Atlas of Neuropathology [Reprint 2020 ed.] 9780520323261

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Atlas of Neuropathology

Nathan Malamud, M.D. ASSOCIATE CLINICAL PROFESSOR OF PSYCHIATRY AND NEUROPATHOLOGY, UNIVERSITY OF CALIFORNIA NEUROPATHOLOGIST, THE LANGLEY PORTER CLINIC, SAN FRANCISCO

Atlas of Neuropathology

University

of California

Press

Berkeley and Los Angeles • IÇS7

U N I V E R S I T Y OF CALIFORNIA PRESS • B E R K E L E Y AND LOS ANGELES, CALIFORNIA CAMBRIDGE U N I V E R S I T Y PRESS • LONDON, ENGLAND ©

1 9 5 7 B Y T H E R E G E N T S OF T H E U N I V E R S I T Y OF CALIFORNIA L I B R A R Y OF CONGRESS CATALOG CARD N U M B E R :

56-I3I33

P R I N T E D IN T H E U N I T E D STATES OF AMERICA B Y T H E U N I V E R S I T Y OF CALIFORNIA P R I N T I N G D E P A R T M E N T DESIGNED B Y J O H N B . GOETZ

To R.K. M.

Preface The need for an atlas of neuropathology has long been apparent to workers in the fields of neurology, psychiatry, neurosurgery, and pathology. Precise and detailed knowledge of the gross and microscopic changes in the nervous system is a necessary prerequisite to the understanding of nervous and mental diseases. The purpose of this book is to provide a visual approach that is not usually provided by textbooks of neuropathology. I have attempted to illustrate the various disorders of the nervous system as comprehensively as possible. Equal space has been given to all categories, but special attention has been paid to such neglected conditions as the heredodegenerative disorders and those pertaining to geriatrics, cerebral palsy, and mental deficiency. The case presentation method was used wherever possible, to afford a correlation between lesions and symptoms and to crystallize the clinical syndromes. Owing to lack of space, theoretical discussions and references to the literature were reduced to a minimum. The material in the atlas represents approximately five thousand specimens collected from many sources, including general and mental hospitals, and personally examined by me in the laboratory of neuropathology at the Langley Porter Clinic. Although it is not possible to list all sources, particular acknowledgment for the use of material is made to the following: the departments of Neurosurgery, Neurology, Psychiatry, and Pathology of the University of California Medical Center, San Francisco; Letterman Army Hospital, San Francisco; United States Naval Hospital, Oakland; and Veterans Administration Hospital, Palo Alto; the state hospitals of the Department of Mental Hygiene in California; and the Armed Forces Institute of Pathology, Washington, D. C. In reproducing illustrations and certain data, I have indicated sources in suitable places throughout the book. I want to express gratitude to the following journals for granting permission to reproduce such material: Archives of Neurology and Psychiatry, Archives of Internal Medicine, American Journal of Diseases of Children, American Journal of Pathology, American Journal of Mental Deficiency, American Journal of Psychiatry, Archiv fur Psychiatrie und Nervenkrankheiten, Journal of Nervous and Mental Diseases, Journal of Neurosurgery, Journal of Neuropathology and Experimental Neurology, Journal of Pediatrics, Neurology, Military Medicine, Transactions of the American Neurological Association, and to Charles C. Thomas, publisher. M y special thanks are extended to: Mrs. Irene Robley, for her untiring effort and excellent technique in preparing the microscopic slides; Mrs. Marie-Jeanne Angenent, for typing the manuscript; Dr. Herbert Herzon, for reading the text. N. M.

vii

Contents I

CYTOLOGY AND CELLULAR PATHOLOGY

2

Staining M e t h o d s , 2 — Pathologic Changes in N e r v e Cells, 6 — Pathologic C h a n g e s in N e r v e Fibers, 8 — Pathologic Changes in Glia, 10 — Microglia, 10 — A s t r o c y t e s , 14 — Oligodendroglia, 16 — Pathologic C h a n g e s in C o n n e c t i v e Tissue, 16

II

INFLAMMATORY DISORDERS

18

Purulent Infections, 18 — Purulent Meningitis, 18 — Purulent E p e n d y m i t i s , 22 — Complications of Leptomeningitis, 24 — Abscess, 26 — M e t a s t a t i c Encephalitis, 30 — Granulomatous Infections, 32 — Tuberculosis, 32 — Tuberculous Meningitis, 32 — T u berculoma, 38 — Boeck's Sarcoid, 40 — Syphilis, 40 — G u m m a , 42 — M y c o t i c and Parasitic Infections, 44 — Torulosis, 44 — Coccidioidomycosis, 46 — Actinomycosis, 48 — Histoplasmosis, 50 — Toxoplasmosis, 50 — Cysticercosis, 52 — Nonpurulent Infections, 54 — Spirochetal Infections, 54 — General Paresis, 54 — T a b e s Dorsalis, 64 — Syphilitic Optic A t r o p h y , 66 — Neurotropic Virus Infections, 68 — Poliomyelitis, 68 — Epidemic Encephalitis, 72 — St. Louis Encephalitis, 72 — Lethargic Encephalitis, 78 — Herpes Simplex Encephalitis, 82 — " I n c l u s i o n " Encephalitis, 84 — Rickettsial Infections, 86 — Guillain-Barre Syndrome, 88

III

TOXIC AND NUTRITIONAL DISORDERS

90

H y p e r t h e r m i a , 90 — Radiation Necrosis, 92 — Nitrous Oxide Anesthesia, 96 — C a r b o n Monoxide Poisoning, 96 — Arsenical Poisoning, 98 — Hyperinsulinism, 100 — Prolonged C o m a in Insulin Shock T h e r a p y , 100 — Islet-Cell A d e n o m a , 102 — S y d e n h a m ' s Chorea, 104 — Hepatocerebral Degeneration, 106 — V i t a m i n B Deficiency, 110 — W e r nicke's Syndrome, 110 — Korsakoff's Syndrome, 112 — Pellagra E n c e p h a l o p a t h y , 114 — Subacute Combined Degeneration, 116 — S u b a c u t e Degeneration of the Cerebellum, 118

IV

DEMYELINATING DISORDERS

120

Perivenous Encephalomyelitis, 120 — Measles Encephalomyelitis, 120 — P o s t v a c c i n a l Encephalomyelitis, 126 — M u m p s Encephalomyelitis, 126 — Chickenpox E n c e p h a l o m y elitis, 126 — Perivenous Encephalomyelitis of U n k n o w n E t i o l o g y , 128 — Disseminated Encephalomyelitis, 132 — Subacute Necrotic M y e l i t i s and Encephalitis, 136 — Neuromyelitis Optica, 136 — Subacute Necrotic Encephalitis, 138 — M u l t i p l e Sclerosis, 140 — Concentric Sclerosis, 150 — Diffuse Sclerosis, 152 — Sporadic Forms, 152 — Heredofamilial Forms, 156 — K r a b b e ' s Disease, 156 — Polizaeus-Merzbacher Disease, 160 IX

V

V A S C U L A R DISORDERS

162

Cerebral Arterial Thrombosis, 162 — Cerebral Embolism, 170 — Aseptic Embolism, 170 — Septic Embolism, 172 — F a t Embolism, 172 — Air Embolism, 172 — Sinus and Venous Thrombosis, 174 — Cerebral Hemorrhage, 178 — Incomplete Interruption of Cerebral Circulation, 182 — Cerebral Arteriosclerosis, 184 — Cerebral Arteriolosclerosis, 188 — Cerebral Syphilitic Arteritis, 190 — " C o l l a g e n " Diseases, 192 — Disseminated L u p u s E r y t h e m a t o s u s , 192 — Purpura, N o n t h r o m b o c y t o p e n i c , 194 — R h e u m a t i c E n d arteritis, 196 — Periarteritis Nodosa, 198 — Cerebral A n e u r y s m s , 200 — Congenital A n e u r y s m s , 200 — M y c o t i c A n e u r y s m s , 202 — Atherosclerotic A n e u r y s m s , 202 — Functional Disorders of Circulation, 204 — " F a t a l " C a t a t o n i a , 204 — Epilepsy, 206 — Circulatory Arrest During Surgical Operations, 210 — Cardiac Arrest, 210 — Respiratory Arrest, 214

VI

TRAUMATIC DISORDERS

216

Closed Injuries, 216 — A c u t e Cerebral Contusion, 216 — A c u t e Uncal Herniation, 218 — Chronic Cerebral Contusion, 220 — Chronic Subdural H e m a t o m a , 222 — Chronic Uncal Herniation, 224 — Unusual Complications of Closed H e a d Injuries, 226 — Open Injuries, 228 — Prefrontal L o b o t o m y , 230 — P o s t t r a u m a t i c Abscess, 234 — P o s t t r a u m a t i c Pachymeningitis, 234 — T r a u m a t i c Myelitis, 234 — Intracranial Birth Injuries, 236 — Spinal Cord Birth I n j u r y , 238

VII

DEGENERATIVE DISORDERS

24O

Senile-Presenile Dementias, 240 — Senile D e m e n t i a , 240 — Alzheimer's Disease, 244 — P i c k ' s Disease, 248 — Unclassified Organic Brain Disease, 252 — E x t r a p y r a m i d a l Disorders, 254 — Huntington's Chorea, 254 — Parkinson's Disease, 256 — Wilson's Disease, 258 — D y s t o n i a Musculorum Deformans, 260 — Hallervorden-Spatz Disease, 262 — Cerebellar and Spinocerebellar Disorders, 264 — Hereditary Cerebellar A t a x i a , 264 — Sporadic Cerebellar A t a x i a , 268 — P a r e n c h y m a t o u s Cerebellar A t r o p h y , 270 — Friedreich's A t a x i a , 272 — Neuromuscular Disorders, 274 — Atrophies, 274 — A m y o t r o p h i c Lateral Sclerosis, 274 — Progressive Muscular A t r o p h y , 276 — Progressive Peroneal A t r o p h y , 278 — Progressive Hypertrophic Neuritis, 278 — Werdnig-HofFmann Disease, 280 — A m y o t o n i a Congenita, 282 — Dystrophies, 284 — M y a s t h e n i a G r a v i s , 284 — Cerebral Lipoidoses, 286 — A m a u r o t i c Idiocy, 286 — Gargoylism, 292 — " I d i o p a t h i c " Cerebral Calcification, 294

VIII

NEOPLASTIC DISORDERS

298

Gliomas, 298 — Medulloblastoma, 298 — Glioblastoma Multiforme, 302 — Astroblastoma, 306 — Spongioblastoma Polare, 308 — Infundibuloma, 310 — Gliomatosis Cerebri, 312 — A s t r o c y t o m a , 314 — Cerebral A s t r o c y t o m a , 314 — Cerebellar A s t r o c y t o m a , 316 — A s t r o c y t o m a of the A q u e d u c t of Sylvius, 316 — Subependymal A s t r o c y t o m a , 318 — M a l i g n a n t A s t r o c y t o m a , 318 — Oligodendroglioma, 320 — E p e n d y m o m a , 324 — x

Papilloma of Choroid Plexus, 328 — Pinealoma, 330 — Ganglioneuroma, 332 — Meningiomas, 334 — Meningioma of Olfactory Groove, 334 — Meningioma of Sphenoid Ridge, 334 — Meningioma of Tuberculum Sellae, 336 — Parasagittal Meningioma, 336 — Neurinomas, 342 — Blood Vessel Tumors, 344 — Hemangiomas, 344 — Hemangioblastomas, 346 — Congenital Tumors, 350 — Craniopharyngioma, 350 — Epidermoid Cyst, 352 — Teratomas, 352 — "Hamartoma" of Tuber Cinereum, 354 — Lipoma, 356 — Chordoma, 356 — Pituitary Adenomas, 358 — Chromophobe Adenoma, 358 — Eosinophil Adenoma, 360 — Metastatic Tumors, 362 — Metastatic Carcinoma, 362 — Metastatic Sarcoma, 366 — Metastatic Malignant Melanoma, 366 — Leukemia, 368 — Tumors Associated with Developmental Disorders, 370 — Tuberous Sclerosis, 370 — Von Recklinghausen's Neurofibromatosis, 378 — Syringomyelia, Syringobulbia, and Hydromyelia, 382 IX.

D E V E L O P M E N T A L DISORDERS

386

Agyria, 388 — Pachygyria, 392 — Microgyria, 394 — Agenesis, 398 — Agenesis of Corpus Callosum, 398 — Agenesis of Olfactory System, 400 — Agenesis of Optic System, 402 — Agenesis of Cerebellum, 404 — Dysraphic States, 406 — Porencephaly, 406 — Cranium Bifidum, 410 — Spina Bifida, 4 1 2 — Congenital Hydrocephalus, 4 1 2 — Congenital Hydrocephalus Associated with Spina Bifida, 4 1 2 — Congenital Hydrocephalus Associated with Atresia of Aqueduct of Sylvius, 414 — Nonspecific Malformations, 416 — Mongolism, 420 — Malformations and Maternal Rubella, 422

X

S E Q U E L A E OF P A R A N A T A L A N D P O S T N A T A L D I S O R D E R S

424

Primary Degeneration of Cortex, 424 — Generalized Cortical Atrophy, 424 — Cortical Hemiatrophy, 426 — Cortical " L o b a r " Atrophy, 428 — Cortical Cystic Degeneration, 428 — Pathogenesis of Primary Degeneration of Cortex, 430 — Primary Degeneration of White Matter, 432 — Generalized Atrophy of White Matter, 432 — Hemiatrophy of White Matter, 434 — "Lobar Sclerosis" of White Matter, 436 — Cystic Degeneration of White Matter, 438 — Status Marmoratus, 442 — Pathogenesis of Primary Degeneration of White Matter and of Status Marmoratus, 448 — Hydrocephalus, 450 — Toxoplasmosis, 454 — Kernicterus, 456

BIBLIOGRAPHY

INDEX

XI

463

459

Atlas of Neuropathology

I Cytology and Cellular Pathology Pathologic processes are based ultimately on cellular pathology. The nature of a particular disorder is determined by the manner in which nerve cells and fibers are altered, the type of reaction of the supporting cells evoked, and the kind of catabolic products produced. Different staining methods may be required to demonstrate such changes, since the various nerve structures have specific staining properties. STAINING

METHODS

Nissl Method. Aniline dyes, such as thionin, toluidine blue, or cresyl violet, stain the Nissl bodies of neurons and the chromatin of all nuclei varying shades of blue to purple (Fig. ia). Hematoxylin-Eosin Method. This nonspecific method stains the cytoplasm purplish red and the nucleus blue-black. In neurons undergoing ischemic or anoxic change, the cytoplasm stains a bright red that contrasts with the deep blue pyknotic nucleus (Fig. ib). Bielschowsky Method (or its von Braunmiihl modification). Silver nitrate stains the neurofibrils, and such argyrophilic pathologic structures as senile plaques, black against a slightly purplish background (Fig. ic). Davenport Method. Silver nitrate (similar to the Bielschowsky) stains the axis cylinders black against a purplish background (Fig. id). Weigert Method (or any of its modifications). Iron hematoxylin, in tissue previously mordanted with chrome and copper salts, stains the myelin sheaths blue-black in contrast to the slightly yellowish color of normally unmyelinated or demyelinated areas (Fig. ie). Weil Method. A solution of iron alum and hematoxylin stains the normal myelin sheaths a bluish gray against the light gray of unmyelinated and demyelinated structures, as in a degenerating peripheral nerve (Fig. if). Laidlaw Connective Tissue Method. A solution of lithium silver stains reticulin fibers black, as in a gumma (Fig. ig). Hematoxylin-van Gieson Method. A solution of iron hematoxylin and picric acid-fuchsin stains collagen fibers red, and nerve tissue and hemosiderin pigment yellowish brown, as in the capsule of a brain abscess (Fig. ih).

2

H S Fig. ia. Nissl x 475

Fig. ic. Von Braunmühl x 475

Fig. ig. Laidlaw x 200

Fig. ib. Hematoxylin-eosin x 475

Fig. id. Davenport x 475

STAINING METHODS

(continued)

Hortega Silver Carbonate Method. T h e microglia stain black against a purplish background (Fig. 2a). Cajal Gold Sublimate Method. T h e astrocytes and their processes stain black against a reddish-brown background (Fig. 2b). Holzer Method. C r y s t a l violet stains the glial fibers of astrocytes a deep blue, as in a glial scar (Fig. 2c). Scarlet-Red Method. Sudan I V , counterstained with alum hematoxylin, stains droplets of neutral fat deposited in gitter cells a brilliant red and the nuclei blue, as in a demyelinating lesion (Fig. 2d). Nile Blue Sulfate Method. T h i s method stains "prelipoids" ( f a t t y acids and lipids other than neutral fats) v a r y i n g shades of blue to violet, as in the neurons of cases of amaurotic family idiocy (Fig. 2e). Turnbull Blue Method. A solution of yellow-ammonium sulphide and potassium ferricyanide-hydrochloric acid, counterstained with carmine, stains iron blue, and the background purplish red, as in a hemorrhagic infarct (Fig. 2f). Kossa Method. Silver nitrate stains calcium black against a slightly yellowish background, as in cerebral calcification (Fig. 2g).

4

Fig. 2a. Hortega silver carbonate x 475

Fig. 2b. Cajal gold sublimate x 475

mm 9

mi'

Fig. 2d. Scarlet red x 475

Fig. 2c. Holzer x 200

Fig. 2e. Nile blue sulfate x 475

* V * , v •• # • j" i IM •