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3C week 5 - thinking and moving > neuromuscular disease > Flashcards

neuromuscular disease Flashcards

1
Q

5 characteristics of motor neuron disease (presentation)

A
  1. painless and absence of sensory symptoms;
  2. florid fasciculations;
  3. strong triceps and finger flexors;
  4. split hand (sparing of hypothenar eminence);
  5. combination of UMN and LMN signs
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2
Q

what is Romberg’s test

A

tests balance (specifically tests dorsal column for proprioception) - pt stands with feet together and arms crossed of chest/ outstretched -> test to see how many seconds balance can be maintained for

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3
Q

what is split hand sign and what does it indicate

A

wasting of the thumb side intrisic hand muscles+ thenar eminence even though these are supplied by different nerves -> idicates that there is an issue in the anterior horn -> often seen in MND

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4
Q

bulbar signs of MND (8)

A
  1. dysphagia
  2. difficulty chewing
  3. nasal regurgitation
  4. slurring of speech
  5. difficulty handling secretions
  6. choking on liquids
  7. dysphonia
  8. dysarthria
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5
Q

what are benign cramp fasiculations and who are they most common in

A

continual muscle twitches without having an underlying medical condition, mostly localised to the calf -> seen in middle ages men frequently

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6
Q

what symptom in combination w fasiculations rules out benign cramp fasiculaiton syndrom

A

muscle weakness/wasting

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7
Q

what drug class might mask hyperthyroidism

A

beta blockers

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8
Q

thryotoxicosis presentation (3)

A

muscle weakness/wasting; fasiculations; weight loss

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9
Q

what is Kennedy’s disease

A

a PURE LMN disorder seen only in males with a slow progression

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10
Q

Kennedy’s disease presentation (5)

A
  1. proximal symmetrical weakness;
  2. tongue wasting;
  3. peri-oral fasiculations;
  4. sensory changes
  5. gynaecomastia (an overdevelopment or enlargement of the breast tissue in men or boys)
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11
Q

what is cervical spondylotic myelopathy

A

a degenerative condition that causes changes of the spine, resulting in compression of the cord and nearby structures

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12
Q

what is the commonest cause of spastic paraparesis

A

cervical spondylotic myelopathy

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13
Q

cervical spondylotic myelopathy presentation (5)

A
  1. UMN signs caudal to LMN signs (i.e. UMN in legs and LMN in arms)
  2. Lhermitte’s sign
  3. pain
  4. sensory symptoms
  5. initial progressive phase then plateau
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14
Q

what is multifocal motor neuropathy with conduction block

A

a rare, acquired, motor neuropathy characterized by progressive asymmetric weakness without sensory problems

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15
Q

multifocal motor neuropathy with conduction block presentation

A

upper limb presentation -> slowly progressive asymmetrical weakness, cramps and fasiculations

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16
Q

what is a key clue in the diagnosis of multifocal motor neuropathy with conduction block

A

weakness in non-wasted muscles

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17
Q

what is the name for the tunnel in which the ulnar nerve runs at the WRIST

A

tunnel of guyon

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18
Q

tunnel of guyon compression symptoms (2)

A
  1. weakness and wasting of intrinsic muscles of the hand
  2. the absence of pain/sensory loss
19
Q

compression of arcade of froshe symtpoms

A
  1. weakness of wrist extension;
  2. radial drift;
  3. weakness of finger extension, thumb extension and abduction
20
Q

what is the arcade of froshe

A

The most superior part of the superficial layer of the supinator muscle -> deep radial nerve runs under

21
Q

what is a neuronopathy

A

a problem with neuronal cell bodies (anterior horns and DRGs)

22
Q

what is a mono neuropathy multiplex

A

a problem with lots of individual nerves

23
Q

8 common causes of peripheral neuropathy

A
  1. diabetes
  2. hypothyroidism
  3. alcohol
  4. drugs (chemo)
  5. autoimmune conditions
  6. hereditatory conditions
  7. infection
  8. vitamin deficency

DAVID - Diabetes, Alcohol, Vitamin deficiency (B12), infection/inherited, Drugs

24
Q

what symptoms rule out peripheral neuropathy as a diagnosis

A

weakness

25
Q

3 types of peripheral neuropathy

A
  1. purely motor
  2. purely sensory
  3. mixed
26
Q

what is mono-neuritis multiplex

A

a type of peripheral neuropathy where individual nerves are picked off randomly, it has a sub-acute presentation

27
Q

causes of mono-neuritis multiplex (2)

A

vasculidities, CTDs

28
Q

what is chronic inflammatory demyelinating polyneuropathy (CIDP)

A

a slowly developing autoimmune disorder in which the body’s immune system attacks the myelin -> the chronic version of Guillian-barre syndrome

29
Q

CIDP presentation

A

chronically progressing/relapsing symmetric sensorimotor disorder with weakness of limb, facial and extra occular muscles

30
Q

4 investigations for a diagnosis of CIDP

A
  1. lumbar puncture
  2. nerve conduction studies
  3. MRI spine (with gagolinium)
  4. nerve biopsy
31
Q

Mgx of CIDP (4)

A
  1. corticosteroids
  2. immunosuppressants
  3. plasmapherisis (extreme cases)
  4. IV Ig
32
Q

what is inclusion body myositis

A

an idiopathic inflammatory myopathy - a rare condition that causes muscle weakness and damage

33
Q

inculsion body myositis pathophys

A

Inflammatory cells invade the muscle tissue and concentrate between the muscle fibers; multiple inclusion bodies that contain cellular material of dead tissue surrounded by inflammatory cells is seen in a biopsy sample

34
Q

inclusion body myositis presentation (5)

A
  1. early prominent weakness of finger flexors and quadriceps (different pattern to MND);
  2. dysphagia;
  3. foot drop;
  4. muscle wasting - forearm scalloping especially prevalent;
  5. NO fasiculations
35
Q

inclusion body myositis mgx

A

PT, OT , psychological support;

can give steroids but won’t be that effective

36
Q

what is the commonest cause of muscle weakness in YAs

A

limb girdle muscular dystrophy

37
Q

what is Becker’s muscular dystrophy

A

a genetic disorder that gradually makes the body’s muscles weaker and smaller -> less severe than Duchenne’s

38
Q

Becker’s muscular dystrophy presentation (7)

A

1.Delayed developmental motor milestones;
2. Clumsy/Frequent falls;
3. Difficulty rising from the floor, may show Gowers’s sign
4. sway back;
5. thin, weak thighs;
6. toe walking (tight achilles due to contracture)
7. thick calfs (muscle replaced w fat)

(see yr 1)

39
Q

becker’s muscular dystrophy pathophys

A

absence of dystrophin stops calcium from entering the cell membrane affecting the signaling of the cell, water enters the mitochondria causing the cell the burst -> increased oxidative stress within the cell damages the sarcolemma resulting in the death of the cell, and muscle fibers undergo necrosis and are replaced with connective tissue
(see yr 1)

40
Q

what is duchenne muscular dystrophy

A

a genetic disorder characterized by the progressive loss of muscle

41
Q

duchenne muscular dystrophy presentation (7)

A
  1. toe walking;
  2. difficulty running/climbing up stairs;
  3. frequently falling;
  4. Learn to speak later than usual;
  5. proximal muscle weakness (usually appear in lower limbs first);
  6. Pseudohypertrophy of calf muscles;
  7. gower’s sign
42
Q

what is rhabdomyolisis and what is seen to indicate this in the blood

A

the destruction of striated muscle cells -> raised CK in bloods

43
Q

common cause for rhabdomyolysis (4)

A
  1. trauma;
  2. drugs/toxins;
  3. infection;
  4. extreme physical exertion
44
Q

apart from bloods, what investigation should be done in suspected rhabdomyolysis

A

urine sample -> dark urine, presence of myoglobin

3C week 5 - thinking and moving (5 decks)

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