Lecture 20 and 21 -- Peripheral Neuro D/o Flashcards
UMN symptoms
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LMN symptoms
what is the specific and classic LMN symptom?
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Upper Motor Neuron:
Weakness Spasticity (Stiff) Increased DTRs, Clonus Pathological Reflexes (up going babinksi)
Lower Motor Neuron
Weakness,
Muscle atrophy,
Fasciculation’s
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
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
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
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
• 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
Infectious – what types of viruses?
UMN sx?
LMN sx?
Sensory sx ?
polio, WNV
polio = descending flaccid paralysis
Only LMN
Causes of DRG (sensory) disease?
○ Paraneoplastic with anti-Hu antibody ○ Sjogren’s syndrome ○ Acute autoimmune sensory neuronopathy ○ Toxic • Cis platinum
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
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;
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
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,
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
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
Charcot Marie Tooth – Neuropathy
mutation to?
pathology?
reason for this pathology?
PMP22
Chronic and demyelinating condition
Leading to onion skinning
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)
CIPD
pathology?
reason for this pathology
A chronic form of GBS;
Immune mediated
chronic demyelinating
what two disease present with onion skinning pathology?
Charcot Marie Tooth
CIPD
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
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
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)
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
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
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
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
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
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
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
with what disease should you screen for PNPs?
Lambert Eaton
Dermatomyositis
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
