Acute Aseptic Meningitis Encephalitis and Myelitis Clinical Features

Meningitis presents with fever, general malaise, headaches, photophobia, nuchal rigidity, nausea, vomiting, and muscle aches. Altered states of consciousness, focal neurologic deficits, and seizures are common in encephalitis. Flaccid mono- or paraparesis suggests myelitis. The CSF yields a mild to moderate pleocytosis, typically with lymphocytes however, during the initial stage, PNLs may also be present. Protein is moderately elevated and glucose is normal. Viral-specific DNA and RNA are...

Lipoma

This is a rare benign tumor generally is situated over the corpus callosum or quadrigeminal plate, less often in the region of the tuber cinereum or cerebellopontine Lipoma associated with agenesis of the corpus callosum. Lipoma associated with agenesis of the corpus callosum. angle. It often coexists with the agenesis of the corpus callosum and convolutional malformations. Grossly and histologically, the lipoma resembles normal adipose tissue and may also contain variable amounts of connective...

Pineocytoma Grade

This benign tumor originates from mature parenchymal cells with neuroendocrine and photosensory functions. More common in adolescents and young adults, it accounts for 15 to 30 of parenchymal tumors. It is slowly growing, grossly circumscribed, and may extend into the third ventricle (Fig. 11.26A). Histologically, the tumor consists of uniform cells with a small nucleus and a moderate amount of cytoplasm containing secretory granules. In silver-stained sections, the cells display short...

Neuronal Inclusions in Viral Infections

In viral diseases, the inclusions are commonly intranuclear, except that in rabies they occur in the cytoplasm of the Purkinje cells and the pyramidal neurons of the Ammon's horn. inclusions immunoreact for tau, a microtubule-associated protein, others for a-synuclein, a synaptic protein, and still others for ubiquitin, a stress protein (Table 2.3). Intranuclear inclusions derived from the proteins of polyglutamate tracts characterize trinucleotide repeat diseases. Neurofibrillary tangles, a...

Schwannomas of the Spinal Nerve Roots

Nerve Van Gieson

The tumors constitute about 15 to 30 of spinal tumors. They are more common on the sensory roots, preferentially occurring on the cauda equina. Clinically, they present with pain and sensory and motor deficits in a radicular distribution. If they reach a large size, symptoms and signs of spinal cord compression develop. Hourglass or dumbbell schwannoma refers to extension of the tumor through the intervertebral foramen into the mediastinum or peritoneal cavity. Intraparenchymal schwannomas are...

Clinical Features

Progressive supranuclear palsy PSP affects middle-aged and elderly individuals. The disease is sporadic, but a few familial occurrences have been reported, some associated with mutations in the tau gene. The annual incidence rate is 5 per 100,000 population this figure increases with advancing age. The disease presents a broad spectrum of symptoms and signs including a supranuclear vertical and horizontal gaze palsy b motor disturbances, such as bradykinesia-akinesia, rigidity of the neck,...

Neuroaxonal Dystrophy with Brain Iron Deposition

Axonal Spheroids Lfb Stain

Neuroaxonal dystrophy with brain iron deposition or Hallervorden-Spatz disease is caused by mutations in the pantothenate kinase gene PANK2 located on chromosome 20. The disease can occur from infancy through adulthood. Extrapyramidal symptoms and signs are in the foreground of the clinical picture dystonic posturing, choreoathetotic movements, rigidity, and dysarthric speech. The movement disorders are associated with progressive mental deterioration. Specific diagnostic tests are not...

Pathology

Grossly, the cerebellar cortex is atrophic, the basis of the pons and middle cerebellar peduncles are shrunken, and the inferior olives are flattened see Fig. 5.35 . The substantia nigra and locus ceruleus are discolored, and the putamen is atrophic with grayish discoloration. Histologically, widespread degeneration of the neurons in three anatomic systems and the presence of distinct glial and neuronal cytoplasmic inclusions are the defining features. Neuronal degeneration occurs in the...

Hypoglycemic Encephalopathy

The brain's metabolism depends on a constant and adequate supply of glucose, because only small amounts of it are stored. A sudden drop in the blood glucose level to 30 to 40 mg 100 mL results in permanent brain damage within 1 to 2 hours. Major causes of hypogly-cemic episodes are insulin overdose in diabetic patients, Hepatic encephalopathy in a 53-year-old chronic alcoholic man with severe liver cirrhosis. Alzheimer type 2 astrocytes in basal ganglia display A large vesicular nuclei, scanty...

Canavans Disease or Spongy Degeneration

Metachromatic Leukodystrophy

Canavan's disease, spongy degeneration of the neuroaxis, is an autosomal recessive disorder caused by deficiency of the enzyme N-aspartoacylase, encoded by a gene on chromosome 17. Subsequently, the amino acid N-acetyl aspartic acid accumulates in the cerebrospinal fluid CSF and brain and is excreted in urine. Onset is in early infancy blindness and macrocephaly are characteristic clinical features. The cerebral pathology is distinguished by a the lack of myelination, extensive vacuolation, and...

Meningothelial Tumors Meningiomas Grades

Paraparesis Parasagittal

These generally benign tumors arising from the menin-gothelial cells of the arachnoidal cap cells occur in mid and late mid life, rarely in children. They constitute Pineoblastoma in an adult male. A. Macrosection of the tumor HE . B. The tumor fills the posterior part of the third ventricle LFB-CV . C. Small seeding tumorous nodule in subarachnoid space consists of small cells with round-to-oval hyperchromatic nuclei HE . D. The cells immunoreact for synaptophysin. E. Small tumorous nodules in...

Uremic Encephalopathy

Various neurologic complications develop in patients with renal failure and in patients receiving hemodialysis and peritoneal dialysis. Uremic encephalopathy presents with tremor, asterixis, myoclonic jerks, seizures, and stupor, which may progress to coma. Hypoglycemic encephalopathy. A 68-year-old diabetic man, following administration of 11 units regular insulin, suffered a hypoglycemic episode with a blood glucose concentration of 28 mg 100 mL for approximately 8 hours. During this episode,...

Spontaneous Intracerebral Hematoma

Hematoma Intracerebral

The majority of intracerebral hemorrhages are associated with hypertension, which affects the small parenchymal arteries and arterioles and leads to fibrosis, hyalinosis, and fibrinoid necrosis. Such diseased arteri-oles easily rupture, causing the hemorrhage. Less common causes of hemorrhages are vascular malformations, vasculopathies, hemorrhagic diseases, and hemorrhage within tumors see Table 4.10 also see the section, Various Stroke Etiologies . Hypertensive hemorrhages have predilections...

Angiomas

Venous Angioma

Angiomas, congenital conglomerates of abnormal vascular channels, are classified as arteriovenous, cavernous, and venous angiomas and capillary telangiectasis see Chapter 11 . Seizures and headaches are characteristic clinical manifestations. Ischemic episodes result Angioma. Massive frontal lobe hemorrhage from rupture of an arteriovenous malformation. Angioma. Massive frontal lobe hemorrhage from rupture of an arteriovenous malformation. when the angioma steals shunts blood from the...

Diseases with Abnormal Involuntary Movements Huntingtons Disease

Huntington's disease HD , a disorder of trinucleotide repeat expansion, is inherited in an autosomal dominant trait. The HD gene on chromosome 4 encodes the protein huntingtin, which is widely expressed in the central nervous system. The gene contains a CAG-trinucleotide repeat that normally expands up to 26. In HD patients, the repeat length expands to over 36. A repeat length of over 100 is associated with juvenile-onset disease and is usually transmitted by the father. The disease presents...

Reyes Syndrome

Reye Syndrome Diagnosing

This rare disease, predominantly of young children, usually develops acutely after a viral infection. Salicylate toxicity as a precipitating factor has been implicated in some cases. It manifests with fever, vomiting, enlarging liver, and rapidly progressing encephalopathy leading to death within days or a few weeks. High blood ammonia and low sugar levels are characteristic laboratory findings. Death occurs due to liver failure and raised intra-cranial pressure. Grossly, the brain is swollen,...

Parenchymal Neurosyphilis

Neurosyphilis

Parenchymal neurosyphilis presents 10 to 20 years after the initial infection under several forms. The characteristic clinical features of tabes dorsalis are summarized in Table 6.6. Grossly, the posterior nerve roots of the spinal cord are thin and grayish, and the posterior columns are translucent and atrophic, chiefly in the lumbosacral segments. Histologically, the spinal ganglia and the posterior nerve roots show lymphocytic infiltrations, fibrosis, and demyelination. The demyelination...

Astrocytic Tumors

Infantile Spasm Gross Pathology

Astrocytic tumors constitute the largest group of intracranial tumors in both adults and children. Their locations, gross and histologic features, biologic behavior, and genetic alterations vary greatly. The WHO distinguishes four grades of astrocytic tumors based on their histologic characteristics Table 11.5 . Astrocytic Tumors Grades 2, 3, and 4 Diffuse astrocytoma grade 2, anaplastic astrocytoma grade 3, and glioblastoma grade 4 constitute the largest group of primary intracranial tumors...

1

Is depleted of neurons, becomes spongy, and displays a dense astrofibrosis. The white matter is variably devoid of myelin, which is replaced by astrogliosis. Astrocytes in the cerebral cortex and oligodendrocytes in the white matter may display argyrophilic and tau-immunoreactive cytoplasmic inclusions. Dementia with Parkinsonism Linked to Chromosome 17 Clinical features. This is a familial dementia inherited in an autosomal dominant pattern The interest of the disease lies in its association...

Vascular Lesions

Herniations, by pressing the cerebral arteries against the surrounding structures, produce pale or hemorrhagic infarcts Fig. 2.20 see also Table 2.7 . Types of cerebral herniations. A. Anatomic specimen showing the structures in the tentorial opening. Note the relationship between the midbrain, oculomotor nerves, posterior cerebral arteries, uncus, and parahippocampal gyrus. B. Transtentorial herniation of the left uncus and parahippocampal gyrus in an acute subdural hematoma. Note the deep...

Subarachnoid Hemorrhage

Basal Subarachnoid Hemorrhage

The rupture of a saccular berry aneurysm is the most common cause of a nontraumatic subarachnoid hemorrhage SAH Fig. 4.26 . Common sites of aneurysms are the circle of Willis at the junctions of major arteries and at their first bifurcation. Less common sites are the tip of the basilar artery and the junction of the vertebral arteries. Aneurysms vary in size from a few millimeters to 2 to 3 cm giant aneurysms . They are often multiple, situated bilaterally as mirror images Fig. 4.27 . A...

Vitamin B12 Cobalamine Deficiency

Pernicious Anemia Spinal Cord Tracts

The intestinal absorption of vitamin B12 requires its binding to an intrinsic factor synthesized by the parietal cells of the gastric mucosa. An autoimmune gastritis, by interfering with the production or function of this intrinsic factor, is a major cause of the impaired absorption of vitamin B12. Other causes are gastrectomy, enteritis, malabsorption diseases, malignancies, and fish tapeworm infestation. Subacute combined degeneration of the spinal cord and megaloblastic pernicious anemia are...

Central Pontine Myelinolysis

Central pontine myelinolysis has been reported in malnourished chronic alcoholics. It also occurs in liver dis eases, liver transplants, severe burns, and hyponatremia. A rapid correction of hyponatremia is suggested to be a major cause of the disease. The onset is acute, with para- or quadriparesis, dysarthria, and dysphagia resembling the locked-in syndrome. The prognosis is poor. Death may occur within a few weeks a small number of patients may recover partially or completely. The diagnosis...

Neuronal Inclusion Bodies in Degenerative Diseases

Neuronal cytoplasmic inclusion bodies are the distinguishing features of several important neurodegenera-tive diseases. Some are identified in hematoxylin-eosin HE -stained sections, others only in silver-stained sections, and still others with immunohistologic stains see Fig. 2.3 . The molecular components of these inclusions are identified using immunohistochemical stains. Some Diseases with Neurofibrillary Tangles Parkinson-Dementia-ALS complex of Guam Progressive supranuclear palsy...

Ischemic Stroke Cerebral Infarction

Lacunar Infarcts

The brain requires a constant and adequate blood flow to supply oxygen and glucose essential for its high energy metabolism. A constant blood flow is assured by an autoregulatory mechanism of arteries and arterioles they constrict in response to rising systemic blood pressure and dilate in response to falling systemic blood pressure. This mechanism operates while the arterial pressure remains between 50 and 160 mm Hg. One- Atherosclerosis of cerebral arteries. A. Severe atheromatosis of basal...

Lysosomal Carbohydrate Diseases

Purkinje Cell Degeneration

Deficiencies of lysosomal enzymes necessary for the degradation of complex carbohydrates result in an accumulation of nondegraded products in various tissues and cells. Such products are the mucopolysaccharides, the mucolipids, the glycoproteins, and the glycogen. The diseases affect chiefly infants and children. Despite genetic and enzymatic heterogeneity, many diseases share facial and skeletal abnormalities, multiorgan manifestations, and urinary excretion of abnormal metabolites,...

Acute Disseminated Postinfectious and Postvaccination Encephalomyelitis

Bal Concentric Sclerosis Pathology

Acute disseminated postinfectious and postvaccination encephalomyelitis develops 1 to 3 weeks following a Balo's concentric sclerosis shows zones of myelin losses alternating with zones of intact myelin in a circular fashion myelin stain . viral or bacterial infection such as measles, varicella, rubella, influenza, mumps, infectious mononucleosis, or scarlet fever, or it develops following vaccination for rabies, smallpox, measles, typhoid, and paratyphoid. The onset is acute with headaches,...

Herpes Simplex Virus1 Infection

Herpes simplex virus-1 infection is the major cause of sporadic and malignant encephalitis, chiefly in adults and young subjects. The infection is acquired by exposure to contaminated saliva or respiratory secretion. The virus initially causes a nasopharyngitis. By retrograde axonal transport, it reaches the trigeminal ganglia, where it becomes latent. Reactivation of the virus produces herpes vesicles on the lips cold sore or oral mucosa. The brain is infected by spread of the virus along the...

Diseases with Akinetic Rigidity Idiopathic Parkinsons Disease

Lewy Body Pathology

Idiopathic Parkinson's disease iPD , the most common movement disorder and a major cause of neurologic Akinetic-rigid form Parkinson's disease Progressive supranuclear palsy Dementia with parkinsonism linked to chromosome 17 Striatonigral degeneration Corticobasal degeneration Postencephalitic parkinsonism Parkinsonism-dementia complex of Guam Hyperkinetic form Huntington's disease Dystonias disability in the elderly, may affect individuals of 40 to 60 years of age, but it is seen primarily in...

Cerebral Pathology In Chronic Epilepsy

Coma Cerebral

Hypoxic-ischemic lesions often are encountered in the brains of patients who suffer from epilepsy with generalized tonic-clonic seizures and status epilepticus. The lesions result from respiratory and circulatory Prolonged coma following cardiac arrest. Chronic cortical pan-necrosis. Three-month survival in a 69-year-old man. A. Transverse section of the cerebral hemispheres shows thinning and sponginess of the cortex, necrosis of the basal ganglia, and focal softening of the white matter. The...

New Variant CJD

Microvacuolation

In 1994 and1995, a little less than 10 years after the outbreak of BSE in Europe, a new variant of CJD nvCJD was recognized in the United Kingdom and France. The disease is distinguished by an earlier onset of behavioral changes and psychiatric symptoms, followed by cerebellar dysfunction and myoclonus. Later in the course, dementia develops. The course is protracted. Bilateral pulvinar hyperintensities on MRI are held to be characteristic. Because PrPsc is detectable in lymphoid tissue,...

Meningovascular Syphilis

Neurosifilis Meningovascular

Meningovascular syphilis manifests 5 to 7 years after the initial infection and presents with meningeal and multifocal cerebral and or spinal cord symptoms and signs. The pathology is characterized by a proliferative endarteritis of the small, medium, and large arteries Heubner endarteritis . A prominent endothelial proliferation along with mononuclear cell infiltrates in the media and adventitia lead to severe luminal narrowing and, eventually, occlusion and subsequent infarctions Fig. 6.25 ....

Purulent Leptomeningitis

Purulent leptomeningitis refers to an inflammation of the pia and arachnoid with a purulent exudate in the subarachnoid space. Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae are responsible for about 75 of these infections. 6 mos.-2 yrs. Children-young adults Adults B. streptococcus Listeria monocytogenes Staphylococcus aureus Haemophilus influenzae Neisseria meningitidis Streptococcus pneumoniae Purulent leptomeningitis occurs at all ages from birth through old...

Pathology of the Neurons

Neuron Achromasia

The reactions of neurons to disease manifest in alterations in the size, shape, and staining properties of the Neuronal alterations. A. Vacuolated swollen motor neuron in medulla. B. Chronic atrophy of pyramidal neuron The perikaryon is shrunken, the dendrites are corkscrew-like, and the nucleus is densely basophilic. C. Axonal or retrograde changes in hypo-glossal neuron caused by tumorous infiltration of the nerve roots. The swollen perikaryon is rounded, the dendrites are retracted, the...

Introduction to Clinical Neuropathology

Corticospinal Tract Substantia Nigra

The objectives of the neuropathologic examination are twofold First, to identify and localize any lesion s , interpret histologic changes and ultimately, formulate a diagnosis. Second, to correlate the location and histopathologic features of the lesion s with the clinical presentation. Fulfilling these goals requires familiarity with the anatomy and histology of the nervous system. For this purpose, photographs of representative brain and spinal cord slices and myelin-stained sections are...

Cerebral Changes In Physiologic Aging

Lipofuscin Accumulation Brain

Aging is an inescapable natural biologic process affecting all organ systems of the body. This process, regulated by genetic factors longevity genes and influenced by environmental factors, begins after age 50 to 60 years or later. Aging of the central nervous system primarily A. Normal pressure hydrocephalus in a 60-year-old man with gait difficulties over the years and memory decline. MRI shows enormously enlarged ventricles and only mildly enlarged subarachnoid space. B. Hydrocephalus ex...