History:

This 61-year-old man, who has a long history of alcohol abuse, was sitting on a bar stool when he was noted to suddenly fall to the floor. He was unable to arise and the paramedics were called. When they arrived, he was able to answer questions and he stated that he had a severe headache. Upon arrival to the hospital the admitting physical examination demonstrated a right hemiparesis. The patient became increasingly somnolent after admission.

Further history:

In spite of supportive care, the patient became comatose and died two hours after admission.

Image 1.1

This is a CT scan of the patient's head upon admission. What is the probable diagnosis?

Image 1.2

This is a coronal section of the brain and midbrain at autopsy. What is the mechanism of death in this case?

Questions:

1. What are possible causes of this acute incident?
2. What treatment could have prevented this event?
3. Is there any treatment after the event occurs?
4. What happens if the patient does not regain consciousness?

CASE 2

History:

This 81-year-old man was in good health until developing a cough with the production of yellow sputum. He complained to his relatives of a headache the day before admission. He was found stuporous by his son on the day of admission. In the emergency room, the physical examination demonstrated an elderly man who was not responding very well to questions. His temperature was 38 C (99.7 F), respirations 16/min, pulse 100/min and weak, and blood pressure 110/50 mm Hg. His neck was stiff. A lumbar puncture revealed cloudy cerebrospinal fluid with a marked pleocytosis with 1500 WBC's (90% of them PMN's), no RBC's, glucose of 31 mg/dL, and protein of 60 mg/dL. His serum glucose was 80 mg/dL.

Further history:

The patient does not respond to treatment and dies.

Image 2.1

This is a gross photograph of his brain. Describe the appearance. What is the pathogenesis of this lesion?

Image 2.2

This is a microscopic photograph with H&E staining of the subarachnoid space. Describe the microscopic findings.

Questions:

1. What is the diagnosis? What is the most likely organism in this man?
2. What would be your treatment?
3. What is a possible cause of death in this man?

CASE 3

History:

This 68-year-old man was noted by his family to have become forgetful in the months before being seen by his family physician. He was brought to his physician by his son because he had been found wandering in the street and could not find his way home. On physical examination, he was unable to remember any of 3 objects after 3 minutes and, although an avid football fan, he was unable to recount the previous Monday night's game which he had watched with his son. A CT scan was obtained and showed mild cerebral atrophy.

Image 3.1

This is the gross appearance of the brain from a man who died from the same disease suffered by this patient. Describe the findings.

Image 3.2

This is a microscopic section of brain stained with H&E. Describe the abnormalities. Do these changes help make a definitive diagnosis?

Image 3.3

This is another microscopic section of brain stained with a silver stain. What is the significance of this finding? What would you advise the family about this disease?

Questions:

1. What is the apparent diagnosis?
2. What other tests would you order on this man?
3. What are the major causes for this condition?
4. Are there potential therapies for this condition?
5. Explain the biochemistry and genetics of his disease.
6. The patient's son wants to know what arrangements he should make to care for his father. Does he need to be placed in a care facility, and who makes that decision?

CASE 4

History:

This 58-year-old man had a history of alcohol abuse. He developed increasing weakness on his right side over several days. Upon admission he was mildly agitated and complaining of a headache. His right arm and leg were weak and there was flattening of the nasolabial fold on the right. He denied any recent head trauma. A head CT scan was obtained.

Image 4.1

This is the CT scan. What does it show? What is the diagnosis?

Image 4.2

This is a gross photograph of a similar lesion in a patient who died. What would have been the treatment for this lesion?

Questions:

1. What are the possible causes for his weakness?
2. Why did the patient deny any history of recent trauma?
3. What age groups commonly present with this type of lesion after head trauma?
4. What blood vessels are ruptured to produce this lesion?

CASE 5

History:

This 52-year-old man had presented at age 37 with blurred vision. This lasted for several weeks. Five years later he suffered an episode of right leg weakness which resolved over several months. Over the ensuing 10 years he developed dysarthria, internuclear ophthalmoplegia, and paraplegia with spasticity. He became bedridden and died of a pulmonary embolus. At the time of his initial evaluation, a spinal tap revealed a normal CSF pressure, 6 cells (all lymphocytes), an elevated protein, and a normal glucose. Protein electrophoresis revealed an elevation in IgG. An MRI was performed six years before his death and showed several T2 bright images in the white matter of the cerebral hemispheres.

Image 5.1

This is a CT scan from another patient with the same disease. Describe the findings. What is the normal age range for this disease?

Image 5.2

This is a gross photograph of the brain from this patient. Describe the abnormalities. Are these old or new lesions?

Image 5.3

This is a microscopic section with myelin stain of one of the lesions. Describe the changes.

Image 5.4

This is a microscopic section with a silver stain for axons of one of the lesions. What appears to be preserved?

Image 5.5

On this H&E stained microscopic section, note the perivascular lymphocytes in the lesion. What are some of the theories regarding pathogenesis in this disease? What are some of the treatment modalities for this disease?

Questions:

1. What is the most likely diagnosis?
2. What other test would help in confirming your diagnosis? What would you tell the patient about the prognosis?
3. Can this patient refuse further treatment?

CASE 6

History:

This 50-year-old woman was in her normal state of good health when she began to notice a funny feeling in her left hand. Over the ensuing weeks she began to notice a continual nagging headache which was partially relieved with acetaminophen. On the morning of admission she had a grand mal seizure witnessed by her husband. Upon arrival at the emergency room she was awake and slightly disoriented but could give a good history. On physical examination there was mild weakness of the left arm and leg with paresthesias of the left hand.

Image 6.1

This is an enhanced MRI scan of the right hemisphere as seen sagittally. Describe the findings and give possible etiologies for the lesion seen.

Image 6.2

This is an H&E stained microscopic section of the biopsy taken from the lesion. Describe the changes. What is the diagnosis? What is the treatment for this lesion?

Image 6.3

This is a gross photograph of a similar lesion from an elderly man who died. Describe the gross changes. What is the usual age at presentation for patients with this lesion?

Questions:

1. What are possible causes for these symptoms?
2. What would be part of your workup on this patient?

CASE 7

History:

A 25-year-old woman was admitted to the hospital for left sided focal seizures and obtundation. She had complained of increasing headaches over the weeks prior to admission. There was also some clumsiness of her left hand and leg. She noted clonic jerking of her right arm lasting approximately five minutes which resolved with some weakness in her arm. Twenty minutes later, a similar episode occurred. On admission she was barely responsive and had a temperature of 37.8 C (100 F). She was then noted to have a left hemiparesis.

Image 7.1

This is an enhanced head CT scan. Describe the findings. Do they help narrow down the differential diagnosis?

Further history:

The patient was found to have alpha streptococcus growing in one of her blood cultures, and an echocardiogram demonstrated an atrial septal defect.

Image 7.2

This is a gross photograph of a section of brain from another patient with the same problem. Describe the findings.

Image 7.3

This is a microscopic section with connective tissue (trichrome) stain of the lesion after some months have passed. What are the various layers of this lesion?

Questions:

1. What are the possible causes for this type of presentation?
2. What studies would help define this situation?
3. What further studies would help define the etiology of the lesion in the brain?
4. How would you treat her disease? What is the prognosis?

CASE 8

History:

This 55-year-old man presented with the acute onset of left sided headache and mild right leg paresis. On CT scan a focal area of hemorrhage was seen near the gray white junction in the mid left frontal area. There was a questionable lesion in the right parietal lobe, but this was not well defined. It was decided to evacuate the lesion because of the mild mass effect and symptoms.

Image 8.1

This T1 weighted post-contrast MRI scan in coronal view demonstrates the lesion.

Image 8.2

This microscopic section shows the cellular portion of the lesion evacuated and sent to surgical pathology.

Further history:

On questioning, the patient admitted to noting some blood-tinged urine in the weeks prior to his admission. He did not have any dysuria or urgency. A CT scan of the abdomen revealed a large mass in the right kidney.

Image 8.3

This gross section of the brain is from another individual with the same disease. There is a well circumscribed hemorrhagic lesion in the cortex with some surrounding edema. What tumors commonly metastasize to brain?

Questions:

1. What are the possible etiologies for this lesion? What would be your follow-up after the discovery of the lesion?
2. What is the most likely diagnosis which explains both lesions? What is the treatment?