Color Atlas of Neurology
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Appendix |
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Table 42 Criteria for differentiation between dementia and depression (p. 297) |
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Dementia |
Depression |
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Patients seem indifferent to memory impairment; |
Patients describe memory impairment precisely and |
semantic paraphasia |
in detail |
Tests reveal cognitive deficit |
Tests reveal minimal or no cognitive deficit |
Depressive manifestations develop slowly (second- |
Depressive manifestations prominent on presenta- |
ary) |
tion (brooding, anxiety, early awakening, loss of ap- |
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petite, self-doubt) |
Rare past history of depression |
Frequent past history of depression |
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Table 43 Criteria for differentiation between different types of hyperkinesia (p. 300)
Syndrome |
Features |
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Chorea (p. 66) |
Overshooting, spontaneous, abrupt, alternating, irregular movements. Promi- |
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nence varies from restlessness with little gesticulation, fidgety hand movements |
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and hesitant, dance-like gait impairment to continuous, flowing, violent, disabling |
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hyperkinesias |
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Appendix |
Dystonia (p. 64) |
Involuntary, continuous and stereotyped muscle contractions that lead to rotat- |
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ing movements and abnormal posture |
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Athetosis (p. 66) |
Localized peripheral dystonic movements |
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Ballismus (p. 66) |
Violent, mainly proximal flinging movements of the limbs |
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Tics (p. 68) |
Repetitive, stereotyped, localized twitches that can be voluntarily suppressed, but |
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with a build-up of inner tension |
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Myoclonus (p. 68) |
Brief, sudden, shocklike muscle twitches occurring repetitively in the same |
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muscle group(s) |
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(Harper, 1996) |
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Table 44 Symptomatic forms of parkinsonism (p. 302)
Cause |
Examples |
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Infectious disease |
Encephalitis lethargica1 (von Economo postencephalitic parkinsonism), measles, |
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tick-borne encephalitis, poliomyelitis, cytomegalovirus, influenza A, herpes sim- |
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plex |
Intoxication |
MPTP2, manganese (miners, industrial workers), carbon monoxide, methanol |
Drugs3 |
Neuroleptics (phenothiazines4, butyrophenones5, thioxanthenes6, benzamide7), |
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reserpine, calcium channel blockers (cinnarizine, flunarizine) |
Other diseases8 |
Multiple brain infarcts/subcortical arteriosclerotic encephalopathy9, punch-drunk |
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encephalopathy (dementia pugilistica), normal pressure hydrocephalus (p. 160), |
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brain tumor (frontal), subdural hematoma, calcification of basal ganglia10, neu- |
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roleptic malignant syndrome11 |
1 In the aftermath of the influenza pandemic that followed the First World War (now only of historical interest). 2 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine. MPTP is converted into MPP+, which accumulates in dopaminergic neurons and interferes with electron transfer in the mitochondrial respiratory chain, leading to an accumulation of free radicals and neuronal death. An outbreak of MPTP-induced parkinsonism occurred in California in the early 1980s, when this substance appeared as a contaminant of opiate drugs synthesized in clandestine laboratories for illegal use. 3 Parkinsonoid. 4 Fluphenazine, levomepromazine, perazine, perphenazine, promazine, triflupromazine, etc. 5 Benperidol, fluspirilene (diphenylbutylpiperidine), haloperidol, etc. 6 Chlorprothixene, clopenthixol, fluanxol. 7 Metoclopramide. 8 Includes the terms pseudoparkinsonism, hypokinetic-rigid syndrome, and
hypertonic-hyperkinetic syndrome. 9 Lower-body parkinsonism. 10 Autosomal recessive (Fahr disease), associated 383 with hypoparathyroidism and pseudohypoparathyroidism. 11 Parkinsonian hyperthermia syndrome: rigidity, hy-
perthermia, impairment of consciousness; induced by neuroleptic drugs or by the use or withdrawal of levodopa or other dopaminergic agonists.
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Appendix
384
Appendix
Table 45 Diseases affecting the first (upper) motor neuron (p. 304)
Syndrome |
Features |
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Hereditary |
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Familial spastic paraple- |
Uncomplicated SPG1,2: SPG26 (X/Xq22/PLP = proteolipid protein), SPG3A (AD/ |
gia (SPG, p. 286) |
14q11.2–24.3/atlastin), SPG46 (AD/2p22-p21/spastin), SPG5A (AR/8p12-q13/?), |
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SPG6 (AD/15q11.1/?), SPG76 (AR/16q24.3/paraplegin), SPG8/AD/8q23–24/?), |
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SPG10 (AD/12q13/?), SPG11 (AR/15q13–15/?), SPG12 (AD/19q13/?), SPG13 (AD/ |
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2q24–34/?) |
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Complicated SPG3: SPG16 (X/Xq28/L1-CAM = L1 cell adhesion molecule), SPG26 |
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(X/Xq22/PLP), SPG76 (AR/16q24.3/paraplegin), SPG9 (AD/10q23.3–24.1/?), SPG14 |
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(AR/3q27–28/?), SPG16 (X/Xq11.2/?) |
Adrenomyelo- |
X-linked recessive, onset usually after age 20. Progressive spastic paraparesis, |
neuropathy4 |
polyneuropathy, urinary incontinence, sometimes hypocortisolism. A similar |
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syndrome develops in 20% of all female heterozygotes (carriers) |
Spinocerebellar ataxia |
See p. 280 |
type 3 |
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Acquired |
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Primary lateral sclerosis |
Onset usually after age 50. Slowly progressive symmetrical paraspasticity without |
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marked weakness, dysarthria. More common in men than in women |
Lathyrism (p. 304) |
Onset usually before age 50. Subacute or chronic development of gait distur- |
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bances (tip-toe/scissors gait, dorsal tilting of trunk), leg cramps, paresthesiae, uri- |
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nary retention |
Tropical spastic para- |
Onset: Slow up to age 60. Back pain, dysesthesiae, spastic paraparesis, urinary re- |
paresis (TSP)5 |
tention, impotence |
1 Abbreviations: AD = autosomal dominant, AR = autosomal recessive, X = X-chromosome/gene locus/gene product (? = unknown). 2 Isolated progressive spastic paraparesis. 3 As in uncomplicated SPG with additional manifestations including cerebellar ataxia, dystonia, optic neuropathy, tapetoretinal degeneration, muscle atrophy, dysarthria, deafness, sensory neuropathy, ichthyosis, dementia. 4 Impaired !-oxidation of very long chain fatty acids (VLCFA; C24–26) due to defective peroxisomal transport accumulation of VLFCA in nervous system, adrenal cortex, plasma. 5 HTLV-I-associated myelopathy (= HAM, human T-cell/lymphotropic virus type I); Jamaica, Japan, Caribbean. 6 Molecular genetic tests are available.
Rohkamm, Color Atlas of Neurology © 2004 Thieme
All rights reserved. Usage subject to terms and conditions of license.
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Appendix |
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Table 46 Diseases affecting the second (lower) motor neuron (p. 304) |
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Syndrome1 |
Features |
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Hereditary |
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Proximal2 (SMA)3 |
SMA I4: onset !3 months. AR. Flaccid quadriparesis5. Triangular mouth shape, |
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paradoxical respiratory movements, impaired sucking ability, unable to sit |
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SMA II: onset !5 years. AR. The children learn to sit independently, but never to |
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stand/walk. Scoliosis, joint contractures |
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SMA III6; SMA IIIa: onset 3 years. AR. Delayed motor development. Children learn |
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to stand/walk |
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SMA IIIb: onset 3–30 years. AR. Development normal. Calf (pseudo)hypertrophy. |
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Absence of bulbar muscle involvement. CK7 sometimes elevated |
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SMA IV: onset "30 years. AR |
Nonproximal8 SMA |
Distal SMA9: forms with different ages of onset (infantile, juvenile, adult). Usually |
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slow course, sometimes stabilizing after a few years, sometimes progressive. May |
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be accompanied by myoclonus, deafness, dysphonia, dysarthria, and/or ataxia. |
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Scapuloperoneal muscular atrophy10: Onset: Adolescence or adulthood. Weakness |
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foot dorsiflexors, shoulder girdle, arm |
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Progressive bulbar palsy11: Onset in adulthood12. Progressive weakness of bulbar |
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muscles. Muscular atrophy and respiratory muscle involvement develop as the |
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disease progresses |
Spinobulbar muscular |
Onset: 20–70 years. Gynecomastia, gradual progression of muscular atrophy (legs |
atrophy (Kennedy type) |
"arms, proximal "distal, asymmetrical; dysarthria, dysphagia, tongue atrophy). |
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Slight elevation of CK. Gene locus Xq12 |
Acquired |
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Acute viral infection |
Poliomyelitis (p. 242), other enteroviruses (e. g., echovirus, coxsackievirus, enter- |
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ovirus type 70/71 acute hemorrhagic conjunctivitis), mumps virus |
Postpolio syndrome |
See p. 242 |
Lymphoma |
Accompanies Hodgkin and non-Hodgkin lymphomas. Elevation of CSF protein, |
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oligoclonal IgG in CSF |
Radiation-induced |
Develops months to years after irradiation of para-aortic lymph nodes (testicular |
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tumors, uterine carcinoma). May progress rapidly |
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(Tandan, 1996; Rudnik-Schöneborn, Mortier and Zerres, 1998)
AR = autosomal recessive, AD = autosomal dominant; XR = X-linked recessive.
1 Selected syndromes. 2 Proximal muscle involvement. 3 SMA = spinal muscular atrophy; gene locus for SMA I to III: 5q12.2–q13.3. 4 AR; infantile SMA, Werdnig–Hoffmann disease. 5 Floppy baby syndrome, froglike posture in supine position; lack of head control. 6 AR "AD; juvenile SMA; Kugelberg–Welander disease. 7 Creatine kinase (CK). 8 Distal or localized (monomelic segmental SMA) muscle groups, symmetrical or asymmetrical involvement. 9 AR "AD. 10 AD Onset age 30–50 years, slowly progressive; AR onset !5 years; may progress slowly. 11 AD/AR. 12 Fazio–Londe type with onset at age 2–13 years, rapidly progressive
Appendix
385
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Appendix
Appendix
Table 47 Diseases affecting both the first (upper) and the second (lower) motor neurons (p. 304)
Diagnostic Categories1
Definite ALS2 |
Evidence of first + second motor neuron lesion in 3 regions of the body3 |
Probable ALS |
Evidence of first + second motor neuron lesion in 2 regions of the body |
Possible ALS |
Evidence of first + second motor neuron lesion in 1 region of the body or evi- |
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dence of first motor neuron lesion in 2 to 3 regions of the body |
Suspected ALS |
Evidence of second motor neuron lesion in 2 to 3 regions of the body |
Diagnostic features |
Progressive symptoms of a first (p. 46) and second motor neuron lesion (p. 50). |
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Fasciculation in more than one region of the body. Neurogenic EMG findings, nor- |
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mal nerve conduction velocity/absence of motor conduction block. Absence of |
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sensory deficits, sphincter dysfunction, visual disturbances, autonomic dysfunc- |
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tion, parkinsonism, and Alzheimer, Pick or Huntington disease |
Syndromes with manife- |
Cervical radicular syndromes, cervical myelopathy, monoclonal gammopathy. |
stations similar to those |
Multifocal motor neuropathy (GM1 antibodies). Lymphoma; paraneoplastic syn- |
of ALS |
drome; hyperthyroidism, hyperparathyroidism; diabetic amyotrophy; postpolio |
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syndrome; hexosamidinase A deficiency. Radiation-induced lesion. Toxicity (lead, |
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mercury, manganese). Myopathy (inclusion body myositis, polymyositis, muscular |
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dystrophy). Spinal muscular atrophy. Creutzfeldt–Jakob disease |
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1 According to Leigh and Ray-Chaudhuri (1994). 2 ALS = amyotrophic lateral sclerosis. 3 Brain stem, proximal/distal arm, chest, proximal/distal leg.
Table 48 Neonatal metabolic encephalopathies (from birth to 28 days, p. 306)
Syndrome |
Defect/Enzyme Defect |
Symptoms and Signs |
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Galactosemia |
Galactose-1-phosphate |
Milk intolerance, apathy, jaundice, anemia, cata- |
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uridyltransferase1 |
ract, psychomotor retardation |
Nonketonic hyperglycine- |
Defective conversion of |
Hypotonia, dyspnea, myoclonus, generalized |
mia |
glycine to serine |
seizures |
Hyperammonemia |
Urea cycle2 |
Crisislike episodes of vomiting, sucking weakness, |
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somnolence, coma, seizures, hyperpnea, hyper- |
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pyrexia |
Maple syrup urine disease |
Defective breakdown of |
Hypotonia, seizures, coma, ketoacidosis |
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branched-chain amino |
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acids |
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Zellweger syndrome |
Peroxisomes3 |
Hypotonia, sucking weakness, nystagmus, |
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seizures, craniofacial dysmorphism |
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1 Multiple types high galactose-1-phosphate levels. 2 Defects of all six enzymes of the urea cycle are known. Adult onset is rare. Hyperammonemia in defects of carbamoyl-phosphate synthetase, ornithine carbamoyltransferase, argininosuccinic acid synthetase (citrullinemia), argininosuccinase. Arginase defect leads to arginemia. 3 Cytoplasmic organelles that mediate fatty acid oxidation, biliary acid and cholesterol synthesis, pipecolic and phytanic acid metabolism, and plasmalogen (myelin) synthesis. Other peroxisomal syndromes: neonatal adrenoleukodystrophy, infantile Refsum disease, hyperpipecolatemia.
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All rights reserved. Usage subject to terms and conditions of license.
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Appendix |
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Table 49 Metabolic encephalopathies of infancy (first year of life, p. 306) |
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Syndrome |
Defect/Enzyme Defect |
Symptoms and Signs |
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Tay–Sachs disease1 |
Hexosaminidase A ( ac- |
Abnormal acoustic startle reaction, delayed |
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cumulation of ganglioside |
development. Begins with muscular hypotonia, |
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GM2) |
followed by spasticity, seizures, blindness, |
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dementia, and optic nerve atrophy2 |
Gaucher disease3 (type II, |
Glucocerebrosidase ( |
Loss of motor control, apathy, dysphagia, retro- |
acute neuropathic) |
lipid storage) |
flexion of the head, strabismus, splenomegaly |
Niemann–Pick disease4 |
Sphingomyelinase defi- |
Enlargement of spleen, liver and lymph nodes; |
(type A) |
ciency (sphingomyelin |
pulmonary infiltrates, spasticity, muscular axial |
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storage Niemann–Pick |
hypotonia, blindness, nystagmus, macular cherry |
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cells) |
red spot |
GM1 gangliosidosis |
!-Galactosidase |
Craniofacial dysmorphism. Initial flaccid paralysis |
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that later becomes spastic; loss of visual acuity, |
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nystagmus, strabismus, hepatomegaly |
Krabbe disease (globoid |
!-Galactocerebrosidase |
General muscular hypertonia, vomiting, opistho- |
cell leukodystrophy) |
(galactocerebroside ) |
tonus, spasticity, blindness, deafness |
Pelizaeus–Merzbacher |
Proteolipid protein synthe- |
Nystagmus, ataxia, psychomotor retardation, |
disease5 |
sis |
choreoathetosis |
1 GM2 gangliosidosis. 2 Cherry red spot in optic fundus is found in over 90% of cases. 3 Glucocerebrosidase; three known subtypes type 1: nonneuropathic (juvenile) form with hematological changes and bone fractures; type 3: see p. 307. 4 Different types (A, B, C) exist. Type B does not produce neurological symptoms. 5 Other sudanophil (orthochromatic) forms of leukodystrophy are known.
Table 50 Stages of hepatic/portosystemic encephalopathy (p. 308)
Stage |
Behavior1 |
Motor function |
EEG2 |
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I |
Attention deficit, impaired |
Handwriting illegible, asterixis |
Usually normal to θ waves |
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concentration, euphoria or de- |
+/– |
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pression, dysarthria, insomnia |
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II |
Sleepy, marked behavioral |
Asterixis |
Pathological (δ waves) |
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changes (confusion, disorien- |
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tation, apraxia) |
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III |
Severely confused, somnolent |
Asterixis |
Pathological (δ/triphasic |
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to soporific |
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waves) |
IV |
Coma |
Presence (stage IVa) or ab- |
Pathological (triphasic/ |
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sence (stage IVb) of motor re- |
arrhythmic δ/sub-δ waves) |
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sponses to pain |
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(Adams and Foley, 1952) 1 Earlier stages are assessed with psychometric methods, e. g., number connection test (time required for patient to connect 25 numbered circles in numerical order), ability to draw a five-pointed star. 2 Nonspecific changes; actual findings may differ.
Appendix
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Appendix
388
Appendix
Table 51 Paraneoplastic syndromes of the CNS (p. 312)
Site |
Syndrome Time |
Symptoms and |
Common Tumors |
Lesions/Antibodies |
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course |
Signs |
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Cerebrum |
Photoreceptor/retinal |
Progressive blindness |
Small-cell lung cancer |
Loss of photorecep- |
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degeneration |
without pain |
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tors/anti-CAR1 |
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weeks to months |
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Limbic encephalitis |
Restlessness, confu- |
Small-cell lung cancer |
Neuronal loss, peri- |
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weeks to months |
sion, memory impair- |
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vascular and mening- |
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ment |
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eal lymphocytic infil- |
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trates medial tem- |
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poral lobe, limbic sys- |
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tem/ANNA-12 |
Cerebel- |
Brain stem encephali- |
Dysphagia, dys- |
Small-cell lung cancer |
Neuronal loss, inflam- |
lum, brain |
tis days to weeks |
arthria, nystagmus, |
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matory infiltrates in |
stem |
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diplopia, ataxia, dizzi- |
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the brain stem |
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ness |
Small-cell lung |
Death of Purkinje |
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Subacute cerebellar |
Cerebellar ataxia, |
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degeneration |
dysarthria, nystag- |
cancer, carcinoma of |
cells/APCA3 |
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weeks to months |
mus, diplopia, ver- |
ovary/breast, Hodg- |
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tigo |
kin disease |
Neuronal loss in den- |
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Opsoclonus-myo- |
Abrupt, irregular eye |
Neuroblastoma |
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clonus weeks |
and muscle move- |
(children); breast |
tate nucleus |
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ments, cerebellar |
cancer/lung cancer |
(adults)/ANNA-24 |
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ataxia, encephalo- |
(adults) |
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pathy |
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Spinal |
Necrotizing my- |
Flaccid para-/ quadri- |
Small-cell lung |
Necrosis of white and |
cord |
elopathy hours, |
paresis, bladder/ |
cancer, lymphoma |
gray matter of spinal |
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days to weeks |
bowel dysfunction, |
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cord |
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segmental sensory |
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Stiff man syndrome5 |
loss |
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Painful muscular |
Small-cell lung |
Transitory high-cervi- |
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days to weeks |
rigidity initially trig- |
cancer, Hodgkin lym- |
cal lesions may be |
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gered by emotional, |
phoma, breast |
seen in MRI scans/ |
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acoustic and/or tac- |
cancer, pharyngeal |
anti-GAD6 |
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tile stimuli; auton- |
carcinoma |
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omic dysfunction |
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(Brown, 1998) |
1 CAR = cancer-associated retinopathy. 2 ANNA-1 = antineural nuclear antibody type 1 = anti-Hu. 3 APCA = antiPurkinje cell cytoplasmic antibody = anti-Yo. 4 ANNA-2 = antineural nuclear antibody type 2 = anti-Ri. 5 Manifestations variable (e. g., axial or distal muscle may be more prominent); attributed to diminished supraspinal inhibition of motor neurons, leading to continuous contraction of agonists and antagonists. 6 Anti-GAD = glutamic acid decarboxylase antibodies; antibodies directed against amphiphysin (terminal synaptic protein) have also been found.
Rohkamm, Color Atlas of Neurology © 2004 Thieme
All rights reserved. Usage subject to terms and conditions of license.
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Appendix |
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Table 52 Iatrogenic encephalopathies (p. 314) |
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Substance |
Adverse Effects1 |
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Neuroleptics |
Drug-induced parkinsonism (p. 383, Table 44), early/late dyskinesia (p. 66), |
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akathisia2, low seizure threshold |
Antidepressants |
Somnolence, increased drive, confusion, akathisia, low seizure threshold, tremor, |
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serotonin syndrome3 |
Aspirin4 |
Tinnitus, dizziness |
Baclofen |
Fatigue, depression, headaches, low seizure threshold |
Levodopa, dopamine |
Confusion, hallucinations, psychosis, insomnia, hyperkinesia |
agonists |
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Corticosteroids, ACTH |
Depression, increased drive, mania, insomnia, headaches, dizziness, sweating, low |
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seizure threshold, tremor |
Antibiotics |
Aminoglycosides: Tinnitus, hearing impairment. Quinolone derivatives: Insomnia, |
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hallucinations, headaches, low seizure threshold, dizziness, somnolence, tinnitus. |
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Tetracyclines: Pseudotumor cerebri (children), abducens paralysis (adults) |
Glycosides |
Visual disturbances, somnolence, hallucinations, seizures, delirium |
Calcium antagonists |
Fatigue, insomnia, headaches, depression. Flunarizine/cinnarizine: Drug-induced |
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parkinsonism |
Coumarins |
Intracranial hemorrhage (2–12%/year) |
Radiotherapy5 |
Acute (!1 week): Headaches, nausea, somnolence, fever. Subacute: (2–16 weeks): |
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Somnolence, focal neurological deficits, leukoencephalopathy, brain stem syn- |
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drome (rare). Late ("4 months): radiation necrosis6, leukoencephalopathy, |
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dementia, secondary tumor |
Chemotherapy7 |
Acute: Insomnia, confusion, restlessness, stupor, generalized seizures, myoclonus. |
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Late: Apathy, dementia, insomnia, incontinence, gait impairment, ataxia |
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(Biller, 1998; Diener and Kastrup, 1998; Keime-Guibert et al., 1998)
1 Common adverse effects. 2 Inability to sit still with tormenting sensations in the legs that improve briefly when the patient moves about. 3 Characterized by confusion, fever, restlessness, myoclonus, diaphoresis, tremor, diarrhea, and ataxia; usually due to drug interactions, e. g., fluoxetine + sertraline, serotonin reuptake inhibitor + tryptophan, MAO inhibitors, carbamazepine, lithium, or clomipramine. 4 Acetylsalicylic acid (ASA). 5 Syndromes also occur in combination with chemotherapy. 6 One to two years after percutaneous radiotherapy, ca. 6 months after interstitial radiotherapy. Focal neurological deficits. 7 Methotrexate (high-dose i. v., intrathecal) cisplatin, vincristine, asparaginase, procarbazine, 5-fluorouracil, cytosine arabinoside, nitrosourea compounds (high-dose), ifosfamide, tamoxifen, etoposide (high-dose).
Appendix
389
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Appendix
390
Appendix
Table 53 Neuropathy syndromes (p. 316)
Syndrome |
Causes |
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Predominantly sym- |
Amyotrophic lateral sclerosis (ALS), multifocal motor neuropathy, Guillain–Barré |
metrical motor deficits |
syndrome, CIDP1, acute porphyria, hereditary sensorimotor neuropathy |
Predominantly asym- |
Neuronopathy: ALS, poliomyelitis, spinal muscular atrophy |
metrical or focal motor |
Radicular lesion: Root compression (herniated intervertebral disk, tumor), herpes |
deficits |
zoster, carcinomatous meningitis, diabetes mellitus |
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Plexus lesion: Neuralgic amyotrophy of shoulder, tumor infiltration, diabetes melli- |
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tus, tomaculous neuropathy, compression (positional) |
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Multiple mononeuropathy: Vasculitis, diabetes mellitus, multifocal motor neu- |
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ropathy, neuroborreliosis, sarcoidosis, HIV, tomaculous neuropathy, leprosy, neu- |
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rofibromatosis, cryoglobulinemia, HNPP (p. 332), neoplastic infiltration |
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Mononeuropathy: Compartment syndrome (median n., ulnar n.), compression |
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(anterior interosseous n., peroneal n.), lead poisoning, diabetes mellitus |
Predominantly auton- |
Diabetes mellitus, amyloidosis, Guillain–Barré syndrome, vincristine, porphyria, |
omic disturbances |
HIV, idiopathic pandysautonomia, botulism, paraneoplastic neuropathy |
Predominant pain |
Diabetes mellitus, vasculitis, Guillain–Barré syndrome, uremia, amyloidosis, ar- |
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senic, thallium, HIV, Fabry disease, cryptogenic neuropathy |
Predominantly sensory |
Diabetes mellitus, alcohol, ethambutol, vitamin B12 deficiency, folic acid defi- |
disturbances |
ciency, overdosage of vitamin B6, paraneoplastic, metronidazole, phenytoin, thali- |
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domide, leprosy, cytostatic agents (e. g., vincristine, vinblastine, vindesine, |
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cisplatin, paclitaxel), amyloidosis (dissociated sensory loss), hereditary sensory |
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neuropathy, monoclonal gammopathy, tabes dorsalis, Friedreich ataxia (p. 280) |
Ganglioneuropathy2 |
Paraneoplastic, Sjögren syndrome, cisplatin, vitamin B6 intoxication, HIV, id- |
(ataxia) |
iopathic sensory neuronopathy |
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(Barohn, 1998) |
1 Chronic inflammatory demyelinating polyneuropathy. 2 Asymmetrical proprioceptive loss without paralysis.
Table 54 Acquired and hereditary neuropathies (p. 316)
Cause |
Examples |
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Acquired
!Metabolic disorder ! Diabetes mellitus, uremia, hypothyroidism, acromegaly
!Dietary deficiency ! Vitamin deficiency (B1 = beriberi, B6, B12, E), malabsorption
!Immune-mediated ! Guillain–Barré syndrome, Fisher syndrome, chronic inflammatory demyelinat-
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ing polyneuropathy (CIDP), multifocal motor neuropathy, pandysautonomia, |
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|
|
neuralgic amyotrophy of shoulder1, vasculitis, connective tissue disease, |
|
|
|
plasmocytoma, benign monoclonal gammopathy, Churg–Strauss syndrome, |
|
|
|
cryoglobulinemia, rheumatoid arthritis |
! |
Infection |
! Herpes zoster, leprosy, Lyme disease, HIV, neurosyphilis, diphtheria, typhus, |
|
|
|
|
paratyphus |
! |
Drugs |
! Carbimazole, cisplatin, cytarabine, enalapril, ethambutol, etoposide, gentami- |
|
|
|
|
cin, gold, imipramine, indomethacin, INH, paclitaxel, phenytoin, procarbazine, |
|
|
|
suramine, thalidomide, vinca alkaloids, vitamin B6 |
! |
Toxins (environmen- |
! Alcohol, arsenic, benzene, lead, heroin, hexachlorophene, pentachlorophenol, |
|
|
tal, industrial)2, |
|
polychlorinated biphenyls, mercury, carbon tetrachloride, thallium, triarylo- |
|
drugs2 |
|
phosphate |
! |
Neoplasm |
! Paraneoplastic: Lung, stomach, or breast cancer; Hodgkin disease, leukemia. In- |
|
! |
|
! |
filtration: Hodgkin disease, leukemia, carcinomatous meningitis, polycythemia |
Mechanical |
Compression, trauma, distortion |
||
! |
Unknown |
! |
Critical illness polyneuropathy |
Hereditary |
See pp. 332 and 396 f |
||
(Barohn, 1998)
1 Causes not confirmed. 2 A large number of substances can lead to polyneuropathy (PNP). Only some of them are listed.
Rohkamm, Color Atlas of Neurology © 2004 Thieme
All rights reserved. Usage subject to terms and conditions of license.
Appendix
Table 55 Additional diagnostic studies for neuropathies (p. 316)
|
Method |
Information/Parameters |
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|
|
|
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|
Neurography |
Motor neuron lesion: Normal (consider conduction block) |
||
|
|
Ganglionopathy: MSAP1 normal to , SNAP2 normal to , (dermatomal) SEP3 |
||
|
|
Radiculopathy: H reflex: Lateral inequality/absence4; F waves: some prolonged; |
||
|
|
(dermatomal) SEP |
|
|
|
|
Axonal lesion: Motor NCV5: Normal; MSAP , SNAP |
(localize by inching); |
|
|
|
Demyelination: DML , NCV: or local conduction |
block |
|
|
|
6 |
|
|
|
|
MSAP /dispersed; F waves: Prolongation or absence; SNAP: normal/dispersed in |
||
|
|
motor neuropathy, in sensory neuropathy |
|
|
|
Needle electro- |
Motor neuron lesion: Fibrillation, positive waves, fasciculation. Amplitude, poly- |
||
|
myography |
phasia rate, MAP7 duration |
|
|
|
|
Ganglionopathy: MAP: Low-grade neurogenic changes may be observed |
||
|
|
Radiculopathy: Pathological spontaneous activity in paravertebral muscles/seg- |
||
|
|
ment-indicating muscles (p. 357); MAP8: neurogenic changes |
||
|
|
Axonal lesion: Pathological spontaneous activity (fibrillation, fasciculation); MAP: |
||
|
|
neurogenic changes |
|
|
|
|
Demyelination: Absence of pathological spontaneous activity, maximum innerva- |
||
|
|
tion with thinning pattern |
|
|
|
Laboratory tests9 |
Standard tests: Erythrocyte sedimentation rate, differential blood count, blood |
||
|
|
glucose (diurnal profile), C-reactive protein, calcium, sodium potassium, alkaline |
||
|
|
phosphatase, SGOT10, SGPT11, CK12, γ-GT13, electrophoresis, rheumatoid factors, |
||
|
|
vitamin B12/ folic acid, Borrelia/HIV antibodies, basal TSH14, triglycerides, |
||
|
|
cholesterol, urine status, blood culture |
|
|
|
|
Special tests: CSF, homocysteine, hemoglobin A1C, syphilis serology, parathyroid |
||
|
|
hormone, antinuclear antibodies (e. g., Sm, RNP, Ro SS-A, La SS-B, Scl-70, Jo-1, |
||
|
|
Pm-Scl), antineuronal antibodies (ANNA-1, anti-Hu), myelin-associated glyco- |
||
|
|
protein (MAG), ganglioside antibodies (GM1, GD1a, GD1b, GQ1b), heavy metals |
||
|
|
(blood, urine), porphyrins, cryoglobulins, serum phytanic acid, very long chain |
||
|
|
fatty acids (VLCFA, C24–26), molecular genetic testing |
|
|
|
Sural nerve biopsy15 |
Vasculitis, amyloid neuropathy, neuropathy with sarcoidosis, leprosy, chronic neu- |
||
|
|
ropathy (HMSN III/metachromatic leukodystrophy, with or without other forms of |
||
|
|
hereditary neuropathy p. 332; chronic inflammatory neuropathy, polyglucosan |
||
|
|
body neuropathy), tumor (neurofibroma/schwannoma, neoplastic infiltration; par- |
||
|
|
aneoplastic neuropathy), neuropathy with monoclonal gammopathy, if applicable |
||
|
Diagnostic imaging |
Guided by clinical findings (spinal, radicular, plexus, distal peripheral lesions?), |
||
|
|
plain radiographs, ultrasound, CT, MRI, myelography, skeletal scintigraphy and/or |
||
|
|
angiography |
|
|
= elevated, prolonged; |
= diminished, absent |
|
|
|
1 Summated muscle action potential (evoked). 2 Amplitude of sensory nerve action potential. 3 Somatosensory evoked action potential amplitude reduced or absent. 4 For technical reasons, can only be determined for S1.
5 Nerve conduction velocity. 6 Distal motor latency. 7 Muscle action potential. 8 After 2 weeks, at earliest. 9 Partial list. 10 Serum glutamic–oxaloacetic transaminase. 11 Serum glutamate–pyruvate transaminase. 12 Creatine kinase. 13 γ-Glutamyl transpepsidase. 14 Thyrotropin. 15 Muscle biopsy may also be helpful in some cases.
Appendix
391
Rohkamm, Color Atlas of Neurology © 2004 Thieme
All rights reserved. Usage subject to terms and conditions of license.
Appendix
392
Appendix
Table 56 Causes of radicular syndromes (p. 318)
Cause |
Comments |
|
|
Degenerative changes
!Intervertebral disk hernia- ! Symptoms usually resolve with conservative treatment1 tion
!Spondylosis deformans ! Torus-, buckleor spur-shaped spondylophytes form due to degenerative
|
|
changes in the intervertebral disks |
! |
Spinal canal stenosis |
! See p. 284 |
! |
Spondylolisthesis |
! Slippage of a vertebra with respect to the next lower vertebra because |
|
|
of bilateral spondylolysis2 |
Trauma |
See p. 272 |
|
Neoplasm |
Primary spinal tumor (p. 284), metastatic tumor/neoplastic meningeosis |
|
|
|
(p. 262) |
Infection/inflammation |
See p. 222ff. herpes zoster, borreliosis, epidural abscess, spondylitis, sar- |
|
|
|
coidosis, arachnopathy |
Vascular |
See p. 282 |
|
Metabolic |
Diabetes mellitus (p. 324; Table 59, p. 395) |
|
Inflammatory rheumatic |
Ankylosing spondylitis, rheumatoid arthritis |
|
Malformation |
See p. 288 ff |
|
Iatrogenic |
Injection, lumbar puncture, surgery |
|
Radiotherapy |
Radiation-induced amyotrophy (cauda equina)3 |
|
Pseudoradicular syndrome4, |
! Arm: Carpal tunnel syndrome, stiff shoulder, humeroscapular periar- |
|
nonradicular pain |
thropathy, syringomyelia |
|
! Leg: Facet syndrome, sacroiliac joint syndrome, coccygodynia, coxarthrosis, heterotopic ossification
! General: Polyradiculitis, connective tissue diseases, rheumatoid diseases, malformations, myopathy/muscle trauma, strain, arthropathy, endometriosis, osteomyelitis, osteoporosis, arterial dissection, prostatitis, cystitis, Paget disease, somatization disorder
(Mumenthaler et al., 1998)
1 Absolute indications for surgery: Massive lumbar disk herniation with sphincter dysfunction, cervical disk herniation with spinal cord compression, severe weakness. Relative indications: Persistent radicular pain, frequent recurrence of radicular symptoms. 2 Defect in the pars interarticularis of the vertebral arch. 3 Development of following manifestations months to years after radiotherapy (para-aortic irradiation): malignant testicular tumor, lymphoma; progressive flaccid paraparesis without major sensory loss. 4 To be considered in the differential diagnosis.
Rohkamm, Color Atlas of Neurology © 2004 Thieme
All rights reserved. Usage subject to terms and conditions of license.