Non-Alzheimer's and Atypical Dementia E-Book

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Sprache: Englisch

Seitenzahl: 843

Veröffentlichungsjahr: 2016

Beschreibung

Dementia is the most common type of neurodegenerative disorder. Non-Alzheimer's and Atypical Dementia concentrates on each form of dementia individually, considering symptoms, diagnosis and treatment

• Focuses on non-Atypical Dementia 
• Multidisciplinary approach to diagnosis and management
• Allows development of management and care plan strategies
• Practical approach including case studies
• Written by a world-renowned editorial team

Leseprobe

Table of Contents

Cover

Title Page

Notes on contributors

CHAPTER 1: Introduction

Multidisciplinary evaluation of the atypical dementia patient

Atypical Alzheimer’s disease

Vascular cognitive impairment: Diagnosis and treatment

Frontotemporal dementia

Lewy body dementias

Corticobasal degeneration and progressive supranuclear palsy

Repeat expansion diseases and dementia

Prion disorders

Autoimmune dementias

Toxic and metabolic dementias

Leukoencephalopathies/leukodystrophies

Infectious causes of dementia

Rheumatologic and other autoimmune dementias

Comprehensive management of the patient with an atypical dementia

CHAPTER 2: The multidisciplinary evaluation of the atypical dementia patient

Introduction

History

Neurological examination

Neuropsychological testing

Laboratory testing

Putting it all together: Multidisciplinary assessment/review

References

CHAPTER 3: Atypical Alzheimer’s disease

Introduction

Epidemiology

Diagnosis

Neuropathology

Genetics

Structural and functional neuroimaging: MRI, FDG-PET, and SPECT

CSF/amyloid imaging

Treatment

Conclusion

Acknowledgments

References

CHAPTER 4: Vascular cognitive impairment

Background

Case presentations

Clinical subtypes of VCI

Evaluation and diagnosis

Treatment

Summary

References

CHAPTER 5: Frontotemporal dementia

Introduction and definition of terms

Epidemiology

Core FTD clinical syndromes

Other clinical syndromes associated with FTD

Neuropathology

Clinicopathological correlation

Genetics

Treatment

Conclusions

References

CHAPTER 6: Lewy body dementias (DLB/PDD)

Introduction

Nosology

DLB/PDD clinical features

Differential diagnosis

Neuroimaging findings

Laboratory findings

Pathophysiology and pathology

Treatment and management

Summary

References

CHAPTER 7: Corticobasal degeneration and progressive supranuclear palsy

History and nomenclature

Epidemiology

Clinical features of corticobasal syndrome

Correlation between CBS and CBD

Clinical features of progressive supranuclear palsy syndrome

Correlation between PSP syndrome and PSP

Diagnostic studies

Pathology and pathophysiology

Treatments

Summary

References

CHAPTER 8: Repeat expansion diseases and dementia

Introduction

HD

SCA17

Fragile X-associated tremor/ataxia syndrome (FXTAS)

Summary

References

CHAPTER 9: Prion diseases and rapidly progressive dementias

Rapidly progressive dementias

Prion diseases

Treatment and management

Prion decontamination and preventive measures

Differential diagnosis of rapidly progressive dementias

Summary

Acronyms

Acknowledgments

References

CHAPTER 10: Autoimmune dementias

Introduction

Nomenclature

Epidemiology

Clinical features

Treatment

Prognosis

Conclusion

References

CHAPTER 11: Toxic and metabolic dementias

Introduction

Toxic dementias

Metabolic dementias

Summary

References

CHAPTER 12: Leukoencephalopathies/leukodystrophies

Introduction

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia: Hereditary diffuse leukoencephalopathy with spheroids and pigmentary orthochromatic leukodystrophy

Adult-onset autosomal dominant leukodystrophy with autonomic dysfunction (

LMNB1

mutation)

Adult polyglucosan body disease

Lysosomal storage disorders

Summary

References

CHAPTER 13: Infectious causes of dementia

Introduction

Viruses

Bacteria

Fungi

Parasites

Summary

References

CHAPTER 14: Rheumatologic and other autoimmune dementias

Introduction

The diagnostic conundrum of rheumatologic disease in the neurologic setting

SLE

Antiphospholipid syndrome and Sneddon’s syndrome

Sjögren’s syndrome

Vasculitides

Systemic sclerosis (scleroderma)

Sarcoidosis

Celiac disease

Summary and conclusions

References

CHAPTER 15: Comprehensive management of the patient with an atypical dementia

After the diagnosis

Evaluating functional abilities

Vulnerability and safety

Occupational issues for the patient and caregiver

Managing motor symptoms

Dysphagia

Managing behavioral symptoms

Care across the disease trajectory

End-of-life care

Autopsy decisions

Key considerations for the clinical management of atypical dementias

Caregiver health and coping

Summary

15.A.1 Example of business-style card

15.A.2 Resources for patients and families (some organizations are based in the United States, although most countries have equivalent organizations)

References

Index

End User License Agreement

List of Tables

Chapter 03

Table 3.1 1984 NINCDS-ADRDA criteria for probable Alzheimer’s disease [15].

Table 3.2 Various criteria for AD incorporating clinical presentation and biomarkers.

Table 3.3 Criteria for primary progressive aphasia.

Table 3.4 Criteria for logopenic variant PPA.

Table 3.5 Two proposed criteria for PCA.

Chapter 04

Table 4.1 Terms and abbreviations.

Table 4.2 The pathogenic spectrum of vascular cognitive impairment: RF→ CVD → VBI →VCI.

Table 4.3 Clinical Criteria for vascular dementia (VaD).

Table 4.4 Neurobehavioral approach to diagnosis of VCI, AD, or mixed VCI/AD.

Table 4.5 Primary and secondary prevention: clinical trials that include a cognition outcome measure.

Chapter 05

Table 5.1 1998 Neary consensus criteria for behavioral variant frontotemporal dementia.

Table 5.2 Proposed international consensus criteria for bvFTD.

Table 5.3 1998 Neary consensus criteria for semantic variant.

Table 5.4 Diagnostic criteria for semantic variant PPA.

Table 5.5 1998 Neary consensus criteria for progressive nonfluent aphasia.

Table 5.6 Diagnostic criteria for nonfluent PPA.

Chapter 06

Table 6.1 Comparison of DLB and PDD.

Table 6.2 Bedside tests to evaluate cognitive features in PDD/DLB.

Table 6.3 Differential diagnosis of DLB/PDD.

Chapter 07

Table 7.1 NINDS–SPSP clinical criteria for PSP (1996).

Chapter 09

Table 9.1 Diagnostic criteria for sporadic Jakob–Creutzfeldt disease.

Table 9.2 UCSF 2011 MRI criteria for sCJD.

Table 9.3 Diagnostic criteria for vCJD.

Table 9.4 Mnemonic acronym for RPD differential.

Table 9.5 Clinical diagnosis algorithm.

Chapter 10

Table 10.1 Key points for autoimmune dementias.

Table 10.2 Potentially reversible causes of cognitive impairment.

Table 10.3 Antibodies with specificity for neural antigens, accompanying cognitive and other neurological disorders, and oncological accompaniments.

Table 10.4 Some commonly used therapies for autoimmune dementias.

Chapter 11

Table 11.1 Toxic causes of dementia (differential diagnosis).

Table 11.2 Clinical signs and symptoms associated with CO poisoning and correlated COHb levels.

Table 11.3 Major dementia issues in alcoholics (sometimes nutritional).

Table 11.4 Metabolic causes of dementia (differential diagnosis).

Table 11.5 Central nervous system (CNS) effects after ethanol ingestion as a function of blood-alcohol content (BAC).

Table 11.6 Medical management strategies for ethanol intoxication and withdrawal.

Chapter 12

Table 12.1 Selected leukodystrophies.

Table 12.2 Clinical signs of a leukodystrophy.

Table 12.3 Key features of leukodystrophies and leukoencephalopathies discussed in this chapter.

Chapter 13

Table 13.1 Memorial Sloan Kettering scale for AIDS Dementia Complex (ADC).

Table 13.2 Diagnostic guidelines for CNS Whipple’s disease.

Table 13.3 Neuropsychological impairment in cerebral infections.

Chapter 14

Table 14.1 Rheumatologic and related dementias and neurological characteristics.

Table 14.2 Neuropsychiatric manifestations of SLE described by the American College of Rheumatology.

Table 14.3 American College of Rheumatology-recommended neuropsychological assessment battery for use in systemic lupus erythematosus.

Chapter 15

Table 15.1 Safety concerns.

Table 15.2 Assessment of caregiver health and coping.

Table 15.3 Resources for caregivers.

List of Illustrations

Chapter 03

Figure 3.1 Coronal T1-weighted MRI of case 1 showing bilateral hippocampal and less severe frontal and temporal cortical atrophy.

Figure 3.2

(a)

Axial T1-weighted MRI of case 2 showing left parietal atrophy.

(b)

Coronal T1-weighted MRI showing normal hippocampal size.

(c)

PIB-PET showing cortical PIB binding (yellow to red indicate increasing spectrum of PIB binding). Orientation of MRIs is radiologic (left is right). Orientation of PET scan is neurological (right is right).

Figure 3.3

(a)

Benson Figure (top) with patient copy (bottom).

(b)

Axial T1-weighted MRI showing occipital atrophy.

(c)

Sagittal T1-weighted MRI showing parietal and occipital atrophy. Orientation is radiologic.

Figure 3.4

(a)

Sagittal T1-weighted MRI showing parietal and occipital atrophy.

(b)

FDG-PET showing hypometabolism in frontal and parietal cortices.

(c)

PIB-PET showing diffuse cortical binding. Orientation is neurological.

Figure 3.5

(a)

Coronal T1-weighted MRI showing bilateral (right > left) temporal and right parietal (not shown) atrophy; orientation is radiological.

(b)

His pathology showed AD characteristic amyloid-beta-positive plaques (brown) in the middle frontal gyrus (4G8 (anti-amyloid-beta) immunohistochemistry; 100x).

(c)

AD characteristic tau-positive inclusions in the hippocampus. Neurofibrillary tangles (arrows), neuritic plaques (arrow heads), and neuropil threads (brown background) are present (CP13 (anti-phosphorylated tau) immunohistochemistry; 40x). DG, dentate gyrus.

Chapter 04

Figure 4.1 Case 1: axial MRI (T1, proton density (PD), and T2 weighted) shows cystic infarcts in the right anterior thalamus and left genu internal capsule. SBI are seen in the right putamen, left posterior limb of the internal capsule (top row), and left frontal white matter (bottom row). Periventricular white matter changes are rated grade 1 on CHS scale.

Figure 4.2 Case 1: coronal T1-weighted MRI shows moderate 2+ hippocampal atrophy on Scheltens’ rating scale [110, 111] and moderate generalized cerebral atrophy.

Figure 4.3 Case 2: axial MRI (T1, PD, and T2 weighted) shows T2-weighted hyperintensities in bilateral periventricular white matter, SBI in the right anterior limb of the internal capsule, and prominent perivascular spaces plus encephalomalacia in the right putamen. White matter hyperintensities are rated 6–7 on the CHS scale.

Figure 4.4 Case 2: coronal T1-weighted MRI shows 3+ atrophy by Scheltens’ scale [110] of the hippocampus and severe cerebral atrophy.

Figure 4.5 Case 3: MRI (T1, PD, and T2 weighted) shows severe confluent deep white matter changes (grade 7–8), SBI in the right lateral thalamus, and mineralization of the globus pallidi.

Figure 4.6 Case 3: coronal T1-weighted MRI shows 3+ atrophy by Scheltens’ scale [110] of both hippocampi and moderate cerebral atrophy.

Figure 4.7 Incidence of poststroke dementia at different time intervals after stroke onset in hospital-based studies (gray) and community-based studies (black). When the reference appears several times, data provided correspond to different assessments at different time intervals in the same cohort of patients.

Figure 4.8 Prefrontal-subcortical circuits important for executive function.

Figure 4.9 Both SBI and perivascular spaces (PVS) are bright on T2-weighted sequences. On proton density or fluid-attenuated inversion recovery (FLAIR) sequences, however, SBI are hyperintense (bright), whereas perivascular spaces (PVS) are isointense, compared to cerebrospinal fluid (CSF). On T1-weighted sequences, both SBI and PVS are hypointense or dark.

Figure 4.10 Meta-analysis of double-blind placebo-controlled trials of cholinesterase inhibitors and memantine for vascular dementia. Cognitive outcomes on the ADAS-Cog subscale (change from baseline) in vascular dementia patients in cholinesterase inhibitors and memantine trials by drug and dose (last observation carried forward sample); WMD, weighted mean difference.

Chapter 05

Figure 5.1 MRI axial (

a

), coronal (

b

), and sagittal (

c

) T1 showing bifrontal atrophy associated with behavioral variant FTD (bvFTD), greater on the right than left.

Figure 5.2 Axial (

a

) and coronal (

b

) T1 MRI showing left anterior temporal lobe atrophy associated with semantic variant PPA (svPPA).

Figure 5.3 Coronal (

a

) and axial (

b

) T1 MRI showing left perisylvian atrophy associated with nfv-PPA.

Figure 5.4 Pick bodies in the left midinsula in a 74-year-old woman with nonfluent variant PPA due to Pick’s disease. Immunohistochemistry for 3-repeat tau, hematoxylin counterstain.

Figure 5.5 Clinical and pathologic correlates between FTD-spectrum syndromes and FTLD pathologies. PSP = progressive supranuclear palsy; CBS = corticobasal syndrome; bvFTD = behavioral variant frontotemporal dementia; PPA = primary progressive aphasia; svPPA = semantic variant primary progressive aphasia; nfv-PPA = nonfluent variant primary progressive aphasia; lvPPA = logopenic variant primary progressive aphasia; FTD-MND = frontotemporal dementia with motor neuron disease; FTLD-tau = frontotemporal lobar degeneration with tau pathology; FTLD-TDP = FTLD with TAR DNA-binding protein 43 (TDP-43) pathology; FTLD-FUS = FTLD with fused in sarcoma (FUS) pathology; AD = Alzheimer’s disease.

Chapter 06

Figure 6.1 Patients were asked to copy the image exactly. Image

(a)

is the original figure that patients were asked to copy. Image

(b)

is the reproduced image from an 80-year-old patient with Alzheimer’s disease. Note that the patient struggles with reproducing one of the pentagons but the spatial aspects of the figure are easily identifiable. Image

(c)

is from a cognitively intact 82-year-old elderly individual. Image

(d)

shows an attempt by an 80-year-old patient with probable DLB; although cognitive symptoms were mild, he is unable to reproduce the spatial aspects of the figures.

Figure 6.2 High magnification neuropathology from a DLB case. Cortical

(a)

and nigral

(c)

LBs after staining with hematoxylin and eosin. Note that cortical LBs are smaller and lack the halo that typifies LBs within the substantia nigra. Images

(b)

and

(d)

are taken after immunostaining DLB tissue with antibodies against alpha-synuclein. Cortical

(b)

and nigral LBs

(d)

both stand out. The entire inclusion stains in the cortex, but only the halo in the substantia nigra contains alpha-synuclein. Arrows point to the LBs in all images. All images at 60× magnification. Scale bar is 30 µm.

Chapter 07

Figure 7.1 Brain MRI in executive–motor CBD. T1-weighted brain MRI of a 68-year-old right-handed woman with pathological-proven CBD with an executive–motor syndrome. Sagittal MRI (left) shows dorsolateral frontal atrophy, and axial MRI (right) shows bilateral parietal atrophy, particularly in the primary motor cortex. Orientation is radiological (left is right).

Figure 7.2 Brain MRI in nonfluent variant primary progressive aphasia secondary to CBD. Coronal T1-weighted brain MRI of a 54-year-old right-handed woman showing severe focal left insular and left inferior frontal gyrus atrophy with mild atrophy in bilateral orbitofrontal cortex and medial frontal regions. Orientation is radiological.

Figure 7.3 Brain MRI in behavioral variant frontotemporal dementia secondary to CBD. Coronal T1-weighted brain MRI in a 64-year-old gentleman showing bilateral frontoinsular, dorsomedial frontal, and dorsolateral prefrontal atrophy. Orientation is radiological.

Figure 7.4 Brain MRI in progressive supranuclear palsy syndrome secondary to CBD. T1-weighted brain MRI in a 63-year-old gentleman showing mild midbrain atrophy in sagittal (left) and axial (right) planes.

Figure 7.5 Statistical parametric mapping version 5 (SPM5) voxel-based morphometry (VBM) MRI analysis contrasting gray and white matter volume in

(a)

a cohort of patients with corticobasal degeneration (CBD) (

N

 = 13) with healthy older controls (NC,

N

 = 44) who had VBM-compatible 1.5T structural T1 scans and

(b)

the three main clinical syndromes seen in CBD compared to NC viewed on a DARTEL-derived template based on 48 healthy controls (voxel resolution: 1 mm). Patients with VBM-compatible scans in the three CBD clinical syndromes included nfvPPA (

N

 = 4), EM-CBD (

N

 = 5), and bvFTD-CBD (

N

 = 3).

Figure 7.6 SPM5 VBM analysis showing the patterns of gray and white matter volume loss in

(a)

left panel: each CBS subgroup, all with autopsy studies (CBS-AD (

N

 = 7), CBS-CBD (

N

 = 11), CBS-PSP (

N

 = 4), and CBS-TDP (

N

 = 3)) relative to healthy controls (NC,

N

 = 44) and

(b)

right panel: all three CBS subgroups combined relative to NC viewed on a DARTEL-derived template based on 48 healthy controls (voxel resolution: 1 mm).

Figure 7.7 Regions of brain atrophy in patients with corticobasal syndrome (CBS) and progressive supranuclear palsy syndrome (PSP-S) relative to controls. VBM-identified regions of decreased gray and white matter volume in 14 CBS and 15 PSP-S patients relative to 80 age-matched control subjects are displayed on a normal adult brain template (

P

 < 0.05, corrected).

(a)

CBS patients versus controls.

(b)

PSP patients versus controls. Row 1 shows the regions of significant gray matter loss rendered on a healthy subject’s brain. Row 2 shows regions of significant gray (displayed in red) and white (displayed in yellow) matter loss relative to controls at the following Montreal Neurological Institute (MNI) coordinates:

x

 = −33,

y

 = −4, and

z

 = 49. Row 3 shows regions of significant gray (displayed in red) and white (displayed in yellow) matter loss relative to controls at the following MNI coordinates:

x

 = 5,

y

 = −15, and

z

 = −8. A indicates anterior; P, posterior.

Chapter 08

Figure 8.1 Coronal T1-weighted brain MRI in Huntington’s disease.

(a)

Healthy control.

(b)

HD carrier with early-stage (stage 2) disease. MRI shows caudate (white arrow) and putaminal (black arrow) atrophy showing that there is enlargement of the lateral ventricles due to partial degeneration of the caudate nucleus.

Figure 8.2 T1-weighted brain MRI images from SCA17 patients showing cerebellar atrophy of the vermis and cerebellar hemispheres.

Figure 8.3 FLAIR MRI brain images of a 59-year-old asymptomatic male with the FXTAS premutation. Consecutive images of the posterior fossa highlight the characteristic bilateral signal abnormality of the middle cerebellar peduncles extending into cerebellar white matter. In addition, high FLAIR signal abnormalities are observed in the cerebri and in the splenium of the corpus callosum.

Chapter 09

Figure 9.1 Axial brain MRI of case 1.

(a)

DWI and

(b)

ADC MRI scans showing striatal (solid arrows) and medial-dorsal thalamic (dotted arrows) hyperintensities on DWI imaging, with corresponding ADC hypointensities (arrows) in a patient with probable sCJD.

Figure 9.2 Axial brain MRI in sporadic CJD and variant CJD. (

a-c

) Each show a FLAIR, DWI and ADC sequences, whereas d shows only a FLAIR and DWI sequence. (

a

) Neocortical (solid arrow), limbic (dashed arrow) and subcortical (dotted arrow) DWI and FLAIR hyperintensities with corresponding ADC hypointensities in sporadic CJD. (

b

) Neocortical (solid arrow) and limbic (dashed arrow) DWI hyperintensities with corresponding ADC hypointensities in sporadic CJD. (

c

) Subcortical (dotted arrows) DWI and FLAIR hyperintensities, with corresponding ADC hypointensities in sporadic CJD. (

d

) Pulvinar sign (arrow) in variant CJD. ADC, apparent diffusion coefficient; CJD, Creutzfeldt–Jakob disease; DWI, diffusion-weighted imaging; FLAIR, fluid-attenuated inversion recovery; MRI, magnetic resonance imaging.

Figure 9.3 Neuropathological findings in prion diseases.

(a)

In sporadic CJD, some brain areas may have no (hippocampal end plate, left), mild (subiculum, middle), or severe (temporal cortex, right) spongiform change. Hematoxylin and eosin (H&E) stain.

(b)

Cortical sections immunostained for PrP

Sc

in sporadic CJD: synaptic (left), patchy/perivacuolar (middle), or plaque type (right) patterns of PrP

Sc

deposition.

(c)

Large kuru-type plaque, H&E stain.

(d)

Typical “florid” plaques in vCJD, H&E stain.

Figure 9.4 Axial brain MRI of case 2 with GSS due to a P102L mutation in PRNP. (

a

) FLAIR and (

b

) DWI MRI scans show no clear abnormality. There is a suspicion of DWI hyperintensity in the bilateral posterior insula and posterior limb of bilateral hippocampi, which were not, however, hypointense on the ADC map (not shown). Cerebellum was also normal (not shown).

Figure 9.5 (Case 3)—Bilateral basal ganglia hyperintensities in axial FLAIR MRI scan with edema tracking along the limbs of the internal capsule. The lesions were gadolinium enhancing.

Chapter 10

Figure 10.1 Case 1: FLAIR axial MRIs (radiological orientation).

(a)

At initial symptom onset, T2 signal abnormality is seen in the left hippocampus.

(b)

After relapse, T2 signal is less prominent in the left hippocampus. New, subtle T2 signal abnormality is now seen in the right hippocampus (arrow). Bilateral temporal lobe atrophy is seen. See text.

Figure 10.2 NMDAR antibody (relevant to Case 2). Indirect immunofluorescence staining pattern of patient’s serum on a composite of mouse neural tissue, provided courtesy of Dr. Vanda A. Lennon, Neuroimmunology Laboratory, Mayo Clinic, Rochester, MN. Hippocampus (Hi) stains brighter than cerebral cortex (Co), basal ganglia (BG), and thalamus (Th). Granular layer (GL) of cerebellum also stains brightly, typical for NMDAR antibody; molecular layer (ML) is negative.

Figure 10.3 FLAIR axial MRIs in a patient with LGI1 (“VGKC”) antibody and limbic encephalitis with left greater than right medial temporal lobe hyperintensities

(a)

that had radiologic and clinical improvements

(b)

after corticosteroid therapy.

Figure 10.4 A nonevidence-based algorithm for the treatment of patients with suspected autoimmune dementias.

Chapter 12

Figure 12.1 Flow chart listing

s

elected leukodystrophies, their characteristic features, and mode of inheritance.

Figure 12.2 FLAIR brain MRI in a 50-year-old with CADASIL. Some classic MRI findings of CADASIL including T2-weighted medial temporal lobe hyperintensities (solid arrow), cavitations in the white matter (dashed arrows), and confluent white matter disease are shown.

Figure 12.3 Brain MRI in ALSP/POLD. Axial

(a)

, coronal

(b)

, and sagittal

(c)

T2-weighted images of a case are presented. There is significant atrophy of the bilateral frontal lobes. There are areas of diffuse hyperintensity in the white matter of the bilateral frontal lobes and the corpus callosum. Note that there is substantial callosal atrophy, which is especially severe at the genu and body

(c)

. A small focus of signal abnormality is identified within the splenium of the corpus callosum. Other parts of the brain appear to be relatively well preserved.

Figure 12.4 Magnetic resonance images (axial sections, T2-weighted) from four HDLS patients:

(a)

Patient 1 (MRI performed 1.2 years after start of symptoms); localized white matter lesions (arrow) in both frontal and parietal hemispheres involving the corpus callosum (arrow dashed).

(b)

Patient 2 (MRI performed 1.9 years after start of symptoms); confluent white matter lesions in both frontal and parietal hemispheres with cortical atrophy in the affected areas.

(c)

Patient 3 (MRI performed 3.5 years after start of symptoms); localized periventricular lesions (arrow) with corresponding frontoparietal atrophy and involvement of the corpus callosum (arrow dashed).

(d)

Patient 4 (MRI performed 2.5 years after start of symptoms); bilateral frontoparietal white matter changes (arrow) extending into the corpus callosum (arrow dashed).

Figure 12.5 White matter lesions of the motor cortex from an ALSP/HDLS case.

(a)

Marked myelin loss of the subcortical white matter with spared U-fiber, KB staining.

(b–d)

Numerous axonal spheroids in the white matter lesion:

(b)

KB staining,

(c)

Bodian staining, and

(d)

immunohistochemistry for ubiquitin.

(e)

Abundant sudanophilic macrophages (

arrowhead

) in the white matter, Sudan III (

KB

Klüver–Barrera).

Bar

(a)

400 µm,

(b–e)

90 µm.

Figure 12.6 FLAIR Brain MRI of a 59-year-old man with APBD, showing periventricular, subcortical, and deep white matter signal abnormalities. Note the typical atrophic cervical spinal cord, typical of APBD.

Figure 12.7 Sural nerve biopsy in APBD showing intra-axonal basophilic inclusions (polyglucosan bodies) in several nerve fascicles (light microscopy, H&E stain). Further investigations showed that the storage material is not membrane bound, is diastase resistant, and is PAS positive (not shown).

Figure 12.8 Brain MRI in MLD. Axial T2

(a)

and FLAIR

(b)

images show diffuse hyperintensity in the cerebral white matter with sparing of U-fibers.

Figure 12.9 T2-weighted magnetic resonance images in a patient with Fabry disease, showing multifocal white matter hyperintensities approaching confluency.

Figure 12.10 Fabry disease. Axial CT

(a)

scan at the level of deep gray matter nuclei shows calcification in the pulvinar nuclei of thalamus (arrowheads). Corresponding areas show hyperintensity on T1-weighted image

(b)

.

Chapter 13

Figure 13.1 Neuroimaging in HIV-associated dementia studies obtained from a patient with untreated HIV infection, cognitive impairment, and gait disorder (see text). Noncontrast head CT

(a)

and T1-weighted brain MRI after gadolinium administration

(b)

are normal. FLAIR

(c)

and T2-weighted

(d)

images demonstrate ill-defined, symmetric white matter abnormalities, consistent with HIV-associated dementia.

Figure 13.2 Neuroimaging in general paresis noncontrast head CT

(a)

, postgadolinium T1-weighted

(b)

, and proton density

(c)

MRI images obtained from a middle-aged patient with general paresis (see text) show severe cerebral atrophy for age.

Figure 13.3 Neuroimaging in neurocysticercosis noncontrast head CT

(a)

demonstrates numerous, punctate calcified lesions consistent with nonviable cysts and a small focus of low attenuation in the right occipital lobe, consistent with edema. Associated enhancement in these and other areas on the postcontrast study

(b)

is compatible with degenerating cysts. T2-weighted MRI studies

(c

and

d)

from the same patient more clearly demonstrate the extent of edema surrounding degenerating cysts.

Chapter 14

Figure 14.1 MRI

(a)

T1-weighted image showing ventricular enlargement.

(b)

FLAIR image showing meningeal hyperintensity consistent with inflammation.

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Non-Alzheimer’s and Atypical Dementia

Edited by

Michael D. Geschwind, MD PHD

Memory and Aging CenterDepartment of NeurologyUniversity of California, San FranciscoSan Francisco, CA, USA

Caroline Racine Belkoura, PHD

Department of Neurological SurgeryUniversity of California, San FranciscoSan Francisco, CA, USA