Normal Lab Values

Nervous System Pathology Part 1

Readings from Robbins Basic Pathol, 9th ed.
by TUESDAY
   
 
pages
topics
     
ALL Lab Sections

pp 228-233

Lysosomal diseases - Glycogen diseases
     
 
pp 820 - 822
CNS trauma
     
 
pp 824 - 842
CNS infections - Neurodegen. diseases
     

Case assignments by lab group and class day:

  TUESDAY THURSDAY
Labs 1, 2, & 3
Labs 4 & 5

 

CASE NUMBER 504
(no virtual slide for this case)

This case will be presented by neurology faculty - click here when instructed to see images related to this case

 

 

CASE NUMBER 543

This case will be presented by neurology faculty - click here when instructed to see images related to this case

 

CASE NUMBER 515
(no virtual slides for this case)

Clinical History: A 46-year-old man with a long history of alcohol abuse was brought to the emergency department by the police when he was found walking unsteadily and seemed confused. His speech was slurred and slow, though his blood alcohol was 0.01%. A mini mental status exam revealed that his short term memory was severely impaired. Physical exam revealed a right leg tremor and nystagmus. A cranial MRI was performed, but while the patient was still in radiology, when the patient tried get off of the gurney, he fell, hit his head on the floor and died.

Image Gallery:

(Summary of Radiographic Findings)
MRI FLAIR imaging showed marked hyperintensity of the tectal region, periaqueductal area, mamillary bodies, and medial thalamic nuclei.
(Summary of Gross Findings)
There are hemorrhages in the mammillary bodies which appear as petechiae.

515-1. What is the differential diagnosis?

ANSWER

 

515-2. The constellation of symptoms shown by this patient is known as which of the following?

  1. Kwashiorkor
  2. Leigh syndrome
  3. Parkinsonism
  4. Ricketts
  5. Wernicke-Korsakoff syndrome

ANSWER

 

515-3. Which of the following typically develops late in this disease?

  1. Amnesia
  2. Ataxia
  3. Cognitive impairment
  4. Confusion
  5. Nystagmus

ANSWER

 

515-4. Which of the following is true regarding this patient’s symptoms?

  1. Ataxia rarely improves with appropriate treatment
  2. Confusion rarely improves with appropriate treatment
  3. Memory loss typically improves with appropriate treatment
  4. Nystagmus typically improves with appropriate treatment

ANSWER

 

515-5. In addition to alcoholism, in which of the following clinical settings is this disease process MOST LIKELY to arise?

  1. Chronic blood loss
  2. Gastric disorders
  3. Inborn error metabolism
  4. Obesity
  5. Renal disease

ANSWER

 

 

CASE NUMBER 301

This case will be presented by Infectious Disease faculty - click here when instructed to see images related to this case

 

 

CASE NUMBER 300

This case will be presented by Infectious Disease faculty - click here when instructed to see images related to this case

 

CASE NUMBER 503
(no virtual slide for this case)

Clinical History:  A mother brought her 3-month-old infant to the pediatrician because the child had been alternately crying inconsolably or lying down listlessly; vomiting for the last 3 days, and had a mild fever. Physical exam reveals cherry red maculas. CSF analysis shows elevated protein levels. Gene sequencing reveals a mutation in the alpha-subunit of hexosaminidase A.

Image Gallery:

(Summary of Clinical Findings)
The ophthalmic exam shows a cherry red macula.
(Summary of Electron Microscopic Findings)
Electron micrograph findings: These are cytoplasmic inclusions full of layered membranes.
 

 

503-1. What is the differential diagnosis?

ANSWER

 

503-2. This disease is classified as which of the following?

  1. Glycogenosis
  2. Mucopolylipidosis
  3. Mucopolysaccharidosis
  4. Sphingolipidosis
  5. Sulfatidosis

ANSWER

 

503-3. Which of the following is true regarding this disease?

  1. Boys are affected more commonly than girls
  2. Most patients recover full neurological function in their teens
  3. The “cherry red macula” is due to retinal hemangiomas
  4. There is an increased incidence in Ashknazi Jews
  5. These patients are at increased risk for colon cancer

ANSWER

 

 

 

NERVOUS SYSTEM PATHOLOGY Review Items

Key Vocabulary Terms (click here to search any additional terms on Stedman's Online Medical Dictionary)

agyria gemistocytic neuronophagia
AIDS dementia gliosis neuropathy
Alzheimer type II cell glomeruloid body  Nissl substance
amyloid angiopathy granulovacuolar degeneration open head injury
anencephaly herniation ophthalmoplegia
Antoni A pattern Hirano body pachygyria
Antoni B pattern holoprosencephaly pachymeningitis
arrhinencephaly hydranencephaly paresis
aseptic meningitis hydrocephalus parkinsonism
astrocytosis hydrocephalus ex vacuo peripheral nervous system (PNS)
ataxia kernicterus phakomatosis
Bergmann gliosis lacunar infarct  Pick body
central nervous system (CNS) Lafora body polymicrogyria
cerebral laminar cortical necrosis porencephaly
cerebral palsy (CP) leptomeningitis presenilin
cerebritis leukoencephalopathy prion
cerebrospinal fluid (CSF) leukomalacia prion protein (PrP)
cerebrovascular accident (CVA) Lewy body radiculitis
choreiform lissencephaly Rosenthal fiber
chromatolysis mad cow disease rosette/pseudorosette
closed head injury megalocephaly satellitosis
concussion meningitis schizencephaly
contrecoup injury meningocele  spina bifida
contusion meningoencephalitis spongiform
corpora amylacea meningomyelocele storage disease
coup injury meningovasculitis stroke
cranial microcephaly syringomyelia
dementia multiple sclerosis plaque tabes dorsalis
demyelination myelitis transient ischemic attack (TIA)
Duret hemorrhage myelocoele ulegyria
encephalitis   Negri body von Recklinghausen disease
encephalocele neuritic plaque Wallerian degeneration
encephalomyelitis neuritis watershed (border) zone
encephalopathy   neurofibrillary tangle  

 

CNS-1: GOALS and LEARNING OBJECTIVES

Goal: Trauma and the Brain
Apply knowledge of the structure and function of the brain and general pathology concepts to discuss disorders resulting from trauma to the brain and its blood supply.

  • Objective 1: Traumatic Brain Injury
    Describe the pathologic findings seen in the most common causes of traumatic brain injury.
  • Objective 2: Traumatic Vascular Injury
    Compare and contrast the clinicopathologic findings seen in two types of traumatic vascular injury (i.e. epidural hematoma, subdural hematoma)

Goal: CNS Infection
Apply knowledge of clinical features, neuroimaging studies and location of lesions(s) to develop a differential diagnosis for CNS infection.

  • Objective 1: Infections of the CNS
    Compare and contrast the clinical, gross, and microscopic manifestations of common bacterial, viral, and fungal infections of the central nervous system.
  • Objective 2: Opportunistic Infections of the CNS
    List common opportunistic infections that involve the CNS of immunocompromised individuals and describe their pathologic features.
  • Objective 3: Progressive Multifocal Leukoencephalopathy
    Outline the clinicopathologic features of Progressive Multifocal Leukoencephalopathy (JC virus).
  • Objective 4: Suppurative Meningitis and Abscess
    Describe the gross and microscopic features of acute suppurative meningitis and brain abscess; and name the organisms most commonly associated with each.

Goal: Demyelinating Disorders
Apply knowledge of the structure and function of the brain and general immunopathology concepts to summarize disorders that result in demyelination in terms of their etiology, pathogenesis, clinical and morphologic features, natural history and therapeutic options.

  • Objective 1: Multiple Sclerosis
    Describe the autoimmune mechanism and clinicopathologic features of multiple sclerosis.

Goal: Dementia
Apply knowledge of structure and function and general pathologic concepts to describe disorders where dementia is a component.

  • Objective 1: Amyloid and Tau in Dementia
    Define the essential underlying abnormalities of amyloid and tau proteins in the most common causes of dementia in the US.
  • Objective 2: Alzheimer disease
    Describe the clinical features, gross pathology, histopathology and etiology of Alzheimer disease and the regions of the brain that are usually involved.
  • Objective 3: Disorders of the Basal Ganglia
    Name several diseases that involve the basal ganglia and describe how to distinguish among the diseases in terms of gross, microscopic, and clinical pathology.

 

 

 

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