Neurology 3 Flashcards

1
Q

Dying forward hypothesis means?

Dying backward hypothesis means?

A

Forward - glutamate toxicity - degeneration of anterior motor horn

Backward - starts degeneration at muscle/Neuromuscular junction

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2
Q

Splint hand syndrome in motor neuron? - what muscles wasted?

A

Thenar and 1st interossei muscle wasting

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3
Q

Clinical features of Motor neuron disease?

A

Asymmetrical weakness in either distal/proximal
Fasciculations
Pseudobulbar (UMN) symptoms
Respiratory dysfunction

NO SENSORY INVOLVEMENT

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4
Q

Investigations in MND shows?

A

Normal conduction studies

  • Reduced CMAP
  • Normal SNAP

EMG - fasciculations, fibrillations

MRI - corticospinal hyperintensity

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5
Q

Holmes tremor ( Resting + Action + intentional tremor) where is the lesion?

A

midbrain tegmentum outside of red nucleus

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6
Q

What is dystonia?

A

intermittent/sudden muscle contractions - twisting/torticollis

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7
Q

What is Positive myoclonus and negative myoclonus?

A

Fastest/brief sudden increase in tone

Negative myoclonus - decrease in tone due to Ammonia/CO2

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8
Q

What is dyskinesia?

A

Overactivity movement

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9
Q

why cogwheel rigidity occurs?

A

Tremors superimposed on increased tone

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10
Q

REM sleep disorder is marker of what?

A

Poorer outcome for dementia

Only Alpha synuclein can cause it!

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11
Q

Motor fluctuations in PD occurs when?

A

After > 2 years -on conventional therapy + higher levodopa dose
Longer duration of PD ( younger patients)

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12
Q

What to watch out if give MAOB inhibitors?

A

MAOB inhibit MAOB and MAOA

  • MAOA breaks down noradrenaline ( from tyramine)
  • so no MAOA = adrenergic crisis
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13
Q

Treatment for motor fluctuations?

A

Break down dose to increase frequency

Add COMT inhibitors

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14
Q

What sign is often indicator of advanced dementia?

What is strongest predictor of dementia?

A

Visual hallucinations

Age

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15
Q

Fragile X syndrome - how many nucleotide expansion before gene is ‘turned off’ - problematic?

Premutation syndrome - how many nucleotide expansion?

A

200 CGG repeats = FMR1 protein deficiency causing Fragile X syndrome ( intellectual disability/seizures) or FXTAS

> 40, < 200 CGG repeats = Premutation syndrome ( Primary ovarian insufficiency/ Neurodevelopmental delays and psychiatric disturbances) or FXTAS

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16
Q

Huntington disease - HTT protein toxic to what in brain?

A

Striatal neurons

17
Q

What is Westphal Variant?

A

Huntington manifestation < 20 yrs. CAG > 55

18
Q

Commonest organism to cause rhomboencephalitis?

A

Listeria

19
Q

Epilepsia partialis continua/kojevnikov epilepsy is pathognomic for?

A

HSV encephalitis

seizure every seconds to minutes hence PLEDS

20
Q

Herpes simplex PCR from CSF accuracy?

A

High sensitivity and specificity but can be negative first 24 hours

21
Q

Metagenomic next generation sequencing good for?

A

Detect virus antigen from RNA/DNA in CSF ONLY

22
Q

Paraneoplastic Encephalitis - mediated by? prognosis?

Autoimmune encephalitis - mediated by? Prognosis?

A

Paraneoplastic - T cell mediated = intracellular antigen = poor prognosis

*ANTIBODIES ARE BYSTANDER AND A/W MALIGNANCY SO MUST CHECK FOR IT

Autoimmune - B cell mediated = extracellular/surface antigen = good prognosis

23
Q

Paraneoplastic antibodies and a/w encephalitis?

A

anti-HU = SCLC ( Limbic encephalitis)

Anti-YO = Breast/ovarian cancer ( Cerebellar degeneration)

anti-Ri =opsoclonus myoclonus ( Rhomboencephalitis)

Anti-Ma = Testicular cancer (Rhomboencephalitis)

24
Q

HSV encephalitis goes on to develop what autoimmune encephalitis?

A

NMDA encephalitis

25
Q

Commonest autoimmune encephalitis and pathognomonic symptoms?

A

NMDA encephalitis

have preceding viral flu syndrome

26
Q

NMDA treatment?

A

Remove cancer
Give IV methylprednisolone then oral prednisolone
IVIG/ plasmapheresis

27
Q

PRES syndrome MRI shows?

A

Posterior circulation edema and increased hyperintensity

28
Q

Injury in spiral groove - signs?

Injury above spiral groove - signs?

A

In spiral groove - Brachioradialis weakness, wrist and finger EXTENSION weakness

Above spiral groove - no triceps support = elbow extension weakness

29
Q

Sensory pattern
-high lesions affecting radial nerve = where is numbness?

-low lesions ( past posterior cutaneous nerve of forearm) affecting radial nerve = where is numbness?

A
  1. entire dorsal lateral arm ( towards thumb side)

2. Dorsal part of hand ( thumb till half of 4th digit)

30
Q

Brachioradialis innervated by what nerve root?

A

C5/6

31
Q

PSP sign means?

A

Midbrain atrophy without pontine involvement

32
Q

Withdrawing Levodopa abruptly causes?

A

Akinesia

Neuroleptic malignant syndrome!!

33
Q

Side effects of Dopamine replacement therapy?

A

Dyskinesia as dopamine absorbed erratically from GI tract

34
Q

Side effects of Levodopa?

A

Peripheral neuropathy

Dopamine dysregulation syndrome

35
Q

Benign course of parkinson indicators?

A

Female
Tremor predominant
Earlier onset