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Midterm 4 > Pato lab OLMAYAN info > Flashcards

Pato lab OLMAYAN info Flashcards

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1
Q

Match the structures with presentations

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

A

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

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2
Q

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 -

A

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

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3
Q

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) -

A

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

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4
Q

Primary degenerative/ dementia group diseases

A

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

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5
Q

Secondary degenerative/ dementia group diseases

A
  • Vascular pathologies, infarction
  • Toxic-metabolic disease
  • Wernicke-Korsakoff syndrome
  • Vitamin B12 deficiency
  • Hypothyroidism
  • Chronic liver disease
  • Drugs
  • Infection
  • Autoimmune-inflammatory diseases
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6
Q

Which neurodegenerative diseases have tau pt accumulation?

A

Alzheimer’s and Frontotemporal dementia

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7
Q

Which neurodegenerative diseases have TDP-43 accumulation?

A

Frontotemporal dementia and amyotrophic lateral sclerosis

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8
Q

Which neurodegenerative diseases have alpha synuclein accumulation?

A

Parkinson

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9
Q

Which neurodegenerative diseases have amyloid Beta accumulation?

A

Alzheimer’s

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10
Q

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

A

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

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11
Q

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

A

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)

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12
Q

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

A

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

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13
Q

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) –>

A

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

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14
Q

Key features of Parkinson’s disease

A

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

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15
Q

Key features of Idiopathic Parkinson’s disease

A

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

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16
Q

Key features of Huntington’s disease

A

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

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17
Q

Key features of Amyotrophic lateral sclerosis (ALS) disease

A

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

Ä°nfo: 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

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18
Q

What are the demyelinating diseases?

A

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

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19
Q

Key features of MS 1?

A

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

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20
Q

Key features of MS 2

A

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

21
Q

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

A

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

22
Q

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

A
  • 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!!
23
Q

Acute disseminated encephalomyelitis (ADEM)

A

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

24
Q

Key features of Acute Hemorrhagic Leukoencephalitis

A

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

25
Q

Key features of Progressive Multifocal Leukoencephalopathy

A

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

26
Q

Key features of Subacute Sclerosing Panencephalitis(SSPE)

A

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

27
Q

What are the three main degenerative events?

A

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

28
Q

What are the clinical manifestations of peripheral neuropathies?

A

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

29
Q

What are some types of neuropathies?

A

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

30
Q

What is neuropathy?

A

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

31
Q

What is Guillan Barre’s medical name?

A

Acute inflammatory demyelinating polyradiculoneuropathy

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

32
Q

Key features of CIPD (Chronic inflammatory demyelinating polyradiculoneuropathy)

A

Similar to GBS but no recovery

33
Q

Infectious neuropathy types

A

LEPROSY NEUROPATHY (cĂĽzzam = 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)

34
Q

Key features of peripheral neuropathy in DM

A

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
35
Q

What are PNS tumors?

A

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

36
Q

What are the key features of PNS tumors 1?

İçindekiler: Schwannoma AKA Neurilemoma or Neurinoma, neurofibroma, Neurofibromatosis/ NF (type1 2)

A

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

37
Q

hat are the key features of PNS tumors 2?

İçindekiler: Plexiform neurofibroma ve Malignant Schwannoma

A

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.

38
Q

What are skeletal muscle diseases’ general features?

A

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)

39
Q

Key features of Genetic muscular dystrophies

A

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

40
Q

What are muscular dystrophies?

A

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

41
Q

Key features of Duchenne m dystrophy?

A

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

42
Q

Key features of Becker m dystrophy?

A

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

43
Q

Key features of Emery-dreifuss muscular dystrophy?

A

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

44
Q

What are inflammatory myopathies that are also autoimmune disorders?

A

Polymyositis, dermatomyositis, inclusion body myositis.

45
Q

Key features of Dermatomyositis?

A

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

46
Q

Key features of polymyositis and inclusion body myositis

A

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

47
Q

Key features of Myasthenia gravis.

A

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.

48
Q

Devic vs MS

A

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

Midterm 4 (9 decks)

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