary and secondary rare. Where treatment is necessary, antidopaminergic agents such as dopaminereceptor antagonists e.
Chorea: diagnosis and management. Seminar on chorea. The differential diagnosis of chorea. Cause and course in a series of patients with sporadic chorea.
Luria claimed it was associated with deep-seated temporal and temporodiencephalic lesions, possibly right-sided lesions in particular.
Whether this is a true agnosia remains open to investigation. Reference Luria AR. Higher cortical function in man 2nd edition. The pathophysiology of this mechanosensitivity of nerve fibres is uncertain, but is probably related to increased discharges in central pathways.
The ciliospinal response in man. Cross Reference Pupillary reflexes Cinematic Vision Cinematic vision is a form of metamorphopsia, characterized by distortion of movement with action appearing as a series of still frames as if from a movie.
Causes include migraine aura, partial seizures, and schizophrenic psychosis. Tip of the tongue states in lexical access in dementia.
Cross References Rigidity; Spasticity Claudication Claudication literally limping, Latin claudicatio refers to intermittent symptoms of pain secondary to ischaemia.
Claudication of the legs on walking is a symptom of peripheral vascular disease. Claudication of the jaw, tongue, and limbs especially upper may be a feature of giant cell temporal arteritis.
Presence of jaw claudication is one of the clinical features which increases the likelihood of a positive temporal artery biopsy.
Neurologic disease in biopsy-proven giant cell temporal arteritis. This web site presents helpful facts to us, keep it up.
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