Pato lab OLMAYAN info

Question 1

Match the structures with presentations

Cerebral cortical neurons -
Basal ganglia -
Cerebellum -
Motor neurons -

Answer 1

Cerebral cortical neurons - memory loss, linguistic problems, cognitive functions
Basal ganglia - movement problems
Cerebellum - ataxia
Motor neurons - paresis, spasticity

Question 2

Match the neurodegenerative diseases with their key features + deposited proteins (Bunun tek tek soruları da olacak!) 1

Alzheimer’s disease -
Prion disease (AKA deli dana hastalığı)-
Parkinsons’s disease -

Answer 2

Alzheimer’s disease - Neuritic (senile) plaques, neurofibrillary tangles + amyloid precursor proteins (Amyloid beta, A-beta), tau pt

Prion disease - Spongiform degeneration, amyloid and other aggregates + Prion pt

Parkinsons’s disease - Lewy bodies, lewy neurites + A-synuclein

Question 3

Match the neurodegenerative diseases with their key features + deposited proteins (Bunun tek tek soruları da olacak!) 2

Huntington disease -
Amyotrophic lateral sclerosis -
Fronto temporal dementia (AKA Pick’s disease) -

Answer 3

Huntington disease - intracellular inclusions, cytoplasmic aggregates + huntingtin

Amyotrophic lateral sclerosis - axonal spheroids, bonina bodies + TDP-43

Fronto temporal dementia (AKA Pick’s disease) - Pick bodies + tau pt, TDP-43

Question 4

Primary degenerative/ dementia group diseases

Answer 4

Alzheimer’s, dementia with lewy bodies (parkinson), Frontotemporal dementia (AKA Pick’s disease), Motor impairments –> huntington + hallevorden spatz, prion group diseases

Question 5

Secondary degenerative/ dementia group diseases

Answer 5

• Vascular pathologies, infarction
• Toxic-metabolic disease
• Wernicke-Korsakoff syndrome
• Vitamin B12 deficiency
• Hypothyroidism
• Chronic liver disease
• Drugs
• Infection
• Autoimmune-inflammatory diseases

Question 6

Which neurodegenerative diseases have tau pt accumulation?

Answer 6

Alzheimer’s and Frontotemporal dementia

Question 7

Which neurodegenerative diseases have TDP-43 accumulation?

Answer 7

Frontotemporal dementia and amyotrophic lateral sclerosis

Question 8

Which neurodegenerative diseases have alpha synuclein accumulation?

Answer 8

Parkinson

Question 9

Which neurodegenerative diseases have amyloid Beta accumulation?

Answer 9

Alzheimer’s

Question 10

Which type of brain changes are seen in alzheimer’s?

Answer 10

Cortical symmetrical atrophy, mostly in frontal temporal and parietal lobes
Sulcus enlargement and neuron loss, compensatory ventricular enlargement, synaptic loss

Question 11

Which type of pathologic brain structures are seen in alzheimer’s?

Answer 11

Neurofibrillary tangles (in cortex), amyloid plaques due to amyloid angiopathy (Abeta2 deposition in vessels), Granulovacuolar degeneration (hippocampus + olfactory bulbus), Hirano bodies (eosinophilic + in pyramidal hippocampus)

Question 12

What are key features of Pick disease? (Frontotemporal dementia)

Answer 12

Walnut brain!!
Knife gyri!!
Asymmetrical cerebral atrophy in the frontal and temporal lobes
Atrophy in gray matter
Pick cells/ bodies

Question 13

Classify the degenerative diseases according to their anatomic localizations.

Alzheimer’s + Pick disease (frontotemporal dementia) –>
Parkinson + Huntington + chorea –>
(Ataxia disorders are also present but they are obvious as they have ataxia in their names)
Amyotrophic lateral sclerosis (ALS) –>

Answer 13

Alzheimer’s + Pick disease (frontotemporal dementia) –> Cerebral cortex

Parkinson + Huntington + chorea –> Basal ganglion n brain stem
Parkinson –> Pyramidal, Huntington–> extrapyramidal

Amyotrophic lateral sclerosis (ALS) –> Motor neurons

Question 14

Key features of Parkinson’s disease

Answer 14

İn pyramidal system
Stooped posture, bradykinesia, rigidity, Motor dysfunction characterized by involuntary tremors (such as counting money or rolling pills)

Question 15

Key features of Idiopathic Parkinson’s disease

Answer 15

Old ppl
Little mental function change as well as motor impairment
Dementia in some
Pigment loss in substantia nigra and locus cereleus grossly (pale- lightness)
Pigment bodies and Lewy bodies

Question 16

Key features of Huntington’s disease

Answer 16

Chromosome 4, autosomal dominant, death in 15 yrs
Characterized by uncontrolled extrapyramidal or choreiform
movements and progressive dementia
whole-body movements called chorea
Progresses to parkinsonism over tim
Enlargement of the frontal horns of lateral ventricles and 3rd ventricle
Brain is grossly small, atrophy in putamen and caudate nucleus
Frontal and parietal love atrophy

Question 17

Key features of Amyotrophic lateral sclerosis (ALS) disease

Answer 17

Bonina bodies, Autophagic vacuoles
Asymmetrical weakness and respiratory failure
Denervation atrophy in skeletal muscles
More common in men

İnfo: medulla spinalis is characterized by muscular atrophy and hyperreflexia as a result of the degeneration of the lower motor neurons in the anterior horn and then the upper motor neurons in the corticospinal tract

Question 18

What are the demyelinating diseases?

Answer 18

1- Multiple Sclerosis (MS)
2- Devic’s disease (Neuromyelitis optica)
3- Acute disseminated encephalomyelitis (ADEM)
4- Acute necrotizing hemorrhagic encephalomyelitis

Question 19

Key features of MS 1?

Answer 19

One of the most common demyelination diseases
more common in women
Type IV hypersensitivity and inflammatory
HLA DR2
Only in CNS, therefore only in oligodendrocytes not in Schwann cells
From the head pairs it only holds the 1st and 2nd head pairs,
an extension of the CNS !!!
paresthesias, lhermitte sign

Question 20

Key features of MS 2

Answer 20

In MS, the protein level in the CSF is slightly increased and
lymphocytes is also increased but in a small number
* Increased ratio of gamma globulin in cerebrospinal fluid
* Myelin protein can be detected in the CSF during active
demyelination process
* Plaques corresponding to demyelinated areas in serial sections
of the macroscopically normal brain
* High percentage of interferon gamma originating from myelinsensitized CD4 + T lymphocytes, CD8 + T lymphocytes and
activated macrophages in the lesions
* Fresh lesions are considered sclerosis because they are harder
than the surrounding brain parenchyma
Zımba deliği görüntüsü

Question 21

Key features of Neuromyelitis optica (Devic’s disease) 1

Answer 21

Characterized by necrotizing myelinopathy involving the optic nerve and myelitis with longitudinal spreading (involving more than 3 vertebral segments)

Necrotic lesions in the spinal cord
* Necrotic or demyelinated lesions in the optic nerve and chiasm
* Lesions do not spread to other parts
* Lesions continue in many segments of the spinal cord
* Little or no inflammatory cell infiltration

Question 22

Key features of Neuromyelitis optica (Devic’s disease) 2

Answer 22

• Cranial MR is different from MS: It is always considered normal
• Neutrophils are found in the CSF in rapidly progressive samples
• Visual impairment or loss, sensory or motor loss, bladder-bowel
  control problems are clinical findings
• Rapidly progressing clinical course
• Protein level increased
• There is no oligoclonal band
  Aquaporin 4!!

Question 23

Acute disseminated encephalomyelitis (ADEM)

Answer 23

Autoimmune inflammatory, attack against white matter
Multiple point demyelination foci centered on small
veins and venules
Most cases are children
Especially in the centrum semiovale, cerebellum, brain stem
and spinal cord
Infection trigger!
perivascular demyelination and mononuclear inflammatory cell infiltration
Early–> neutrophil Late–> mononuclear
The duration of the disease is short
It is rarely fatal; most patients recover

Question 24

Key features of Acute Hemorrhagic Leukoencephalitis

Answer 24

A demyelination disease showing the perivascular distribution of ADEM
* Hemorrhagic necrosis in more fulminant and gray-white matter
* Patients are usually young adults
* After upper respiratory tract infection
* Veins necrotic, thrombus in their lumen
* It is a hyperacute reaction, sometimes killing within 48 hours

Question 25

Key features of Progressive Multifocal Leukoencephalopathy

Answer 25

Viral infection caused by polioma viruses retention of oligodendrocytes * Demyelination as the virus kills oligodendrocytes

Question 26

Key features of Subacute Sclerosing Panencephalitis(SSPE)

Answer 26

seen in babies It is a slowly progressive CNS infection that occurs years after measles infection * There is gray or white matter involvement

Question 27

What are the three main degenerative events?

Answer 27

Wallerian degeneration, axonal degeneration (most common), segmental demyelination

Question 28

What are the clinical manifestations of peripheral neuropathies?

Answer 28

Sensorial, motor or autonomic. Additional info 1: Especially in axonal diseases, slowly progressing symmetrical loss of sensation often spreads in the form of gloves and socks Additional info 2: If there is an motor deficit, it manifests itself with weakness + Deep tendon reflexes are reduced or absent Add info 3: Findings such as postural hypotension and constipation in the autonomic system Add info 4: There may be asymmetric neurological deficits. Since it may cause randomly distributed multiple nerve involvement, especially in neuropathies caused by vasculitis

Question 29

What are some types of neuropathies?

Answer 29

IFLAMMATORY NEUROPATHIES: Neuropathies with immune transition (Acute inflammatory demyelinating polyradiculoneuropathy aka Guillain-Barré syndrome + Chronic inflammatory demyelinating polyradiculoneuropathy) Infectious polyneurropathies, Hereditary neuropathies, Acquired and toxic neuropathies, Traumatic neuropathies

Question 30

What is neuropathy?

Answer 30

Beyin ve omurilik dışında yer alan sinirlerin (periferik sinirler) hasar alması, fonksiyon kaybı gibi durumlarda ortaya çıkan tıbbi durumdur.

Question 31

What is Guillan Barre's medical name?

Answer 31

Acute inflammatory demyelinating polyradiculoneuropathy Info: It is characterized by Inflammation and demyelination of peripheral nerves and spinal nerve roots (radiculoneuropathy)

Question 32

Key features of CIPD (Chronic inflammatory demyelinating polyradiculoneuropathy)

Answer 32

Similar to GBS but no recovery

Question 33

Infectious neuropathy types

Answer 33

LEPROSY NEUROPATHY (cüzzam = leprosy), DIPHTERIC NEUROPATHY (paresthesia and weakness, sensory ganglia is affected), VARICELLA-ZOSTER NEUROPATHY (zona hastalığı) (Latent infection following chickenpox, neurons in the sensory ganglia of the spinal cord and brainstem)

Question 34

Key features of peripheral neuropathy in DM

Answer 34

Most common acquired neuropathy. Distal symmetrical sensorimotor neuropathy Dominant pathological sign axonal neuropathy * Endoneural arteriolar thickening * Hyalinization * PAS + material accumulation in the wall * BM reduplication (amputation!) * Pain loss → ulcer (feet mostly) * Diffuse microvascular injury → healing disorder * Autonomic neuropathy * Focal or multifocal asymmetric neuropathy

Question 35

What are PNS tumors?

Answer 35

Schwannoma AKA Neurilemoma or Neurinoma, neurofibroma, Neurofibromatosis/ NF (type1 2), Plexiform neurofibroma, Malignant Schwannoma

Question 36

What are the key features of PNS tumors 1? İçindekiler: Schwannoma AKA Neurilemoma or Neurinoma, neurofibroma, Neurofibromatosis/ NF (type1 2)

Answer 36

Schwannoma--> Antoni A (verocay body!!) and Antoni B areas. Kapsül. large vascular structures, spaces and bleeding areas Neurofibroma--> schwannomada kapsül var bunda yok Neurofibromatosis/ NF --> multiple neurofibroma NF type 1: von Recklinghausen’s disease--> most common type, Multiple café-au-lait spots and dermal neurofibromas, on ch 17 NF type 2: Bilateral acoustic neuromas and other brain and spinal cord tumors, ch 22

Question 37

hat are the key features of PNS tumors 2? İçindekiler: Plexiform neurofibroma ve Malignant Schwannoma

Answer 37

Plexiform neurofibroma--> Loose myxoid ground, low cellularity, schwann cells, fibroblasts, mast cells and inflammatory cells are observed Malignant Schwannoma--> similar to NF1 Triton tumor!! Gross: poorly circumscribed, recurrent, metastasized necrosis and hemorrhagic tumors Microscopically: high mitotic activity pleomorphism, spindle-shaped cells rich in pronounced cytological atypia are observed If heterologous elements are found (eg rhabdomyosarcoma) it is called a "Triton" tumor.

Question 38

What are skeletal muscle diseases' general features?

Answer 38

Decreased muscle strength, decreased muscle mass, decrease or loss in DTRs examined in two groups as neurogenic and myogenic Classified as genetic (Muscular Dystrophies, Congenital Structural Myopathies, Metabolic Myopathies, Mitochondrial Myopathies, Muscle Ion Canal Diseases) and acquired (Inflammatory Myopathy, Endocrine Myopathy,Toxic Myopathy, Myopathy with Systemic Diseases)

Question 39

Key features of Genetic muscular dystrophies

Answer 39

X-linked recessive, autosomal recessive and dominant muscular dystrophies are present. (Pato için Xlinked recessive önemli.) X-linked recessive--> Duchenne, Becker, Emery-Dreifuss (joint contractures and cardiac involvement

Question 40

What are muscular dystrophies?

Answer 40

Muscular dystrophies are inherited myopathies with progressive muscle weakness and muscle fiber degeneration

Question 41

Key features of Duchenne m dystrophy?

Answer 41

absence of the dystrophin gene (NOT deficiency as in Becker) impaired walking, duck-like gait, frequent falls, and lag in movement. seen in Children. Pelvic and pectoral m's first. Gower's sign!! Cardiac muscle is also involved, There is often intellectual problems, Patients die in the second or third decade due to cardiac failure or intercurrent infections X-linked recessive

Question 42

Key features of Becker m dystrophy?

Answer 42

dystrophin deficiency (NOT absence as in Duchenne) Milder Duchenne gibi bir şey. X-linked recessive

Question 43

Key features of Emery-dreifuss muscular dystrophy?

Answer 43

Premature contractures in the elbows, achilles tendon and spina (comes out before weakness), Prominently distal muscle weakness in the humeroperoneal region (sometimes slow muscle involvement in facial muscles), Dangerous cardiac conduction defects and cardiomyopathy X-linked recessive

Question 44

What are inflammatory myopathies that are also autoimmune disorders?

Answer 44

Polymyositis, dermatomyositis, inclusion body myositis.

Question 45

Key features of Dermatomyositis?

Answer 45

Women, Loss of strength in proximal muscles, dysphagia, can be paraneoplastic, Heliotropic erythema (mauve) of the upper eyelid, Gottron papules  Pathogenesis: autoantibodies ( Jo-1 antibody) against the endothelium of small arterioles  Perifascicular atrophy is a sufficient finding

Question 46

Key features of polymyositis and inclusion body myositis

Answer 46

polymyositis --> Only muscles NO skin. Adults. NO internal organ malignancies inclusion body myositis--> Small muscles, Asymmetrical involvement, Clear vacuoles within the sarcolemma, Amyloid in inclusions

Question 47

Key features of Myasthenia gravis.

Answer 47

Autoimmune and also inflammatory. Neuromuscular conduction disease, Antibody to acetylcholine receptors, 15-20% thymoma accompanies Muscle weakness that becomes evident in the following hours=> ptosis. Death=> respiratory involvement Ptosis + diplopia.

Question 48

Devic vs MS

Answer 48

Ms—> mr anormal. Oligoclonal band var. Gammaglobulin, pt and lympgocytes in CSF. Devic—> normal. Oc band yok. Necrotizing. Neutrophils in csf. Aquaporin 4 involvement