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periphera l neuropathy Flashcards

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1
Q

ANTERIOR HORN CELL DISEASES

A

PRIMARY disease of motor neurons ; degenerative as ; MND AND SMA
infection; poliomyltitis
spinal cord pathology [ ahc + other pat of cord]
vascular ; anterio spinal artery ischemia
mechanical ; syringomyleia

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2
Q

what is motor neuron disease

A

it is mixed upper and lower motor neuron affection

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3
Q

MND TYPES

A

most common type ; als which affect umn and lmn
primary musclar atrophy ; pure lower motor
primary lateral sclerosis ; pure umn

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4
Q

what is the most common type of MND

A

ALS ; MIXED UMN AND LMN

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5
Q

TREATMENT OF MND

A

SUPPORTIVE AND RILUZOLE

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6
Q

HOW TO DIAGNOSE MND

A

CLINICAL DIAGNOSIS , NCS . EMG

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7
Q

DD OF DISEASE THAT MAKE UMN AND LMN SIGN

A

FRIEDRCHS ATAXIA
MND
CERVICAL SPONDYOLYSIS
CONUS MEDULLARIS
SUBACUTE COMBINED DEGENRATION OF THE CORD [ VIT 12 DEFECIENCY]
TABOPARESIS [ SYPHYLIS]

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8
Q

RADICULOPATHY causes

A

mechanical :
degenerative as spondyolysis (pars intercularis fractuer which is connect 2 joints together )
mass lesion :bone metastsis
trauma
medical :
inflammatory as Gbs
neoplastic i filtrate
post radiotherapy

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9
Q

degenerative spine diseases types

A

compress on the roots :Lmnl
compress on the centeal canal :Umnl
cimpress on both : mixed Umnl and Lmnl

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10
Q

Signs & Symptoms of
Radiculopathy

A

Dermatomal/myotomal distribution
* PAIN
– Locally in neck / back
– Referred to dermatome
* Numbness, tingling – dermatomal.
* Weakness (myotome distribution)
– But sometimes purely sensory.
* Reduced reflexes depending on affected root

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11
Q

CAUDA EQUINA SYNDROME

A

LUMBOSACRAL AFFECTION

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12
Q

CLINICAL PRESENTATION OF CAUDA EQUINA SYNDROME

A

Sacral roots saddle anaesthesia, urinary retention (flaccid bladder, volume bladder),
impotence,
– Often absent reflexes in legs (lumbar roots

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13
Q

SADDLE ANATHESIS

A

“Saddle anesthesia”—numbness and loss of sensation in the buttocks, perineum, and inner surfaces of the thighs

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14
Q

(MYELOPATHY gives a sensory LEVEL ie not dermatomal, spastic bladder (small
volumes, empties without warning) , UMN signs below the level of the lesion)

A
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15
Q

INVESTIGATION of radiculopathy

A

imaging;
CT – good for bones. Can’t see nerve tissue.
– MRI – bones + signal change in neural elements
Nerve conduction & EMG
– Confirm denervation in a root distribution
– Normal sensory action potentials despite
numbness

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16
Q

INVESTIGATION of radiculopathy

A

imaging;
CT – good for bones. Can’t see nerve tissue.
– MRI – bones + signal change in neural elements
Nerve conduction & EMG
– Confirm denervation in a root distribution
– Normal sensory action potentials despite
numbness

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17
Q

management of radiculopathy

A

Pain control
* Spondylosis
– Orthopaedic or neurosurgical opinion
– Surgery to prevent worsening (may not make
weakness better once muscle has wasted).
* Medical causes
– Treat underlying cause

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18
Q

neuropathy causes

A

Metabolic (DM, B12 defic., etc)
– Toxic (alcohol, drugs – e.g.
chemotherapy)
– Infection eg. HIV, Lyme, Syphillis
– Compression
– Autoimmune
– Degenerative (idiopathic axonal
neuropathy)
– Hereditary (CMT

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19
Q

Hoarse voice, wasted hand, weight loss, smoker
what is the disease

A

pancoast tumor

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20
Q

Funny rash, tickbite, arthralgia, heartblock, cranial nerve palsy and radicular pains
what is the disease ?

A

lyme disease

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21
Q

what is the disease ; crohns affecting terminal ileum and polyneuropathy

A

b 12 defeciency

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22
Q

what type of neuropathy ?

A
  • What is the DISTRIBUTION? (individual nerve(s) / all the longest nerves)
  • Acute or chronic? relapsing and remitting? Slowly or rapidly progressing?
  • Axonal / demyelinating (ask for nerve conduction study)
  • Motor fibres / sensory fibres / both
  • Large fibres / small fibres / both (burning and pain = small fibre)
    – Large fibres – check proprioception, light touch, vibration
    – Small fibres – check pain (pin), temperature (cold tuning fork)
  • Autonomic involvement?
    – Postural hypotension, sweating too much/little, impotence, gastrointestinal
    symptoms, pupillary abnormalities
  • Cranial nerve involvement?
  • Family history? – any chance that parents are related?
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23
Q

long nerve affection ? [many nerve affection]

A
  • Polyneuropathy
  • Longest nerve endings die back
  • Feet then legs then hands
  • Glove & stocking
  • Symmetrical
  • Long list of causes
    – B12, Folate
    – Diabetes & thyroid dysfunction
    – Drugs & toxins
    – Alcohol
    – Infections eg HIV/AIDS
    – Critical illness neuropathy
    – Connective tissue diseases egRA, SLE,
    ChurgStrauss
    – Carcinomatous neuropathy
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24
Q

individual nerve affection?

A
  • Single nerve (mononeuropathy)
    – Compression:
  • Median at the carpal tunnel
  • Ulnar at the elbow
  • Common Peroneal at the fibular head
  • Radial in spiral groove of humerus
    – Idiopathic eg Bell’s palsy
    – Inflammatory
    – Infiltration by tumour
  • Several nerves (monon. mul)
  • Patchy, asymmetrical
    – VASCULITIS (painful)
    – INFLAMMATORY
    – DIABETES (causes a vasculitis)
  • Hereditary neuropathy with liability to
    Pressure Palsies (rare)
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25
causes of mylein sheath degeneration
* Hereditary(rare) * – CMT type 1 * Inflammatory/autoimmune – GuillainBarre Syndrome – Chronic inflammatory demyelinating polyneuropathy (CIDP) – Multifocal motor neuropathy – Paraproteinaemic neuropathy
26
motor affection only / what the disease ?
Multifocal motor neuropathy (MMN) – ALWAYS PURELY MOTOR – Treatable but can be mistaken for MND. * GuillainBarre Syndrome * CIDP * CharcotMarieTooth – Can be mainly motor * Rarities: – Lead, Arsenic, Thallium – Porphyria – Diptheria
27
mainly sensory nerve affection disease ?
Causes of pure sensory neuropathy – Paraneoplastic – Drugs – Carcinomatous sensory neuronopathy – Lymphomatous sensory neuronopathy – Sjögren's syndrome – Paraproteinemias – Nonsystemic vasculitic neuropathy – Primary biliary cirrhosis – Crohn's disease – Chronic gluten enteropathy – Hereditary sensory neuropathy
28
lenght dependent sensory neuropathy
most common pattern typically axon most commnly in diabetic , alcholo both are painful or idiopathic treat underlyine cause
29
medication of lenght dependnt sensory neuropathy
for pain as gabentin , amitryptilline and pregablain
30
small fiber neuropathy ?
* Painful, burning * Nerve conduction normal unless coexisting large fibre neuropathy * Measure temperature perception (thermal threshold) * Diabetic neuropathy Alcoholic neuropathy Amyloidosis HIV/AIDS * Leprosy * Hereditary
31
diseases that involve autonomic
* Diabetic neuropathy Alcoholic neuropathy HIV/AIDS * GuillainBarré syndrome Amyloidosis Paraneoplastic neuropathy Lymphoma * Thiamine deficiency * Porphyria * Thallium, arsenic, mercury toxicit
32
cidp
Chronic version of GBS (>8 weeks form onset to peak; can be insidious) * Often relapsing-remitting course * Presumed autoimmune but no identified cause * Responds to treatment (steroids, other oral immunosuppressants, IVIg, plasma excahnge) * Patients my need prolonged / cycles of treatm
33
charcot marie tooth 1
Pes cavus, inverted champagne bottle legs – Autosomal dominant demyelinating neuropathy due to duplication of PMP22 gene (myelin constituent) – Insidious onset – Progressive distal weakness often without subjective sensory loss (but absent sensory responses on NCS) – Very slow nerve conduction – DNA testing allows genetic counselling and selective IVF (only implant embryos without mutation)
34
investigations of neuropathy
Look for treatable cause: – FBC, ESR, B12, folate – Glucose (fasting, OGTT), U&E, LFT, TSH, protein electrophoresis * Characterise / confirm neuropathy with EMG/NCS – Motor vs sensory (may be subclinical) – Demyelinating vs axonal * Background / further investigations – LP (?raised protein) – nerve biopsy (inflammation?) – bone marrow (?paraprotein in myeloma) – genetic
35
Guillain Barre Syndrome
Rare but a medical emergency * Idiosyncratic acute demyelinating neuropathy (<4 weeks from onset to peak) * Autoimmune, often prompted by infection (Campylobacter, viral, rare postvaccination) * Sensory symptoms (back ache, tigling extremities) followed by fatigue and ascending weakness with areflexia * Can look normal in early stages – get sent home from A&E * – Admit
36
gbs treatment
IVIG
37
Acute neuropathy differential diagnosis
GBS *Hepatic Porphyria * Toxins * Vasculitis * Critical illness neuropathy *Infectious radiculopathy e.g. Lyme *Diphtheri
38
investigaions for GBS
* Bloods, urine * CSF * Electrics *Imaging * Biopsy
39
WHAT WILL FIND IN CSF OF GBS
RAISED protein and normal cell and glucose called albuminocytological dissocaition
40
examples fro mononeuropathy
carpel tunnel syndrome ulnar neuropathy wrist drop foot drop
41
Clinical features of vasculitic neuropathy
Distribution * asymmetric, multifocal * Sensory-motor or sensory Pace * Rapid onset ,progressive or intermittent course Pain * painful (parathesias) Systemic features
42
investiagtion of vascuilitc neuropathy
serology for autoimmune disorder EMG nd NVC NERVE BIOPSY
43
CAUSES of NMJ disorders
autoimmune ; MG paraneoplastic ; LAMBERT EATON MYATHENSIA SYNDROME INFECTION; BOUTLISUM HERDETIERY - VARIOUS SYMPTOMS snake venom
44
key of NMJ
FATIIGUBALITY
45
MG PATHOGENIS
ANTIBODIES AGANINST NICOTINC ACETYLCHOLINE RECEPTOR [ ACHR]
46
MG
Fatigable weakness – Symptoms worse in evening – Muscles weaken with exercise * proximal>distal * Ptosis, complex opthalmoplegia, dysphagia, neck flexion weakness * Sensory: normal * Reflexes: normal * Neurophysiology Tests – Repetitive stimulation – Single fibre EMG * Tensilon (Edrophonium) Test – Risk of bradycardia – Sometimes difficult to interpret – Double blind if possible
47
management of MG
pyridostigmine [increase ach level in synaptic cleft by inhibitio acetcloinestrase]
48
investigation MG
CT CHEST TO LLOK FOR THYMOMA SO THIS MAY BE THE CAUSE AND PATIENT NEED THMECTOMY
49
CAUSES OF MYOPATHY
herdietry and acquired
50
herdietry causes of myopathy
Muscular Dystrophies * Congenital Myopathies * Mitochondrial * Enzyme deficiencies * Channelopathy * Others eg. –Myofibrillar myopathies –Scapuloperoneal syndromes –Hereditary Inclusion body myopathies
51
myopathy acquired causes
Inflammatory * Infection eg. HIV * Endocrine/metabolic * Drugs/toxins * Malignancy
52
clinical presentation of myopathy
Weakness: usually proximal – Stairs, getting out of bath – High shelves, washing hair * Weakness > wasting * Sensory: normal * Reflexes: normal (except severely wasted muscle) * EMG: myopathic picture * CK: elevated
53
muscular dystrophies
Various disorders – Duchenne – Becker – Limb girdle – Myotonic – Fascioscapulohumeral – Emery-Dreifuss – Occulopharyngeal – Congenital – Distal * Progressive muscle weakness and wasting * Necrosis of muscle cells * Inherited disorders * Some are multi-system, not just muscle * Disorders often of structural proteins
54
clinical picture of duchenne muscular dystrophy
Commonest dystrophy (1 in 3500 boys) * X-linked * Absence of DYSTROPHIN from muscle – links actinto extracellular matrix * Onset as toddler, Wheelchair by early teens, Death i (respiratory failure) * Cardiomyopathy * Calf pseudohypertrophy * Joint contractures, scoliosis * Mild intellectual impairment
55
clinical picture of duchenne muscular dystrophy
Commonest dystrophy (1 in 3500 boys) * X-linked * Absence of DYSTROPHIN from muscle – links actinto extracellular matrix * Onset as toddler, Wheelchair by early teens, Death i (respiratory failure) * Cardiomyopathy * Calf pseudohypertrophy * Joint contractures, scoliosis * Mild intellectual impairment
56
investigations for duchenne muscular dystrophy
ck very high up to 10000 diagnosis now days by dna echo for heart found cardiomyopathy [ start ACEI and carvideroll]
57
treat dmd
steroid prolong ambulation by b2 -3 years but don t prolong life supportive ; physiotherapy , splint , surgery for contractuer genetic counselling and testing for relatives
58
becker muscular dystrophy
Also a ‘dystrophinopathy’ * Becker mutations cause abnormal, short protein that functions partially – Duchenne mutations lead to complete absence * Onset usually in first decade, but occasionally as late as 30s * May not have to use wheelchair until 20s or later * May have normal lifespan * Cardiomyopathy can be more disabling than weakness – Heart transplant sometimes
59
myotonic dystrophy ?
MOST COMMON ADULT ONSET OF MUSCULAR DYTROPHY
60
clinical presentation of myotonic dystrophy
at age 30 yr weak hands [finger flexors ]and grip myotonia progressive proximally weak face and neck flexors
61
types of myotonic dystrophy
type 1 ; CTG REPETAS DMPK GENE , CHR 19 TYPE 2 ; PROXIMIAL MYOTONIC MYOPATHY [ PROMM]. CCTG REPEATS ANF9 GENE , CHR 3
62
TYPE 1 OF MYTONIC DYSTROPHY
ANTICIPATION OF CTG
63
CLINICAL PRESENTATION FO PROMM IS MILDER
PROXIMAL WEAKNESS NO FACE WEAKNESS NO STRIKING WASTING
64
MYTONIC DYSTOPHY ; WHICH MUTLISYSTEM AFFECT
– Muscle - dystrophy, myotonia – Heart - arrhythmias – Eyes - cataracts – CNS - hypersomnia, mental retardation – Endocrine - hypogonadism, insulin resistance
65
WHICH TYPE OF MYOTONIC DYSTROPHY IS COMMON
TYPR 1 IS THE MOST COMMN ; CTG RPEATS ON CHR 19
66
WHAT IS DM 1 [ TYPE 1 OF MYOTONIC DYSTROPHY]
* Expansion of CTG repeat in DMPK gene * Transcribed but not translated region * 50-4000 repeats pathologic * Repeat size correlates with onset and severity * Anticipation * Repeats increase most with maternal transmission
67
what is facioscapulohummeral dystrophy ?
chromsome 4q35 dominat inhertance at age 20 yr
68
clinical presentation of FSHD?
Weakness – Facial (orbicularis oris) – Shoulder girdle (scapular winging, sparing of deltoid), often asymmetric – Abdominal/truncal – Ankle dorsiflexion
69
FSHD SEVERITY OF THE DISEASE
CORRELATE WITH SIZE OF DELETION , TYPICALLY IN TEENS SLOWLY PROGRESSIVE ,NORMAL LIFE EXPECTANCY
70
causes of inflammatory myopathies
dermatomyosists polymyosists inclusion body myositis
71
what is dermatomyositis
Most common inflammatory myopathy in childhood * In adults associated with malignancies
72
clinical presentation of dermatomyositis
proximal weakness + rash rash ; heliotrope rash around eyes , eyelid and face gottrons papule on kunkles , fingers
73
investigtions of dermatomyosits
ck elevated
74
what invetigation used for monitioring dermatomyosits
ck
75
how to diagnose dermatomyosits
by biopsy and clinical pictuer
76
treatment of dermatomyosist
immunosuppressive
77
patholphysiology of dermatomyosists
b cell mediated antibody production against capiilary membrane leading to vasculitis
78
what is poiymositis ?
proximal weakness elevated ck CD8 TCELL MEDIATED ATTACK ON MUSCLE CELL diagnose on clinical pictuer and muscle biopsy
79
what is inclusion body myositis ?
age more than 50 proximal more than distal may be asymetrical
80
inclusion body myosits include which muscles
quadriceps long flexors esophagus ankle dorsiflexors
81
investigations of IBM
ck mildly elevated muscle biopsy ; rimmed vacuoles
82
ibm can be misdiagnosed with
polymyositis in early stage
83
IBM IS NOT RESPONSIVE TO
IMMUNOSUPPRESIVE THERAPY
84
biopsy fiinding in each of inflammatory myositis
dermatomysoits ; perifasicular atrophy IBM ; rimmed vacuoles polymyosits; mononuclear cells surronding and invading non necrotoc fibers in endomysium

neurology (5 decks)

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