Color Atlas of Neurology

Type

Clinical Features

Etiology (examples)

Nociceptive pain (somatic,

Paresthesia, allodynia,4 loss of sensa-

Meralgia paresthetica, carpal tunnel

p. 110)3

tion, readily localizable

syndrome, skin lesion

Neuropathic pain, neural-

Severe pain in nerve distribution,

Mononeuritis, polyneuropathy,

gia (pp. 186, 318ff.)

paresthesiae, allodynia, sensory loss,

trauma, nerve compression, trigemi-

pain on nerve pressure, readily localiz-

nal neuralgia, neuroma

able

Radicular pain (p. 319 f)

Same as above + aggravated by

Herniated intervertebral disk, poly-

stretching (e. g., Lasègue sign) or

radiculitis, leptomeningeal

movement

metastases, neurofibroma/schwan-

noma

Referred pain

See p. 110

See p. 110

Deafferentation pain,

Pain in an anesthetic or analgesic

Plexus lesion, radicular lesion, trigemi-

anesthesia dolorosa

nerve territory

nal nerve lesion

Phantom limb pain

Pain felt in an amputated limb

Limb amputation

Central pain

Burning, piercing pain in the region of

Cerebral infarct, hemorrhage, or

a neurological deficit; imprecisely lo-

tumor (cortex, thalamus, white mat-

calizable; frequently accompanied by

ter, internal capsule), brain stem, spi-

sensory dissociation, dysesthesia,

nal cord; syrinx, trauma, multiple

paresthesia; triggered by stimuli

sclerosis (brain stem, spinal cord)

Chronic pain

Pain that lasts !6 months, impairs

Sensitization of nociceptors? Transsy-

(“pain disease”)

social contacts, emotional state, and

naptic neuropeptide induction (calci-

physical activity

tonin gene-related peptide = CGRP,

substance P = SP, neurokinin A =

NKA)?

Psychogenic pain

Discrepancy between symptoms and

Mental illness

organ findings and/or syndrome

classification

Stage

EEG1

EOG2

Awake

α activity (8–13 Hz)

Blinks, saccades

NREM stage 1

Increasing θ activity

Slow eye movements5

(2–7 Hz), vertex waves4

NREM stage 2

θ activity, sleep spindles,6

No eye movement until

K-complexes7

stage 4, EEG artifact

NREM stage 3

Groups of high-amplitude δ

waves (0.5–2 Hz, amplitude

! 0.75 µV)

NREM stage 4

Groups of high-amplitude δ

waves

REM sleep

θ activity, saw-tooth waves8

Conjugate, rapid eye move-

may be observed

ments (episodic)

Acute, severe brain damage (either primary or secondary)

Exclusion of other causes1

Coma

Absent light reflex, moderately to maximally dilated pupils2

Absent oculocephalic reflex3

Absent corneal reflex3

Absent response to painful stimulation in trigeminal distribution

Absent pharyngeal and tracheal reflexes3

Absence of spontaneous breathing4

Required time of observation5

Supratentorial primary brain damage

Adults and children over 2 years of age at least 12 hours

Children under 2 years of age at least 24 hours6

Neonates at least 72 hours6

Infratentorial primary brain damage

Same as supratentorial damage, but with at least 1 additional examination6

Secondary brain damage

Adults and children over 2 years of age at least 72 hours

Supplementary criteria7

Isoelectric EEG

Absence of evoked potentials8

Absence of cerebral blood flow

Steps

Measures/Objective

Parameters To Be Measured

Table 12 Sites and manifestations of lesions causing dysarthria (p. 130)

Site of Lesion

Manifestations

Periphery2

Slurred speech (impaired labial/lingual articulation,

rhinolalia = “speaking through nose,” unclear differ-

entiation of vowels and consonants), dyspnea, whis-

pering (recurrent laryngeal nerve paralysis), hoarse-

ness (laryngitis, vocal cord polyp, extubation)

Cerebellum/

Ataxic dysarthria (clipped, scanning speech), articula-

brain stem

tion problems. Hoarse, deep voice (vagus nerve le-

(pp. 54, 70)

sion)

Basal ganglia

Hypophonia (p. 206, monotonous, soft, slurred).

(nonpyramidal

Spasmodic dysphonia (p. 64). Hyperkinetic speech

dysarthria)

(p. 66; explosive, loud, uncoordinated, clipped

speech)

White matter/

Monotonous, slow, hoarse, pressured speech. Deep,

cortex

variable pitch. Poor articulation

Diffuse

Slurred, effortful, slow speech

Type of Amnesia

Manifestations

Transient global

Acute onset, limited duration. Patient repeats

amnesia2

questions (e. g., “What am I doing here?”), is

helpless, anxious; can perform everyday activi-

ties. Anterograde/retrograde amnesia

Acute transient

Anterograde/retrograde amnesia; manifestations

amnesia

of underlying disease

Acute persistent

Anterograde/retrograde amnesia; manifestations

amnesia

of underlying disease

Subacute per-

Many patients are initially confused, with antero-

sistent amnesia

grade/retrograde amnesia; manifestations of un-

derlying disease

Chronic progres-

Anterograde amnesia; retrograde amnesia

sive amnesia

develops in the course of the condition; manife-

stations of underlying disease

Cause

Diagnostic criteria1

Alzheimer disease (p. 297 f)

Mainly temporal and parietal lobe degeneration. Rarely hereditary (au-

tosomal dominant). No focal neurological deficit. CSF: increased

acetylcholine, A!-amyloid, and τ protein levels. Cognitive deficits: An-

terograde amnesia, amnestic aphasia, acalculia, impaired visuospatial

performance. Behavioral changes: Hardly any at first; later anosognosia

or dissimulation, paranoia, disturbance of sleep–wake cycle.

Vascular dementia (p. 298)

Subcortical vascular demyelination due to multiple infarcts, lacunes,

vasculitis or CADASIL. Fluctuating course. Focal neurological signs:

Hemiparesis, aphasia, apraxia, gait impairment, bladder dysfunction,

Babinski sign. Pseudobulbar palsy (bilateral lesion of the corticobulbar

tracts dysarthrophonia or anarthria, dysphagia, lingual/facial paraly-

sis, loss of emotional control with outbursts of laughing or crying).

Cognitive deficits: Memory deficit (“forgetting to remember”), frontal

brain dysfunction (p. 122). Behavioral changes: Sluggishness, reduced

drive, disturbance of sleep–wake cycle

Depression

Psychomotor slowing, reduced drive, and anxiety suggest dementia,

which is not, in fact, present (pseudodementia). See Table 42, p. 383

Alcohol

Korsakoff syndrome: Disorientation/amnesia, confabulation

Hydrocephalus

Normal pressure hydrocephalus (p. 160)

Metabolic/endocrine disorders

Wilson disease, hypothyroidism, hypopituitarism, hepatic/uremic en-

cephalopathy, hypoglycemia, vitamin B12 deficiency, Wernicke en-

cephalopathy, pellagra, hypoxia, hypoparathyroidism or hyperparathy-

roidism, adrenocortical insufficiency, Cushing syndrome, acute inter-

mittent porphyria (p. 332)

Tumor

See p. 254 ff

Degenerative diseases

Parkinson disease (p. 206 ff), atypical parkinsonism (p. 302), Hunting-

ton disease (p. 300), frontotemporal dementia (p. 298), hereditary

ataxia (p. 280), motor neuron disease (p. 304), multiple sclerosis

Infectious diseases (p. 222 ff)

HIV and other viral encephalitides, prion diseases, neurosyphilis,

Whipple disease, brain abscess, neurosarcoidosis, subacute sclerosing

panencephalitis

Trauma

Chronic subdural hematoma, posttraumatic phenomenon, punch-

drunk syndrome (dementia pugilistica)

Toxic

Drugs, substance abuse, heavy metal poisoning, organic toxins

Table 15 The hypothalamic-pituitary regulatory axis (p. 142)

Controlled

Hormones

Stimulus

Effect

Water balance and

ADH

Renal water reabsorption

blood pressure

and arterial vasoconstric-

sipidus;

ADH: ectopic

tion (at higher ADH

levels)

(p. 310)

Triiodothyronine

TRH, TSH

TRH2 increase/decrease

(T3), thyroxine (T4)

TSH3

Cortisol

CRH,

/ Cortisol

CRH5 increase/decrease

ACTH4

drome;

ACTH: second-

ciency

Testosterone (man)

GnRH,

GnRH7 increase/decrease

LH, FSH6

Estradiol, pro-

GnRH,

/

GnRH increase/decrease

gesterone

LH, FSH

(woman)

Prolactin (PRL)

PRL

Dopamine8

VIP9, TRH

Growth

GHRH,

GHRH11

(somatomedins)

GH10

Somatostatin12

Endogenous opioid

!-endorphin

Analgesia, food intake,

peptides

(pituitary

thermoregulation, learn-

gland)

ing, memory

Symptoms and Signs

Disturbances of consciousness, attention,

perception, memory, sleep–wake cycle,

and cognition. Visual hallucinations. Fluc-

tuating motor hypoactivity and hyperac-

tivity. Affective disturbances (anxiety, de-

pression, irritability, euphoria, helpless-

ness)

Uncontrollable emotional outbreaks. Seen

in central paralysis (pseudobulbar palsy,

amyotrophic lateral sclerosis, multiple

sclerosis) and focal epilepsy (gelastic

seizures). Stroke prodrome

Aggression with minimal or no provoca-

tion, seen in focal epilepsy, head trauma,

hypoxic encephalopathy, brain tumor,

herpes simplex encephalitis, rabies, cere-

bral infarction or hemorrhage, hypogly-

cemia, intoxication (drugs, alcohol)

Usually due to a primary illness such as

Alzheimer or Pick disease, herpes simplex

or AIDS encephalitis, hypoxic en-

cephalopathy, cerebral infarction or

hemorrhage

Korsakoff syndrome: impairment of short-

term memory and sense of time. Other

cognitive functions and consciousness are

unimpaired

Hypersexual behavior: after head trauma

or stroke, or as a side effect of dopamin-

ergic antiparkinsonian medication.

Diminished libido: depression, medica-

tions

Test

Method

Normal Findings

Tests of sympathetic function

Schellong test (orthostasis

Measure heart rate and blood pressure

Heart rate rises by no more

test)

once per minute for 10 minutes with the

than 20 beats/min; systolic

patient supine, then 5 minutes with the

blood pressure drops no more

patient erect (tilt table if necessary)

than 10 mmHg, diastolic no

more than 5 mmHg

Valsalva maneuver (VM)

The patient inspires deeply and tries to

During VM, the blood pressure

exhale against resistance (up to

does not drop below 50% of

40 mmHg) for 10–15 seconds. The blood

initial value; after VM, the

pressure is measured before, during and

blood pressure rebounds

after the VM, or continuously

above the initial value (+ reflex

bradycardia)

Hand grip test

Isometric muscle contraction (hand grip)

Diastolic blood pressure

30% of maximal force for 3 minutes

!15 mmHg

Tests of parasympathetic func-

tion

Schellong test with 30/15

Continuous ECG recording

Quotient of R-R interval of

quotient

30th and 15th heart beat after

standing up is !1

Respiratory sinus arrhythmia

ECG recorded with patient supine and

The quotient of the longest

(respiratory test)

breathing maximally deeply at 6/min for a

(expiration) and shortest R-R

total of 8 cycles. Repeat after a period of

intervals is normally !1.2

rest

(age-dependent)

VM with ECG

Continuous ECG recording

Quotient of longest and short-

est R-R interval is !1 (age-de-

pendent)

Carotid sinus massage1

Perform unilateral carotid sinus massage

Reflex decrease in heart rate

for 10–20 seconds while continuously

and blood pressure

monitoring blood pressure and ECG with

emergency resuscitation equipment ready

Site of lesion

Causes

Brain stem, upper cervical spinal cord

Tumor, infarction, hemorrhage, meningoencephalitis (Lis-

teria, poliomyelitis), trauma, multiple sclerosis, intoxica-

tion, parkinsonism (rigidity of respiratory muscles)

Motor anterior horn cell

Amyotrophic lateral sclerosis, tetanus, poliomyelitis, post-

polio syndrome

Peripheral nerve

Guillain–Barré syndrome, phrenic nerve lesion

Neuromuscular

Myasthenia gravis, botulism, Lambert–Eaton syndrome,

muscular dystrophy, polymyositis, acid maltase deficiency,

electrolyte imbalance (Na , K , Ca , phosphate , Mg )

Hyperventilation

Pneumonia, pulmonary embolism, asthma, acidosis (dia-

betic, renal, lactate ), meningoencephalitis, brain tumor,

fever, sepsis, salicylates, anxiety, pain, psychogenic

GI Syndrome1

Manifestations

Dysphagia

See p. 102

Table 8

Gastroparesis

Delayed gastric emptying

Diabetes mellitus, amyloidosis, paraneoplastic syn-

nausea, vomiting, anorexia, bloat-

drome, dermatomyositis, Duchenne-type muscular

ing

dystrophy

Intestinal

Impaired intestinal motility

Parkinson disease, multiple sclerosis, transverse spi-

pseudo-

nausea, vomiting, bloating,

nal cord syndrome, Guillain–Barré syndrome, dia-

obstruction

weight loss, impairment of

betes mellitus, botulism, amyloidosis, paraneoplastic

peristalsis

syndrome, drugs (tricyclic antidepressants, codeine,

morphine, clonidine, phenothiazines, anticholiner-

gics, vincristine), Hirschsprung disease

Constipation3

Highly variable. Infrequent hard

Lack of exercise, dysphagia, poor nutrition, trans-

stools, straining, bloating, sensa-

verse spinal cord syndrome, head trauma, brain stem

tion of incomplete defecation,

lesions, Parkinson disease, multiple system atrophy,

pain, flatulence, belching

multiple sclerosis, diabetes mellitus, porphyria, drugs

(morphine, codeine, tricyclic antidepressants)

Diarrhea4

Defecation rate, liquid stools,

Diabetes mellitus, amyloidosis, HIV infection, drugs,

tenesmus

Whipple disease

Vomiting5

Gagging, yawning, nausea, hyper-

Intracranial hypertension (p. 158), vertigo (p. 58), mi-

salivation, pale skin, outbreaks of

graine, infectious and neoplastic meningitis, drugs

sweating, apathy, low blood pres-

(digitalis, opiates, chemotherapeutic agents), intox-

sure, tachycardia

ication

Anal inconti-

Complete or partial loss of control

Diabetes mellitus, multiple sclerosis, spinal cord le-

nence

of defecation

sions, lesion of the conus medullaris or cauda equina,

dementia, frontal lobe lesions (tumor, infarction)

Site of Neuro-

Neurological Condition

Type of Bladder Dysfunction

logical Lesion

Detrusor hyperreflexia, bladder hypocontractility

Frequency , urge , urge incontinence

Usually a late manifestation: frequency , urge incon-

Frequency , urge , imperative urinary urge, urge

incontinence, detrusor hyperreflexia, DSD4

Frequency , urge incontinence, detrusor hyper-

Nocturia, frequency , urge incontinence, impairment

of voluntary voiding

Lesion above S2 (“reflex bladder”) hyperreflexia,

Lesion of sacral micturition center (“autonomic blad-

der”) residual urine, detrusor areflexia, impaired

Residual urine, detrusor areflexia, impaired bladder

reflex, impaired filling sensation, frequency

Pathogenetic Mechanism

Causes

Mass lesion

Hematoma (epidural, subdural, intracerebral), brain tumor/

metastasis, brain abscess

CSF outflow obstruction

Hydrocephalus

Increased brain volume

Pseudotumor cerebri, infarct, global hypoxia or ischemia,

hepatic encephalopathy, acute hyponatremia

Increased brain volume and increased

Head trauma, meningitis, encephalitis, eclampsia, hyper-

intravascular blood volume

tensive encephalopathy, venous sinus thrombosis

Pathogenetic

Causes

Impaired spinal

Absence of pressure rise in

Tumor, arachnoiditis, herniated intervertebral disk

CSF circulation1

Queckenstedt test

CSF leak

Spinal dural defect, rhinor-

Prior LP2, trauma, neurosurgical procedures, tumor,

rhea, otorrhea

osteomyelitis

Dehydration

Thirst, dry skin, fatigue,

Isotonic, hypotonic: Vomiting, diarrhea, diuretics. Hyper-

low blood pressure, light-

tonic: Thirst, profuse sweating, mannitol administration

headedness or uncon-

sciousness

Spontaneous

Low ICP

Spinal CSF fistula (?)

Cause

Atheroembolic/thromboembolic (e. g., internal carotid artery dissection/stenosis),

cardioembolic (right–left shunt, e. g., in patent foramen ovale, thrombus in atrial

fibrillation, mitral valve defect, acute myocardial infarction, endocarditis, artificial

heart valve)

Low perfusion pressure (orthostatic hypotension, arteriovenous shunt, intracranial

hypertension, glaucoma); high perfusion resistance (migraine, glaucoma, malig-

nant arterial hypertension, increased blood viscosity, retinal venous thrombosis,

vasospasm)

Retinal detachment, paraneoplastic (p. 388), chorioretinitis, blow to eye

Tumor, subluxation of lens, vitreous body hemorrhage

Vascular (ischemia, arteritis [p. 180], malignant arterial hypertension), papil-

ledema, retrobulbar neuritis (Uhthoff sign, p. 216)

Blowing the nose, malaria, pregnancy, hypersensitivity to cold, interleukin-2,

acute stabbing pain, sinus lavage

(Gautier, 1993; Warlow et al., 2001)