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Neurology > Neuromuscular Disorders > Flashcards

Neuromuscular Disorders Flashcards

1
Q

What are the sx of NMD?

A

-Muscle weakness/wasting;
-sensory loss/numbness;
-clumsiness;
-poor balance;
-diplopia;
-dysphagia;
-dyspnoea etc.
Also cramps, burning, stiffness

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2
Q

What is Charcot Marie Tooth disease?

A

Inherited distal predominant group of disorders.

Genetically determined, chronic, slowly progressive and gradually disabling disorders.

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3
Q

Important Hx features of NMD?

A

Time: acute or chronic
FHx: any similar presentations in family

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4
Q

Which NMD conditions cause diplopia and ptosis?

A

Myasthenia Gravis
Ocular nerve palsy
Thyroid eye disease
NOT MND

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5
Q

What are the head and neck sx of NMD?

A

Diplopia, ptosis

Dysphagia

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6
Q

What are the clinical features of MND?

A
  • Muscle wasting (including tongue)
  • Fasiculations
  • Tone (increased or decreased)
  • Generalised weakness (no pattern)
  • Reflexes preserved until late (or exaggerated)
  • Plantars may be upgoing
  • NO SENSORY LOSS
  • Cranial nerves (except bulbar) spared
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7
Q

What is the commonest MND?

A

Amyotrophic lateral sclerosis

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8
Q

Peak onset ALS?

A

55 - 70 years

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9
Q

How is ALS diagnosed?

A
  • Clinical; UMN and LMN signs with intact sensation.
  • Consistent lab findings - EMG
  • Exclusion of other diagnoses
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10
Q

What is the prognosis of MND?

A

Avg life expectancy
Bulbar onset: 6-12 months
Limb onset: 2 - 5 years

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11
Q

What is the Mx MND?

A 
Supportive: 
-speech path swallow and communication, peg feeding.
-non-invasive ventilation
-mobility adis
Rx: riluzole 50mg bd
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12
Q

What is hereditery spastic paraplegia?

A

Spasticity of lower limbs; realtively preserved strength.

Inherited, mutations in spatin gene.

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13
Q

What is adenomyeloneuropathy?

A

X-linked disorder of excess long chain fatty acids

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14
Q

What is primary lateral sclerosis?

A

Similar to ALS but no LMN signs.

Better prognosis

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15
Q

What is lamberton eaton myasthenia syndrome?

A

Presynaptic ABs vs Ca2+ channels

Often associated with neoplasms of small cell lung

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16
Q

What is the Tensilon test?

A

Short acting acetylcholinesterase antagonist (e.g. edrophonium)
Dx of MG

17
Q

What bloods for MG Dx?

A

Autoantibodies for ACh receptor

  • pure ocular positive (50%)
  • Gnereliased MG positive (90%)
  • Seronegative cases approx 70% +ve for anti MuSK Abs
18
Q

What tests for MG diagnosis?

A

Tensilon test
Bloods - autoantibodies
CT chest
EMG

19
Q

How is myasthenia graves treated?

A
  • Immune modulation (plasma exchange, IV Ig, immunosuppression)
  • Acetylcholinesterase antagonists
  • Thymectomy
20
Q

What are the common types of focal neuropathy?

A 
Compressive: Ulnar neuropathy
Infection: leprosy
Inflammatory e.g. Bell's Palsy
Infiltrative: cancer, amyloid
Ischaemic: e.g. DM
21
Q

How are compressive neuropathies diagnosed?

A

History of focal symptoms

EMG/NCS

22
Q

What are the causes of multifocal neuropathies? (mono neuritis multiplex)

A

Almost always ischaemic e.g. diabetes, vasculitis

Occasionally infective: AIDS, CMV, sarcoidosis, leprosy

23
Q

Why is mono neuritis multiplex a medical emergency?

A

Often due to vasculitis (if not DM). Nerves will progressively infarct - cannot be restored.

24
Q

What are the acute demyelinating polyneuropathies?

A
  • Guillain-Barre Syndrome
  • AIDS seroconversion phase
  • Lymphoma
25
Q

What are the causes of acute axonal neuropathy?

A
  • Glue sniffers neuropathy (N-Hexane toxicity)
  • Vasculitis
  • Thiamine deficiency
  • Porphyria
26
Q

What do sensory disturbances exclude?

A

MND, MG and myopathy

27
Q

What does electrophysiology distinguish between?

A

Distinguishes:

  • neuropathy from myopathy
  • axonal from demyelinating
  • sensory v motor
  • NMJ disorder
28
Q

What are the causes of chronic axonal polyneuropathy?

A
  • Genetic: HMSN II
  • Metabolic: DM, hypothyroidism
  • Toxins: EtOH, drugs, prescription Rx, Heavy metals
  • Nutritional: thiamine, B12 deficiency, B6 excess
  • Paraneoplastic: Anti Hu
  • Immune: abnormal paraprotein, Sjogren’s
  • Ischaemic: diabetes, vasculitis
29
Q

What are the causes of chronic demyelinating polyneuropathies?

A
  • Genetic: HMSN Type 1
  • Immune: inflammatory (CIDP), non-inflammatory (IgM anti-MAG)
  • Paraneoplastic: POEMS

Neurology (38 decks)

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