A 56 year old man develops a 2 year history of increasing hand and forearm weakness and clumsiness. He later develops dysarthria and dysphagia. A year later he dies of pneumonia. An image shows two sections of his spinal cord with arrows pointing to pathology. The most likely diagnosis is motor neuron disease. Briefly, motor neuron disease involves the degeneration of motor neurons in the brain and spinal cord, leading to progressive muscle weakness. The prognosis is poor as there is no cure. Microscopically, there would be loss of motor neurons.

1. CNS Degenerative disorders…
…To leave the world a better place. To know even
one life has breathed easier because you have
lived… that is success..! -- Ralph Waldo Emerson 1

2. 52y male, dementia, depression with choreiform movements.
Image of his brain(B) compared with normal (A) ?Diagnosis
A. Wernicke’s enceph.
A B
B. Parkinsons disease
C. Alzheimers disease
D. Pick’s disease
E. Huntington’s disease
100%
0% 0% 0% 0%
A. B. C. D. E.

3. 38y male progressive weakness, spasticity, fasciculations
and loss of muscle mass. Image shows section of spinal
cord stained for myelin. Diagnosis?
1. Multiple sclerosis
2. Amyotrophic Lateral
Sclerosis
3. Motor neurone disease
4. Syringomyelia
5. Spinal cord infarction.
80%
20%
0% 0% 0%
1 2 3 4 5

4. 38y recurrent limb weakness 9m, MRI ?
Diagnosis
1. Multiple sclerosis
2. Parkinsons
3. Alzheimer’s
4. Huntington’s
5.
100%
Pick’s disease
0% 0% 0% 0%
1 2 3 4 5

5. 58y male personality change,
depression, with chorea. ? Diagnosis
1. Multiple sclerosis
2. Parkinsons
3. Alzheimer’s
4. Huntington’s
5. Pick’s disease
60%
30%
10%
0% 0%
1 2 3 4 5

6. A 65 year old woman with 3y history of emotional disturbances, irritability,
irresponsible and difficulty with activities of daily living. Now she has difficulty talking.
Image shows appearance of her brain. What is the most likely diagnosis?
A. Huntington’s disease.
B. Alzheimer’s disease.
C. Pick’s disease.
D. Parkinson’s disease
E. Multiinfarct dementia.
80%
20%
0% 0% 0%
A. B. C. D. E.
A. Briefly discuss pathogenesis of this condition?
B. List 3 gross and Microscopic feature of this disease?
C. List 3 differentiating features of Alzheimers & this disease?.

7. A 56-year-old man is rushed to the emergency room after
collapsing while shoveling snow. The patient has no pulse
on admission but is resuscitated. Laboratory studies show
elevated serum levels of cardiac-specific proteins, and ECG
confirms a transmural infarct of the left ventricle. The patient
expires 2 weeks later of cardiac tamponade. Examination of
the patient's brain at autopsy would most likely reveal
necrosis of Purkinje cells and selective loss of neurons in
which of the following regions?
70% A. Frontal lobes
B. Hippocampus
C. Hypothalamus
20%
D. Occipital lobes
10%
0% 0% E. Thalamus
A. B. C. D. E.

8. A 28 year old woman with ataxia, diplopia on lateral gaze and flashes of light on eye
movement. CSF analysis shows increased proteins, lymphocytes and oligoclonal IgG
bands and normal glucose. Image shows gross specimen of brain with lesions typical
of this disease. What is the most likely diagnosis?
A. Parkinson’s disease
B. MCA infarct.
C. Lacunar Infarcts.
D. Multiple sclerosis.
E. Viral encephalitis.
56%
22%
11% 11%
0%
A. B. C. D. E.
A. Briefly discuss etiology & pathogenesis of this condition?
B. Describe the gross features shown in the image?
C. List other clinical features? What is the prognosis of this condition?

9. 38y female recurrent limb weakness. Image
shows white mater stained for myelin.? Diagnosis
A. Multiple sclerosis
B. Parkinsons
C. Alzheimer’s
D. Huntington’s
E. Pick’s disease
100%
0% 0% 0% 0%
A. B. C. D. E.

10. 72y Male progressive severe dementia,
Image shows amyloid stain of his brain.
? Diagnosis
1. Pick’s disease.
2. Huntington’s disease.
3. Alzheimer’s disease.
4. Multi-infarct dementia.
5. Senile dementia.
100%
0% 0% 0% 0%
1 2 3 4 5

11. 38y fem recurrent tremor & limb weakness
progressive over 13 years. ? Diag
1. Multiple sclerosis
2. Hypertensive-stroke.
3. Alzheimer’s
4. Huntington’s
5. Pick’s disease
89%
11%
0% 0% 0%
1 2 3 4 5

12. A 48 year old man with a year long history of worsening jerky, choreo-athetoid
movements of all limbs, bradykinesia and rigidity. Image shows coronal section of a
brain specimen showing typical features of his disease. What is the most likely
diagnosis?
A. Picks disease.
B. Lewy body disease.
C. Huntington’s disease.
D. Multiple sclerosis.
E. Amyotropic lateral sclerosis.
67%
33%
0% 0% 0%
A. B. C. D. E.
A. Briefly discuss pathogenesis of this condition? (Trineucleotide repeat dis)
B. Describe the gross features shown in the image?
C. what other clinical features are expected in this disease?

13. A 60-year-old man with a 15-year history of diabetes mellitus
type II complains of deep burning pain and sensitivity to
touch over his hands and fingers. Nerve conduction studies
show slow transmission of impulses and diminished muscle
stretch reflexes in both ankles and knees. Sensations to
vibrations and light touch are also markedly diminished.
Laboratory analysis of CSF shows no biochemical
abnormalities. Which of the following is the most likely type
of peripheral nerve disease in this patient?
55% A. Autonomic neuropathy
B. Distal polyneuropathy
36%
C. Inflammatory neuropathy
D. Mononeuropathy
9% E. Paraproteinemic
0% 0%
polyneuropathy
A. B. C. D. E.

14. 58y M, Progressive bradykinesia, dementia &
Hallucinations ? Diagnosis
A. Alzheimer’s
B. Parkinson’s
C. Motor neurone disease
D. Syringomyelia
E. Lewy body disease
50%
40%
10%
0% 0%
A. B. C. D. E.

15. A 35-year-old man with Down syndrome dies of acute
lympho-blastic leukemia. Gross examination of the patient's
brain at autopsy shows mild microcephaly and
underdevelopment of the superior temporal gyri. Histologic
examination would most likely show which of the following
neuropathologic changes?
60% A. AA amyloidosis
B. Lewy body dementia
C. Leukemic infiltrates
20%
D. Neurofibrillary tangles
10% 10% E. Old healed infarcts.
0%
A. B. C. D. E.

16. A 35 year old HIV +ve man with Kaposisarcoma has a 3 day history of headache and
confusion followed by seizures and hemiparesis. CT scan showed multiple ring-
enhancing lesions. Image shows Gross appearance and MRI of a brain typical of this
disorder. What is the most likely diagnosis?
A. Multiple Aspergilloma.
B. Cerebral TB lesions.
C. Multiple Abscesses.
D. Multiple sclerosis plaques.
E. Cerebral metastases.
40%
30% 30%
0% 0%
A. B. C. D. E.
A. What does “ring enhancing” lesion mean?
B. Briefly discuss etiology and pathogenesis?
C. List other pathologic findings expected in the brain of a AIDS patiets?.

17. 38y fem, recurrent attacks of limb weakness, paraesthesia
worsened over 7 years resulting in death due to respiratory
failure. Image shows her brain specimen. What is the most
likely Diagnosis?
1. Multiinfarct dementia.
2. Parkinsons disease.
3. Multiple sclerosis
4. Motor neurone disease.
5. Lacunar infarcts.
90%
10%
0% 0% 0%
1 2 3 4 5

18. 78y M, Dementia, memory loss and spacial
disorientation. Cortical biopsy. ? Diagnosis
1. Alzheimer’s
2. Parkinson’s
3. Lewy body disease
4. Huntington’s disease
5. Pick’s disease
100%
Cortical biopsy - β amyloid immunostain
0% 0% 0% 0%
1 2 3 4 5

19. 78y, Dementia, Aphasia, Image shows
Hippocampus biopsy stained with anti tau stain.
? Diagnosis
A. Multiple sclerosis
B. Parkinsons
C. Alzheimer’s
D. Multi-infarct dementia
E. Pick’s disease
55%
36%
9%
0% 0%
A. B. C. D. E.

20. 78y Male progressive severe dementia,
Image shows amyloid stain of his brain.
? Identify structure A
1. Neurofibrillary tangles.
2. Amyloid plaques.
3. Amyloid Angiopathy
4. Astrocytes - Gliosis.
5. Neuronophagia.
90%
10%
0% 0% 0%
1 2 3 4 5

21. 58y female vocalist difficulty remembering songs, depression,
dementia & gait abnormality. Image shows brain biopsy.
What is the most likely diagnosis?
1. Alzheimer’s disease.
2. Pick’s disease.
3. Huntington’s disease.
4. Lewy body disease.
5. Creutzfeldt-Jakob disease.
90%
CJD: Spongiform degeneration (vacuoles) no inflammation - Slowly
progressive ataxia and dementia.
Prion protein (APrP amyloid) – form template to make more abnormal protein
(not a living being) Infection  From contaminated brain of infected animal.
10%
Prion0%
diseases: Kuru, CJD, Scrapie, and bovine spongiform encephalopathy
0% 0%
(mad cow disease).
1 2 3 4 5

22. 30y F, 7 day history of mild tremor in her arms and impaired balance when
walking. Vital signs are normal. Her symptoms disappear the following week, but
recures several times over next 4 years gradually developed weakness requiring
assistance when walking. Neurologic examination reveals ataxia, dysarthria,
decreased vibratory sensation, increased deep tendon reflexes, and a +ve
Babinski sign on the left. Fifteen years after the onset of symptoms, the patient
becomes bedridden and dies. A coronal section of the patient's brain at autopsy is
stained for myelin (shown). Which of the following histopathologic findings would
be expected in the lesions seen in the patient's brain?
A. Astrogliosis
B. Lewy bodies
C. Myelin loss.
D. Neuritic plaques
E. Neuronophagia.
100%
0% 0% 0% 0%
A. B. C. D. E.

23. What is the best clinical description for the image?
1. 83y man – dementia, memory loss and disorientation.
2. 43y man -abnormal movements who committed suicide
3. 56y woman -progressive paralysis
4. 60y man -tremor, rigidity and dementia
5. 73y woman –Dementia aphasia but intact memory.
67%
17% 17%
0% 0%
1 2 3 4 5

24. What is the best clinical description for the image?
AD1. 83y man – dementia, memory loss and disorientation.
HD2. 43y man -abnormal movements who committed suicide
ALS
3. 56y woman -progressive paralysis
PD4. 60y man -tremor, rigidity and dementia
Pick
5. 73y woman –Dementia aphasia intact memory.
71%
14% 14%
0% 0%
1 2 3 4 5

25. CNS Degenerative disorders…
A 56 year old man develops 2 year history of increasing hand and forearm weakness
and clumsiness. Later develops dysarthria and dysphagia. A year later he dies of
pneumonia. Image shows two sections of spinal cord with arrows showing the
pathology. What is the most likely diagnosis? MND .. many subtypes:
• Amyotrophic Lateral Sclerosis.
A. Parkinsonism. • Progressive muscle atrophy (LMN)
B. Multiple Sclerosis. • Primary lateral sclerosis (UMN)
• Progressive bulbar palsy
C. Vitamin B12 deficiency (CDC)
D. Motor Neurone Disease (MND)
E. Amyotrophic Lateral Sclerosis (ALS)
0% 0% 0% 0% 0%
A. B. C. D. E.
A. Briefly describe pathological features seen in the image?
B. Explain the symptoms? What is the prognosis?
C. List one microscopic feature?

26. CNS Degenerative disorders…
CPC-3.6– KFP Questions:
 Dementia – Definition, Clinical, types.
 Primary:
 Alzheimer's, Pick's, Parkinson’s & Diffuse
Lewy Body Dementia, Huntington's.
 Secondary:
 CVA, Infections (meningitis), Neoplasms,
hydrocephalus, Drugs and toxins, metabolic,
vitamin def. (e.g. B1, B2, B12).
 Demyelinating disorders:
 Multiple sclerosis, MND,
26

27. The only real mistake is the one
from which we learn nothing!
JOHN POWELL: