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Neurologic Terminology

Chapter

Abstract

This chapter provides the clarification of ­acronyms, brief definitions of signs, and short descriptions of syndromes. Detailed elaboration of some of these will be found in the text. Short outlines of rare syndromes will be added as full discussions cannot be included in an introductory text. By necessity this will only be a selection from the myriad numbers of syndromes and signs to choose from.

Keywords

Terminology Acronyms Definitions Signs Syndromes 

Notes

Glossary

Acronyms

ACA

Anterior cerebral artery

ACoA

Anterior communicating artery

ADEM

Acute demyelinating encephalomyelitis

AHI

Apnea–hypopnea index

AICA

Anterior inferior cerebellar artery

AIDP

Anterior inflammatory demyelinating polyneuropathy

AIDS

Acquired immune deficiency syndrome

AION

Anterior ischemic optic neuropathy

ALS

Amyotrophic lateral sclerosis

AMS

Altered mental status

APD

Afferent pupillary defect

AVM

Arteriovenous malformation

BPPV

Benign paroxysmal positional vertigo

CADASIL

Cerebral autosomal dominant ­arteriopathy with subcortical infarcts and leukoencephalopathy

CGRP

Calcitonin gene-related peptide

CIDP

Chronic inflammatory demyelinating polyneuropathy

CNS

Central nervous system

CPEO

Chronic progressive external ophthalmoplegia

CPS

Cycles per second

CSA

Central sleep apnea

CSD

Cortical spreading depression

CSF

Cerebrospinal fluid

CST

Corticospinal tract

CTS

Carpal tunnel syndrome

DHE

Dihydroergotamine

GABA

Gamma-aminobutyric acid

GAD

Glutamic acid decarboxylase

HIV

Human immunodeficiency virus

Hz

Hertz

INO

Internuclear ophthalmoplegia

IO

Inferior oblique muscle

IR

Inferior rectus muscle

JME

Juvenile myoclonic epilepsy

LPS

Levator palpebrae superioris

LR

Lateral rectus muscle

MCA

Middle cerebral artery

MELAS

Mitochondrial encephalopathy with lactic acidosis and stroke

MLF

Medial longitudinal fasciculus

MR

Medial rectus muscle

MSA

Multiple system atrophy

MSLT

Multiple sleep latency test

NMO

Neuromyelitis optica (Devic’s disease)

NPH

Normal pressure hydrocephalus

OCD

Obsessive compulsive disorder

OKN

Optokinetic nystagmus

OSA

Obstructive sleep apnea

PCA

Posterior cerebral artery

PCR

Polymerase chain reaction

PICA

Posterior inferior cerebellar artery

PION

Posterior ischemic optic neuropathy

PLMS

Periodic limb movements of sleep

PML

Progressive multifocal leukoencephalopathy

PNS

Peripheral nervous system

PPRF

Paramedian pontine reticular formation

PRES

Posterior reversible encephalopathy syndrome

PSP

Progressive supranuclear palsy

RBD

REM behavior disorder of sleep

REM

Rapid eye movement

riMLF

Rostral interstitial nucleus of the MLF

RSD

Reflex sympathetic dystrophy

SAH

Subarachnoid hemorrhage

SCA

Superior cerebellar artery

SR

Superior rectus muscle

SNRI

Selective norepinephrine reuptake inhibitor

SSEP

Somatosensory-evoked potential

SSRI

Selective serotonin reuptake inhibitor

SUNCT

Short-lasting unilateral neuralgiform headaches with conjunctival injection and tearing

VEP

Visual-evoked potential

VPL

Ventral posterolateral nucleus of thalamus

VPM

Ventral posteromedial nucleus of thalamus

WEBINO

Wall-eyed bilateral internuclear ophthalmoplegia

WEMINO

Wall-eyed monocular internuclear ophthalmoplegia

Signs

Aberrant reinnervation

After a nerve is injured there is regrowth of axons but some are redirected to the wrong muscle. This commonly results in a synkinesia. A good example is after recovery from a Bell’s palsy when an eye blink provokes movement of the mouth (orbicularis oris).

Abulia

Patients with abulia are passive, apathetic, and have little interest in their surroundings.

Adie’s pupil (tonic pupil)

This is a large, usually unilateral pupil which is nonreactive to light and has slow constriction to a prolonged near stimulus.

Adson’s maneuver

Examination method for detecting a thoracic outlet syndrome (see  Chap. 4).

Afferent pupillary defect (Marcus-Gunn pupil)

There is a normal consensual response to light but dilation when the light is rapidly switched back from the normal to the involved eye. The involved pupil may react normally or sluggishly to a direct light stimulus. This response indicates optic nerve pathology.

Agrammatism

Defective syntax.

Agnosia

Normal reception of a specific sensory stimulus with the inability to recognize the object using the same sensory system, but there is retained ability to recognize it using a different sensory pathway.

Agraphia

Inability to write.

Akathisia

A voluntary movement to relieve an inner restlessness typically manifested by walking or rocking. Ordinarily this is due to use of dopamine receptor blockers.

Akinetic mutism.

Patients in an akinetic mute state are immobile, do not speak but appear vigilant as the eyes are open, and there are occasional pursuit and saccadic eye movements.

Alexander’s law

Nystagmus is more rapid when the eyes are deviated in the direction of the quick phase.

Alexia

Inability to read.

Alexia without agraphia

Patients are unable to read what they have just written due to a lesion of the dominant occipital lobe and splenium of the corpus callosum.

Alien hand sign

Unilateral wandering involuntary hand movements, a sign often observed with corticobasal ganglia degeneration, but not pathognomonic of this disorder.

Allesthesia

Sensation of a stimulus on one side is perceived as arising from the contralateral side in the same location.

Allodynia

A nonpainful stimulus perceived as painful.

Anarthria

See aphemia.

Anesthesia dolorosa

Pain in a region of hypesthesia, typical of postherpetic neuralgia.

Anosodiaphoria

Lack of concern about a neurologic deficit, typically a hemiparesis.

Anosognosia

Absolute denial of a neurologic deficit, typically a hemiparesis.

Aphasia

Impaired language function (see  Chap. 4).

Aphemia

The patient is mute but is able to write. Lesions are usually in Broca’s area or in adjacent subcortical white matter.

Apneustic

This refers to a breathing pattern of 2–3 s of apnea after a deep inspiration.

Apraxia

Impaired performance of a learned movement despite preserved strength.

Argyll-Robertson pupil

Small irregular pupils, poorly or nonreactive to light with a normal reaction to a near stimulus.

Asomatognosia

Loss of awareness of one-half of the body.

Astasia-abasia

A lurching gait with irregular steps associated with a conversion reaction.

Asterixis

The flapping downward tremor typically seen in the hands when the arms are extended in the pronated position with the wrists dorsiflexed. This is considered a negative myoclonus and is associated with any type of metabolic encephalopathy.

Athetosis

Slow, writhing, involuntary, large amplitude movements involving distal upper extremities, and less often face and trunk musculature.

Autotopagnosia

Failure of a patient to recognize or name his own limb or body part.

Ballism

Abrupt swinging movements of an arm or leg involving proximal muscles due to a lesion in the contralateral subthalamic nucleus.

Beevor’s sign

Movement of the umbilicus upward with head flexion when there is paresis of lower abdominal muscles due to a spinal cord lesion at T10.

Bell’s phenomenon

A normal eye movement up and out with eye closure.

Bielschowsky head tilt test

Increased diplopia due to a 4th nerve lesion when the head is tilted toward the side of the lesion.

Bradykinesia

Slow movements.

Bradyphrenia

Slow thinking.

Broca’s aphasia

A nonfluent aphasia with good comprehension and poor repetition (see  Chap. 4).

Brudzinski’s sign

A sign of meningeal irritation (see  Chap. 4).

Camptocormia

A condition in which the thoraco-lumbar spine is flexed when walking but not in bed. This sign is common in Parkinson’s disease, Parkinson-plus syndromes, and dystonia.

Cataplexy

A sudden decrease in muscular tone most often in the legs occasionally resulting in falls. This is a cardinal feature of narcolepsy.

Causalgia

See complex regional pain syndrome.

Chaddock’s sign

An extensor plantar response when the lateral side of the foot is scraped beginning at the heel and extending up to the base of the fifth toe.

Cheyne-Stokes

This refers to abnormal respiration manifested by short periods of hyperpnea alternating with apnea in a crescendo-decrescendo pattern.

Chorea

Rapid jerky movements of the extremities, most prominent in distal musculature.

Cogan’s lid twitch sign

When patients with ocular myasthenia gravis look from a down to a neutral position, the upper eyelid may twitch upward transiently uncovering the sclera.

Collier’s sign

Eyelid retraction associated with the pretectal syndrome (Parinaud’s, Sylvian aqueduct, dorsal midbrain syndromes).

Conduction aphasia

A fluent aphasia with good comprehension and poor repetition.

Coprolalia

Use of obscene language.

Copropraxia

Use of obscene gestures.

Cruciate paresis

Paresis of one arm and the contralateral leg.

Crural paresis

This refers to a hemiparesis, leg greater than arm.

Cushing’s response

The signs of increased intracranial pressure, hypertension, bradycardia, and slow respiratory rate.

Déjà vu

A sense of reliving an experience.

Delusion

A false belief.

Depersonalization

A sense of detachment from the body.

Derealization

A feeling of being disconnected from the environment.

Dix–Hallpike test

A specific test for benign ­paroxysmal positional vertigo (see  Chap. 4).

Double elevator palsy

Monocular elevation paresis due to a contralateral supranuclear pretectal lesion or an ipsilateral lesion of efferent fibers from the riMLF to SR and IO subnuclei.

Dysarthria

Slurred speech.

Dysdiadochokinesis

Impairment of rapid alternating movements, a nonlocalizing neurologic sign.

Dysesthesia

Unpleasant sensations provoked by an ordinary stimulus.

Dyskinesia

Abnormal involuntary repetitive movements.

Dysphagia

Difficulty swallowing.

Dysprosody

This term is usually applied to loss of inflection in speech producing a monotone quality. It also refers to changes in the emphasis or timing of words and pauses between words in a phrase.

Dystonia

Slow involuntary movements or sustained postures usually involving truncal and proximal-greater-than-distal movements of the extremities.

Echolalia

The repetition of words or phrases just spoken to the patient.

Epiphora

Excessive tearing.

Erb’s palsy

This term refers to an upper brachial plexus lesion affecting C5–C6 roots. The name is usually applied to a birth injury but may be used with any traumatic lesion or idiopathic plexitis.

Extra-axial

This term refers to a lesion adjacent to brain.

Festination

An uncontrollable acceleration of gait most often observed in patients with Parkinson’s disease.

Gegenhalten

See paratonic rigidity.

Gelastic

The term used to describe sudden, uncontrollable laughter associated with a seizure disorder.

Glabellar reflex

An abnormal response to repetitive tapping over the bridge of the nose manifested by continuous involuntary blinking.

Glasgow Coma Scale

(See  Chap. 5).

Graefe’s sign

Lid lag on looking down due to thyroid disease.

Hallucination

A sensory experience without an external stimulus.

Hoffmann’s sign

Flexion of the distal phalanx of the thumb when the middle finger is hyperextended and the nail is flicked downward.

Hoover’s sign

A finding associated with feigned weakness of a leg as the patient does not apply the downward pressure on the contralateral leg when requested to raise the weak leg.

Hunt–Hess Scale

A scale to assess severity of subarachnoid hemorrhage (see  Chap. 7).

Hutchinson pupil

This is a widely dilated, poorly or nonreactive pupil which is an early sign of uncal herniation due to compression of the peripherally located pupillary fibers of the third nerve.

Hyperpathia

Exaggerated response to a painful stimulus.

Hyperesthesia

Acute sensitivity to a sensory stimulus.

Hypesthesia

Decreased perception of a stimulus.

Hypomimia

This refers to a patient with little spontaneous expression, masked facies.

Illusion

Distortion of a sensory perception.

Incubus

Night terrors in adults manifested by screaming during stage 3 sleep.

Internuclear ophthalmoplegia

Ipsilateral medial rectus paresis with or without nystagmus in the contralateral abducting eye, with or without preservation of convergence. The lesion is in the medial longitudinal fasciculus.

Internuclear ophthalmoplegia of abduction (Lutz’s disorder)

Abduction paresis attributed to impaired inhibition of the ipsilateral medial rectus secondary to a lesion of the midbrain or rostral pons.

Intra-axial

This term refers to a lesion within brain.

Jamais vu

A feeling that one’s surroundings are unfamiliar.

Kernig’s sign

A sign of meningeal irritation (see  Chap. 4).

Kernohan’s notch

Compression of the opposite cerebral peduncle against the incisura of the tentorium cerebelli during uncal herniation. This causes a hemiparesis ipsilateral to the involved cerebral hemisphere.

Klumpke paresis

Lower brachial plexopathy involving C8–T1 roots usually due to trauma or infrequently an invasive neoplasm.

La belle indifference

Demeanor inconsistent with the presumed illness.

Lasegue’s sign

The straight leg raising test used to examine for lumbar radiculopathy.

Lhermitte’s sign

Electricity-like sensations which radiate down the arms, occasionally the back and legs, provoked by head flexion. This may be due to increased sensitivity of the posterior columns and is a sign most often associated with multiple sclerosis but may occur with other pathologies such as cervical spinal stenosis.

Logorrhea

Pathologic garrulousness.

Main en griffe

Claw hand deformity due to an ulnar nerve lesion above the elbow. There is hyperextension of the 4th, 5th and occasionally third fingers at the metacarpal phalangeal joints and flexion at the interphalangeal joints.

Magnetic gait

Patients have difficulty initiating steps and their feet appear stuck to the floor. If able to walk the gait is shuffling and turning requires several steps.

Marche à petit pas

See magnetic gait.

Marcus Gunn phenomenon

Unilateral lid retraction with jaw movement or swallowing.

Marcus Gunn pupil

See afferent pupillary defect.

Meralgia paresthetica

Compression of the lateral femoral cutaneous nerve of the thigh (L2–L3) under the inguinal ligament causing numbness, pain, and paresthesias in the nerve’s distribution over the anterolateral thigh.

Metamorphopsia

A distortion of form such as a straight line becoming crooked.

Myerson’s sign

See glabellar reflex.

Myoclonus

A brief shock-like movement due to either muscle contraction (positive) or muscle inhibition (negative).

Myokymia

Continuous slow muscle twitching producing an undulation of the muscle surface.

Myotonia

Impaired muscle relaxation after it is tapped, usually at the thenar eminence.

Neglect

This term refers to a patient who pays no attention to a weak limb but, when specifically requested, can use it. This sign is usually associated with contralateral parietal lobe lesions.

Neologism

A paraphasia manifested by a new word manufactured by the patient.

Notalgia paresthetica

Burning, itching, and paresthesias at the medial margin of the scapula in a well-circumscribed region a few inches in diameter. The etiology is unknown.

Nylen-Barany test

See Dix–Hallpike test.

Oculomotor apraxia

Absence of volitional saccades and pursuit.

Ondine’s curse

Loss of automatic breathing during sleep. This can be caused by lesions of the tegmentum of the medulla or high cervical cord at C1–C2.

Onion skin pattern

This term refers to the type of facial sensory loss with intramedullary lesions involving the trigeminal sensory system.

Oppenheim’s sign

An extensor plantar sign evoked by firmly scraping down the shin from the knee to the foot using the knuckles.

Optic ataxia

Inability to reach a target which is clearly seen.

Oscillopsia

An illusory perception of movement of the environment.

Otolithic crisis

Drop attack associated with Ménière’s disease and described by patients as a sensation of being pushed or thrown to the ground.

Palilalia

Compulsive repetition of phrases or words at increasing speed and diminishing volume.

Palinacusis

The patient perceives an auditory stimulus after the auditory stimulus has been removed.

Palinopsia

The perseveration of a visual image which is no longer present.

Paraphasia

This term refers to abnormal formation of words, inappropriate use of words, or manufactured new words. This is a diagnostic feature of aphasia.

Paratonia

The patient with paratonia actively resists a passive motion at a joint despite an admonition to relax. This is also known by the term Gegenhalten.

Pavor-nocturnus

Night terrors in children manifested by screaming during stage 3 sleep.

Pelopsia

A sense that objects are looming up in front of the patient. This may be a simple partial seizure.

Perseveration

Repetition of the same response to a new question.

Phalen’s sign

Flexion of the wrist to 90° for up to 60 s may produce paresthesias in the hand due to irritation of the median nerve.

Phosphenes

Flashes of light which may occur with pressure on the globe, rapid saccades, traction on the vitreous, and accommodation. They can be an early sign of retinal detachment.

Prosopagnosia

A selective inability to recognize faces.

Pseudobulbar palsy

This usually refers to involuntary laughing or crying associated with bilateral lesions of corticobulbar pathways. Impairment of speech, swallowing, chewing, and breathing may also be present.

Pseudo-von Graefe phenomenon

A type of aberrant third nerve regeneration with elevation of the lid on downgaze.

Punding

Purposeless motor activity such as fiddling with the bed sheets.

Reflex sympathetic dystrophy

See complex regional pain syndrome.

Riddoch’s phenomenon

Visual perception of movement in a hemianopic field.

Romberg’s sign

Unsteadiness when standing with eyes closed and feet together.

Simultanagnosia

Ability to see individual elements of a picture or scene but inability to comprehend or synthesize its meaning or how to interrelate the parts.

Skew deviation

Vertical separation of the globes.

Somnambulism

Sleep walking.

Somniloquy

Sleep talking.

Spetzler–Martin Grading Scale

Grading of an arteriovenous malformation by its size, location, and venous drainage (see  Chap. 7).

Stellwag’s sign

Infrequent blinking in Grave’s disease.

Synkinesia

An involuntary movement of a muscle which should not ordinarily contract when a voluntary movement is initiated elsewhere.

Tardive

A term applied to delayed development of dyskinesias or dystonia after initiating treatment with a dopamine-blocking agent.

Teichopsia

“Walled vision” as occurs in a homonymous hemianopsia.

Teleopsia

Objects appear at a distance. Brief episodes of teleopsia may indicate simple partial seizures.

Tic

Brief sudden movements or sounds which are often repetitive and stereotyped.

Tinel’s sign

Tingling provoked by percussion over an injured nerve.

Todd’s phenomenon

This is a focal neurologic deficit present after a seizure.

Torticollis

Cervical dystonia manifested by spasm of neck musculature with the head drawn to one side and the chin pointing to the other side.

Torsional

This term usually refers to nystagmus which has a predominant rotatory component.

Tullio phenomenon

This phenomenon occurs when a sound at a specific frequency produces paroxysmal vertigo and nystagmus.

Tumarkin’s otolithic crisis

See otolithic crisis.

Uthoff’s sign

Exacerbation or provocation of a neurologic deficit due to exercise or elevation of body temperature.

Vegetative state

Condition in which the patient is awake but unaware (see  Chap. 5).

Vestibular triad

Vertigo, vomiting, and diaphoresis.

Wernicke’s aphasia

A fluent aphasia with poor comprehension and poor repetition.

Witzelsucht

Inappropriate affect manifested by joking which may be present in patients with frontal lobe lesions.

Xanthochromia

Yellow color of centrifuged CSF supernatant after CNS hemorrhage. The yellow color is due to bilirubin.

Syndromes

Anterior interosseous syndrome

The anterior interosseous nerve is a purely motor branch of the median nerve. An injury causes pain in the proximal forearm, paresis of pronation, paresis of flexion of the terminal phalanges of the thumb, second and third fingers, and inability to pinch.

Antiphospholipid antibody syndrome

(See  Chap. 11, Case 27).

Anton’s syndrome

Denial of blindness which is usually a sign of bilateral occipital lobe infarctions.

Balint’s syndrome

Simultanagnosia, optic ataxia and oculomotor apraxia.

Bassen–Kornzweig syndrome

This autosomal recessive disorder is associated with an external ophthalmoplegia and clinical signs indicating posterior column, spinocerebellar, and corticospinal tract involvement. Laboratory abnormalities include abetalipoproteinemia, acanthocytosis, and hypocholesterolemia.

Benedikt syndrome

Third nerve palsy and contralateral ataxia, chorea or tremor due to involvement of third nerve fibers and the red nucleus.

Brown-Séquard syndrome

Hemisection or damage to one-half of the spinal cord which results in an ipsilateral spastic paresis, ipsilateral loss of vibration and position sense below the level of the lesion, and contralateral pain and temperature loss one or two levels below the lesion.

Brown superior oblique tendon sheath syndrome

Mechanical restriction of the superior oblique tendon at the pulley suggesting an inferior oblique paresis.

Carpal tunnel syndrome

Paresthesias and/or weakness in the distribution of the median nerve due to its compression between the carpal bones and the transverse carpal ligament.

Cauda equina syndrome

Lesion of lower lumbar (L4–L5) and sacral roots (S1–S5) which may cause a paraparesis, sphincter disturbances, and severe pain with sensory loss in the buttocks, perianal, genital, and perineal regions.

Cavernous sinus syndrome

Lesions within the cavernous sinus which variably affect the 3rd, 4th, and 6th nerves, the first two divisions of the 5th cranial nerve and sympathetic fibers within the carotid sheath.

Cerebellopontine angle syndrome

Lesions in this angle affect primarily the 5th, 7th, and 8th cranial nerves and adjacent cerebellum. Schwannomas of the 8th cranial nerve are the most common etiology.

Charles Bonnet syndrome

This syndrome is characterized by vivid, visual, and nonthreatening hallucinations of people or objects in patients with severe visual loss, most often due to severe retinal disease. The patient is aware that they are unreal.

Claude Bernard syndrome

Oculosympathetic irritation causing periodic pupillary dilatation, lid retraction, facial hyperhidrosis, and headache.

Complex regional pain syndrome

Tissue swelling, muscular atrophy, and bony changes associated with severe pain secondary to trauma which is often minor.

Cubital tunnel syndrome

An ulnar neuropathy due to the nerve being compressed at the elbow by a fibrous band, bone fragment, or bulging of the medial collateral ligament.

Dandy–Walker syndrome

This disorder includes a cystic 4th ventricle, partial or complete absence of the cerebellum and an enlarged posterior fossa.

Dorsal midbrain syndrome

See pretectal syndrome.

Droopy shoulder syndrome

Thoracic outlet syndrome primarily in women secondary to intermittent compression of the brachial plexus caused by drooping shoulders associated with a long swan neck and downward slope of the clavicles.

Duane’s syndrome

Type I is paresis of abduction of one eye associated with narrowing of the palpebral fissure with or without retraction of the globe on attempted adduction. Type II is impaired adduction. Type III is impaired abduction and adduction. The lesion is probably of congenital origin. The retraction syndrome may be due to anomalous innervation of the lateral rectus muscle by the inferior division of the oculomotor nerve.

Foster Kennedy syndrome

Ipsilateral anosmia, optic atrophy, and contralateral papilledema. This is usually due to an olfactory groove meningioma. A pseudo-Foster Kennedy syndrome may be due to sequential AION.

Foville midbrain syndrome

Ipsilateral gaze deviation and contralateral hemiparesis.

Foville syndrome

A pontine lesion which causes an ipsilateral 7th nerve paresis with ipsilateral gaze paresis and contralateral hemiparesis. The eyes look at the hemiparesis.

Gerstmann’s syndrome

Acalculia, finger agnosia, agraphia, and right–left confusion due to a lesion of the dominant angular gyrus.

Gilles de la Tourette syndrome

See Tourette’s syndrome.

Gradenigo’s syndrome

A lesion which affects the ophthalmic division of the trigeminal nerve and the 6th cranial nerve associated with either an infection (petrositis) or neoplasm of the apex of the temporal bone.

Guillain–Barré syndrome

Acute inflammatory demyelinating polyneuropathy (see  Chap. 9, Case 8).

Horner’s syndrome

Ptosis, miosis, and anhidrosis (see  Chap. 8).

Isaac’s syndrome (neuromyotonia)

The prominent feature of neuromyotonia is continuous myokymia especially of distal musculature which persists in sleep. Symptoms include muscle stiffness, cramps, and occasionally hyperhidrosis.

Jugular foramen syndrome

Ninth, 10th, and 11th cranial nerve pathology usually due to a tumor at the foramen.

Kearns–Sayre syndrome

Chronic progressive external ophthalmoplegia (CPEO) beginning in childhood associated with heart block, retinal pigment degeneration, short stature, abnormal muscle mitochondria, spongiform encephalopathy, and occasionally cerebellar ataxia.

Klein−Levin syndrome

Episodes of hypersomnia, hyperphagia, and hypersexual behavior.

Klüver–Bucy syndrome

The lack of capacity for anger or fear, hyperorality, and hypersexuality secondary to bilateral lesions in the frontotemporal parts of the limbic system (amygdala, piriform cortex and adjacent hippocampus).

Korsakoff’s syndrome

Loss of short-term memory plus confabulation. Although it is a well-known complication of chronic alcoholism, it is not a pathognomonic sign and is not infrequent in patients with Alzheimer’s disease.

Lacunar syndromes

Characteristic syndromes associated with small ischemic infarctions in subcortical or brainstem locations. These lacunar infarctions are due to small vessel penetrating artery disease (see  Chap. 7).

Lateral medullary syndrome

See Wallenberg’s syndrome.

Lambert–Eaton syndrome

Disorder of the neuromuscular junction due to decreased release of packets of acetylcholine from the presynaptic terminal caused by an abnormal voltage-dependent calcium channel.

Lance-Adams syndrome

Action myoclonus caused by an hypoxic brain injury.

Landau–Kleffner syndrome

Acquired epileptic aphasia in children.

Lennox–Gastaut syndrome

Childhood onset of multiple seizure types associated with mental retardation which is cryptogenic or symptomatic and often refractory to treatment.

Locked-in syndrome

Quadriplegia, horizontal gaze palsy, preservation of vertical eye movements with normal alertness, and cognition (see  Chap. 5).

Lutz syndrome

See internuclear ophthalmoplegia of abduction.

Man-in-a-barrel syndrome

Bilateral arm paresis with good preservation of leg function.

Marie–Foix syndrome

Ipsilateral cerebellar ataxia, contralateral hemiparesis, and variable contralateral hemihypesthesia. This is due to a lateral pontine lesion involving the middle cerebellar peduncle and adjacent corticospinal tract.

Meige syndrome

Oral mandibular dystonia and blepharospasm.

Melkersson–Rosenthal syndrome

Recurrent unilateral or bilateral facial palsies with fissured (scrotal) tongue and facial swelling.

Möbius syndrome

Congenital lesion of the 6th nucleus causing an abduction paresis. Facial diplegia and occasionally other cranial nerves may be affected.

Munchausen syndrome

These patients simulate illness to obtain medical treatment without an obvious motive.

Neuroleptic malignant syndrome

Hyperthermia, extrapyramidal signs, altered mental status, and elevated CPK due to an idiosyncratic reaction to neuroleptics or an abrupt withdrawal from levodopa treatment.

Neuromyotonia

See Isaac’s syndrome.

Numb-chin syndrome

This is a neuropathy of the mental nerve causing numbness of the chin, occasionally lower lip, and mucus membrane of the inside of the lower lip. It can be a sign of metastatic neoplasm.

One-and-a-half syndrome

This occurs from a unilateral PPRF and adjacent MLF lesion. It causes an ipsilateral gaze palsy and ipsilateral adduction paresis with consequent retention of only abduction of the contralateral eye. Vertical eye movements are usually preserved.

Parinaud’s syndrome

See pretectal syndrome.

Parsonage−Turner syndrome

This is a painful brachial plexitis usually of viral or immune origin.

POEMS syndrome

Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes.

Post-polio syndrome

Recurrent weakness, atrophy, fatigue, and pain occurring 15 years or more after acute poliomyelitis.

Paroxysmal orthostatic tachycardia syndrome

Tachycardia, 30 beats higher than the resting rate, with minimal exertion, and associated with chronic fatigue.

Pretectal syndrome (dorsal midbrain, Sylvian aqueduct and Parinaud’s syndrome)

This includes paresis of upward gaze and, variably, convergence-retractory nystagmus, lid retraction, lid lag, and large round pupils poorly or nonreactive to light. Light-near dissociation may be present.

Piriformis syndrome

Sciatic nerve entrapment as it passes through the greater sciatic notch.

Raeder’s paratrigeminal syndrome

Persistent, unilateral orbital, and eye pain associated with miosis and ptosis.

Ramsay Hunt syndrome

Herpes zoster affecting the geniculate ganglion causing a facial palsy of Bell’s type often with a vesicular eruption on the eardrum and external auditory meatus.

Reflex sympathetic dystrophy

See complex regional pain syndrome.

Rett’s syndrome

This is an X-linked disorder in girls who develop stereotypical behaviors, cognitive impairment, and extrapyramidal manifestations. It is considered an autism spectrum disorder.

Reye’s syndrome

An acute encephalopathy with altered mental status and liver dysfunction manifested by elevated liver enzymes and/or hyperammonemia probably secondary to a virus (influenza A, B and varicella virus) with a concomitant exposure to an exogenous substance, especially aspirin.

Schwartz–Jampel syndrome

An autosomal recessive myotonic disorder in children manifested by continuous motor activity in muscles, especially in the face and thighs, blepharospasm, and constant motor activity of chin and lips.

Serotonin syndrome

Hyperthermia, myoclonus, extrapyramidal signs, and altered mental status secondary to SSRIs.

Shy–Drager syndrome

Parkinsonism plus autonomic dysfunction. This is now encompassed by the term multiple system atrophy (MSA).

Steele–Richardson–Olszewski syndrome

Progressive supranuclear palsy. This combines parkinsonism, vertical gaze paresis (primarily downgaze), dementia, and nuchal rigidity.

Stiff-person syndrome (stiff-man’s)

This motor system disease, occasionally paraneoplastic, is manifested by painful fluctuating rigidity and spasms of axial and limb musculature commonly exacerbated by external stimuli. There are usually markedly increased antibodies to glutamic acid decarboxylase (GAD), an enzyme which breaks down glutamic acid to GABA, an inhibitory neurotransmitter.

Superior orbital fissure syndrome

Findings are similar to the cavernous sinus syndrome since the third, fourth, ophthalmic division of 5th and 6th cranial nerves pass through this fissure. Pain, paresthesias and sensory loss in the ophthalmic division of the 5th nerve, Horner’s syndrome, and exophthalmos may be present.

Sylvian aqueduct syndrome

See pretectal syndrome.

Tarsal tunnel syndrome

Entrapment of the ­posterior tibial nerve at the ankle.

Terson’s syndrome

Vitreous hemorrhage associated with intracranial hemorrhage.

Thalamic syndrome

Severely disagreeable or painful cutaneous symptoms contralateral to a thalamic lesion.

Thoracic outlet syndrome

Symptoms and signs due to compression of the brachial plexus and arteries in that region. The C8 and T1 roots are especially susceptible to damage from fibrous bands over a cervical rib.

Tolosa–Hunt syndrome

Granulomatous inflammation in the cavernous sinus causing a painful ophthalmoplegia with variable involvement of the 3rd, 4th, first division of the 5th and 6th cranial nerves. There is occasional optic nerve involvement and Horner’s syndrome.

Top-of-the-basilar syndrome

Vascular occlusive disease of the rostral portion of the basilar artery, commonly cardioembolic, resulting in midbrain, thalamic, temporal lobe and occipital lobe infarctions, often bilateral.

Vernet’s syndrome

See jugular foramen syndrome.

Vertical one-and-a-half syndrome

Vertical upgaze palsy and monocular paresis of downgaze associated with thalamo-mesencephalic infarctions.

Wallenberg’s syndrome (lateral medullary ­syndrome)

Unilateral infarction of the dorsolateral medulla due to ischemia in the distribution of the posterior inferior cerebellar artery. Characteristic findings are horizontal gaze-evoked nystagmus, ipsilateral Horner’s syndrome, ipsilateral facial hypesthesia, contralateral hemihypesthesia, ipsilateral palate and vocal cord paresis, ipsilateral cerebellar ataxia, and occasional singultus (hiccups).

Wartenberg’s syndrome

Injury to the superficial cutaneous branch of the radial nerve resulting in numbness and paresthesias of the dorsal aspect of the first three fingers and dorsum of the hand, radial portion. This is also known as cheiralgia paresthetica.

Weber’s syndrome

Third nerve palsy and contralateral hemiparesis including the lower face.

Wernicke’s syndrome

Altered sensorium, ataxia, and oculomotor abnormalities due to thiamin deficiency, usually a complication of alcoholism.

West syndrome (infantile spasms, Salaam attacks)

Seizures in infancy manifested by flexor or extensor spasms, mental retardation, and a typical EEG pattern called hypsarrhythmia.

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.St. Luke’s Episcopal Hospital Department of NeurologyUniversity of Texas Medical School at HoustonHoustonUSA

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