Lecture 20 and 21 -- Peripheral Neuro D/o Flashcards Preview

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Flashcards in Lecture 20 and 21 -- Peripheral Neuro D/o Deck (30):
1

UMN symptoms

LMN symptoms
(what is the specific and classic LMN symptom?)

Upper Motor Neuron:

Weakness
Spasticity (Stiff)
Increased DTRs, Clonus
Pathological Reflexes
(up going babinksi)



Lower Motor Neuron
Weakness,
Muscle atrophy,
Fasciculation's

2

sensory neuropathy's --- large fiber vs small fiber symptoms --

where do the tracts run?

large Fibers; Dorsal Column
Light touch
Vibration
Joint position sense

Small Fibers/Lateral Spinothalamic tracts
Pinprick Temperature

3

Idiopathic Motor Neuron disorder?

2 Hereditary hereditary motor neuron disorders ?

2 infectious motor neuron d/o?

ALS

Hereditary -- Spinal muscular trophy
Kennedy disease

Infections - Poliomyelitis, WNV

4

ALS --
describe the symptomatology? what are some early symptoms? classic findings?

upper motor symptoms?
Lower motor symptoms?
sensory symptoms ?

UMN - Yes -- Hyperreflexia, spasticity, weakness
LMN - yes -- muscle atrophy, weakness, fasiculations
Sensory deficits -- No
Cognitive/behavior -- yes (disinhibition)


® Early -- Muscle cramps and fasiculations
® Limb weakness -- asymmetric; moves to opposite side before progressing distally
◊ Muscle atrophy and spasm
® Bulbar-- slurred speech and dysphagia of liquid/solids
® Tongue Atrophy

5

Spinal Muscle Atrophy -- classic finding in kids?
genetic inheritence pattern?
UMN sx?
LMN sx?
Sensory sx ?

Floppy baby
AR
UMN - no
LMN -yes
Sensory - no

6

• Kennedy disease -- (aka Spinal-bulbar muscular atrophy)
Genetic Inheritence?
genetic mutation? -- what other manifestations arise from this ?

pathognomonic combination of findings?
UMN sx?
LMN sx?
Sensory sx ?

LMN findings only
Key finding: Fasciculation's of the tongue in a floppy baby

XLR -- mutation of the Androgen receptor
therefore children with androgen insensitivity

7

Infectious -- what types of viruses?

UMN sx?
LMN sx?
Sensory sx ?

polio, WNV

polio = descending flaccid paralysis

Only LMN

8

Causes of DRG (sensory) disease?

○ Paraneoplastic with anti-Hu antibody
○ Sjogren’s syndrome
○ Acute autoimmune sensory neuronopathy
○ Toxic
• Cis platinum

9

radiculopathy -- where is the damage?

Symptoms and findings?
what is the most common cause?
what is the most common location?

-- lesion at the dorsal nerve root which is a union of a sensory dorsal root and motor ventral root

Symptoms: Pain, tingling, numbness, weakness
Findings -- sensory nerve conduction testing is totally normal bc the DRG is intact

cause -- Trauma, disk hernia, infection

Most common Cervical - C 7
Mostly Lumbosacral -- L5, S1
Lumbsacral > Cervical

10

Disorders of the plexus --

what are the plexi?
symptoms?
common causes?

(lumbosarcal plexus and branchial plexus)

Common cause: Trauma to the plexus and trauma during childbirth
Other -- neoplasma, trauma, hematoma, abscess, radiation, immune

Sensory and Motor Signs -- bc this is a Mixed Root;

11

D/o of peripheral Nerves -- (neuropathy)

what is the most common cause?
What is the classical distribution pattern?

how can it present? (symptoms)

DM
Glove and Stocking distribution


Sensory signs
Motor Signs
Autonomic Signs

12

Examples of sensory signs in neuropathy? (differences between large myelinated vs small myelinated/unmyelinated?)

Example of motor signs?

Examples of autonomic signs?

Sensory -- numbness, paresthesias, dyesthesia, allodynia, hyperpathia

Large Myelinated -- Joint position and Vibration
Small Myelinated/Unmyelinated -- temperature, hyperalgesia

Motor - weakness, fasciulations, temors

Autonomic -- anhydrosis, orthrostatic, GU, GI, sexual,

13

name some causes of peripheral neurpathy?

• Mononeuropathy -- Carpal Tunnel
• Systemic D/o -- DM, B12 def, Infection (HIV, Hepatitis)
• Genetic -- Charot Marie Tooth

Demyelinated --GBS, CIPD

14

carpal tunnel --

distribution of disturbance?
positive signs?
associated conditions?

Sensory disturbance to: First three digits and part of the fourth
Wekaness and atrophy of the thenar muscles
Positive Phalen's and Tinel's Test

Associated conditions --- pregnancy, occupation, RA, DM, HTN

15

Charcot Marie Tooth -- Neuropathy
mutation to?
pathology?
reason for this pathology?

PMP22
Chronic and demyelinating condition
Leading to onion skinning

16

Guillaine Barre Syndrome
aka?
mediated by what cell type?
Preceding condition?
type of paralysis

T cell mediated auto immune attack on myeline

aka -- acute inflammatory demyelinating polyradiculoneuropathy (acute in presentation)

Ascending paralysis

Preceded by infection (campylobacter, CMV, EBV)

17

CIPD
pathology?
reason for this pathology

A chronic form of GBS;

Immune mediated

chronic demyelinating

18

what two disease present with onion skinning pathology?

Charcot Marie Tooth

CIPD

19

what form of neuropathy does not follow the glove/stocking distribution?

describe the pathology

Vasiculitis Neuropathy --
random patterns of neuropathy

bx is taken looking for vasculitis, not necessarily the nerve

20

Disorders of the presynpatic NMJ?
describe the pathology

Lambert Eaton -- autoimmune attack of P/Q VG Calcium Channels -


Botulism -- Clostridium Botulinum spores; prevents vesicles from binding to presynaptic membrane

21

Lambert Eaton --

improves with what?
What should you always check for if this condition presents?

Improves with repetitive motions; flooding the cell with calcium to improve release of vesicles


Associated with Small Cell Lung Cancer (paraneoplastic syndrome)

22

post synaptic d/o at the NMJ?
pathology?
how does it present?

Myasthenia Gravis --
autoimmune d/o at the ACHR-nicotinic

presents with flucutating strength and weakness
Unilateral ptosis, but can affect any muscle


Proximal disease > distal

23

what bacteria is classically associated with demyelination?

what other bacteria and viruses are associated with axonal destruction and demyelination

Diphteria toxin


Leprosy (former most common cause of neuropathy)

Varicella Zoster

24

What are the 5 forms of myopathy? --- name specific of each

what serum value is typically elveated with muscle damage?

Creatinine (Phospho) Kinase -- elevated

Congential -- (Nemeline, central core disease)

Metabolic -- (Mitochondrial disease, Glycogen storage)

Inflammatory (Polymyositis, Dermatomyositis, Inclusion body myositis)

Dystrophies -- (Duchene's, Becker's, Myotonic)

Toxic --Statins, EtOH

25

Congential Myopathes --
Nemeline,
what does histology look like?


central core disease
Pathophysiology;
associated with what deadly process?
pathology?

Nemaline Myopathy -- rods = expansion of Z disk material;


Central core disease -- absence of staining in the center
Ryanodine Receptor gene mutation
Patients are susceptible to Malignant Hyperthermia

26

Metabolic --


Mitochondrial disease -- classic histology?


Glycogen storage --- name a disease; what is the classic histo

Mitochondrial Myopthathy --
Ragged Red Fibers
Parking space inclusions on EM


Glycogen Storage diseases --- Pompe's Disease
Accumulation of Glycogen

27

Inflammatory

mediated by what cell type?
Classic histology of each of the following?

Polymyositis,


Inclusion body myositis

Cytotoxic T cells for Both (CD8)

Polymyositis -- lymphocytes invading non necrotic fibers

Inclusion body myositis
light microscopy -- rimmed vacuoles

28

Dermatomyositis
mediated by what cells?
pathognomonic findings?
Histology?


what should you also screen for?

CD4 Mediated
Rashes: Heliotrope rash, V sign, Gottrons Sign
perifascicular atrophy

also screen for PNPs

29

with what disease should you screen for PNPs?

Lambert Eaton

Dermatomyositis

30

Dystrophies --

Duchene's vs Becker's,

how can you differentiate the two (what lab studies)?

pathology

classic distribution for dystrophic myopathies?

Duchene's -- complete loss of dystophin -- more severe phenotype

Beckers -- partial loss -- more mild phenotype

Consistent with IHC and Western Blot Findings
pathology -- endomysial fibrosis

Limb Girdle Syndromes