Week 232 - Epilepsy Flashcards

0
Q

Week 232

What is a petit mal seizure?

A

This is now known as a FOCAL seizure. Occure in one part of the brain.

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1
Q

Week 232

What is epilepsy?

A

A tendency to recurrent unprovoked seizures
Present in 0.5% of population (~1 in 200 UK children)

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2
Q

Week 232

What is a grand mal seizure?

A

This is actually a TONIC-CLONIC siezure

May be preceded by a Prodrome (‘funny feeling/behaviour’ but no EEG changes) +/or an
Aura (simple partial seizure, with EEG changes)

Tonic Phase - Pt becomes rigid, may collapse if standing Don’t breathe - may become cyanosed

Clonic Phase - Arrhythmic jerking of body/limbs
Breathing is irregular, cyanosis may persist
Tongue biting & incontinence of urine may occur

Post-Ictal Phase
May last several hours, seizures may recur during it

Todd’s Paralysis: rarely occurs - Temporary limb paralysis

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3
Q

Week 232

What is Levetiracetam (Keppra)?

A

Levetiracetam (Lev-et-teer-rass-et-am) is a medicine which is used in myoclonic epilepsy, generalised epilepsy and partial epilepsy.

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4
Q

Week 232

What is Lamotrigine?

A

This is an antiepileptic drug, often used in adolescent girls.

Side effects are: Rash and irritability

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5
Q

Week 232

What is the lifetime prevalence of epilepsy?

A

5-10 people per 1000

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6
Q

Week 232

Does a single seizure qualify as epilepsy?

A

No. At least two seizures - epilepsy is the continuing tendency to have such seizures.

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7
Q

Week 232

How are seizures classified?

A

According to the localisation of seizure, and the aetiology. I.e. Generalise or Partial Further divided to Tonic-clonic, Absence Myoclonic (Generalised): Complex, simple, (partial)

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8
Q

Week 232

What is the most common type of partial epilepsy?

A

Temporal lobe epilepsy (60%)

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9
Q

Week 232

What are the differences between symptomatic and cryptogenic aetiologies of epilepsy?

A

Symptomatic - clear cause, i.e. brain tumour

Cryptogenic - Likely underlying cause, but not yet identified.

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10
Q

Week 232

What are the differentials for epilepsy?

A
  • Syncope/vasp-Vagal
  • Postural hypotension
  • TIA (not v often)
  • Trauma
  • Non-Epileptic attacks
  • Sleep disorders
  • Hypoglycaemia
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11
Q

Week 232

Name some seizure “markers”, useful if you don’t have a history.

A

Stereotyped

lateral tongue biting

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12
Q

Week 232

What are the three P’s of Syncope?

A

Posture - standing

Provocation - heat, venesection, micturition

Prodrome - Nausea, clammy, blurry vision, deafness, tinnitus

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13
Q

Week 232

What are the red flags for cardiogenic syncope?

A

Other cardiac symptoms

FH of sudden death

If it occurs on exertion

Rapid recovery

FH of IHD

No warning

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14
Q

Week 232

What is JME epilepsy?

A

Juvenile myoclonic epilepsy. A young person with an epileptic episode, and Myoclonic jerks.

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15
Q

Week 232

A partial seizure with concomitant loss of consciousness is known as what?

A

A complex partial seizure

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16
Q

Week 232

What are the three A’s of temporal lobe (complex partial) seizure?

A

Aura - warning sign

  • changes in taste/smell/hearing/sight
  • rising sensation Arrest
  • motor and speech Automation
  • Manual or oro-facial (i.e. smacking lips) Post-Ictal confusion
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17
Q

Week 232

In occipital lobe siezures, what do patients tend to see during aura?

A

Coloured balls of light

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18
Q

Week 232 What are the “clues” leading you to a diagnosis of a non-epileptic attack?

A

Awareness retained gradual onset Prolonged - up to a couple of hours! Frequent No response to AED Preceded by autonomic arousal Eyes closed and resist opening Back arching Biting tip of tongue

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19
Q

Week 232

What mediates a non-epileptic attack?

A

They are psychologically mediated.

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20
Q

Week 232

What % of diagnosed epilepsy patients actually have NEAD (non-epileptic attack disorder)?

A

About 20% actually suffer from Non-Epileptic attack disorder.

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21
Q

Week 232

What are the indications for a LOC being a seizure?

A
  • .Trigger
  • Sudden onset
  • Prodrome
  • Lasts 1-5 minutes
  • Common, rhythmic, synchronous jerks
  • Cyanosis
  • Tongue biting is common
  • Prolonged recovery
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22
Q

Week 231

How is “coma” defined?

A

Unrouseable

Unresponsive

Unaware of ext. stimuli (pain/verbal)

GCS low

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23
Q

Week 231

Define: Arousal

A

Level of consciousness/alertness Function of reticular activating system in pons and midbrain

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Week 231 Define: Awareness
Content of consciousness Awareness of self and surroundings
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Week 231 What can cause coma?
Sig. structural injury of the cerebral hemispheres Structural injury to the brainstem Diffuse physiological brain dysfuntion Metabolic/endocrine dysfunction
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Week 231 What is locked-in syndrome?
De-efferented motor tracts Blinking and vertical eye movements intact Awareness and arousal retained Can be mistaken for COMA.
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Week 231 What is a pyschogenic coma?
These can be confused with coma. Can be difficult to diagnose Need to exclude other causes Tests inc. face slap and tuning fork in nose!
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Week 231 What can cause cerebral hemisphere damage?
Blunt trauma - subdural haemorrhage Stroke Infarction Aneurysm (Berry type can cause subarachnoid bleed) Meningitis Encephalitis Mass Acute hydrocephalus Hypoxic-ischaemic encephalopathy
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Week 231 What can cause direct brain stem dysfunction?
* Bilateral thalamic lesions * Basilar artery thrombosis * Stroke in brain stem (usually major) * Neck injury - can cause vertebral artery dissection - clots thrown off from this can cause brain stem stroke * Brain stem tumour * Coning
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Week 231 What can cause diffuse physiological brain dysfunction?
* Wernickes * Global Hypoperfusion (hypoxic brain injury) * Status epilepticus * Hypothermia * Drugs/toxins/poisons Alcohol
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Week 231 In a simple partial seizure, is there any loss of consciousness?
No.
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Week 231 What is the initial management of coma?
* Improve oxygenation * Intubate if necessary * Correct hypotension and extreme hypertension * Warm/cool as required * EMPIRACALLY TREAT Glucose + Thiamine (hypogluc/wernickes) Naloxone/flumenazil if opiate/benzo overdose suspected Identify and treat underlying cause
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Week 231 A simple partial seizure presents as a ____ \_\_\_\_.
A stereotypical aura (can be very short, is usually "experienced")
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Week 231 How can you determine the depth of a coma?
GCS score or equivalent
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Week 231 You can't perform a reliable neuro assessment (in a coma patient) if... (there are several reasons!)
* If metabolically deranged * If sedated * If hypothermic * If they have endocrine disturbance
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Week 231 What aspects should be included in the neurological assessment of a patient in a coma?
* Brain stem reflexes (pupil reaction and eye movement part. useful) * Is there papilloedema? * Is motor response asymetrical? * Are there unilateral UMN signs? * Is there meningism * Are reflexes present? Brisk? Deranged?
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Week 231 What effect does cocaine/E/MDMA have on pupils?
Enlarged pupils! They get BIG BRO. Real big.
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Week 231 A single "blown pupil" is a sign of what?
**_Impending doom!_** ## Footnote Raised ICP.
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Week 231 Roving eye movements suggest what?
That brainstem is intact
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Week 231 A good corneal response indicates what?
A good corneal response Indicates that there is integrity of brainstem.
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Week 231 What is the **oculocephalic** response?
**Normal** - eyes maintain fixation on a point whilst the head is turning. **If brain stem is damaged** - eyes move with head. NB May also be affected by drugs and anaesthetic agents.
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Week 231 **Pontine lesion**s can cause what?
Bilateral small pupils (**miosis**)
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Week 231 A blown pupil is a sign of what?
Impending dooooom. CN 3 damage secondary to **coning through tentorium** and stretching/pressure on nerve. Require URGENT neuro imaging.
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Week 231 Bilateral unresponsive pupils are often caused by what?
Drug abuse (recreational)
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Week 231 What are the **brain stem reflexes**?
Pupils Corneal reflex Gag reflex Response to hypercapnia "dolls eye test"
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Week 231 In patients in a coma, which investigations would you arrange?
* Routine bloods * Toxicology screen * Imaging CT/MRI/MRA:CTA (last only if basilar artery. thrombosis suspected)
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Week 231 If initial tests came back normal, what specialist tests could you use in patients with coma?
* EEG * Lumbar puncture - NB NOT WITH RAISED ICP (it can cause coning) * If suspected meningism **ALWAYS SCAN BEFORE LP**
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Week 231 What is a persistent vegetative state?
**Recovery of arousal but not awareness NO language or comprehension** Roving eye movements Brainstem reflexes intact Maintain respiration and circulation May appear to have sleep-wake cycle.
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Week 231 What is the usual cause of coma in persistent vegetative state?
This progression is usually due to diffuse cortical or subcortical haemorrhage
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Week 231 What are the risk factors for persistent vegetative state?
* Length of time in coma * More common in older patients * Non-traumatic brain injuries * May be transient or permanent
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Week 231 What is meant by the term minimally conscious state?
* Eye contact * Semi purposeful/meaningful actions * Can Track objects * *Probably more common than PVS, though severely disabled*
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Week 231 ## Footnote What is the usual **cause of locked in** syndrome?
**Pontine infarction** due to damage/blockage of **basilar artery**.
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Week 231 What is a secondary generalised seizure?
A generalised seizure that has been preceded by one or both types of partial seizure.
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Week 231 Generalised seizures always involve the whole \_\_\_, and always produce complete/impaired ____ \_\_\_\_ \_\_\_\_\_\_\_\_\_\_.
They always involve **the whole brain**. And they always produce a complete or **impaired loss of consciousness**.
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Week 231 What are the brain stem death tests?
* Absent pupil response * Absent corneal response * Absent vestibulo-ocular reflex (dolls eyes) * Absent cough * Absent motor response to pain * Absent resp response to hypercapnia
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Week 231 Give examples of **autosomal dominant** conditions.
* Huntingdon disease * Neurofibromatosis 1 T * Tuberous sclerosis Complex * HMSN I * Von Hippel Lindau Familial hypercholesterolaemia * Long QT syndrome * BRCA 1 + 2 * Achondroplasia * Marfan's syndrome
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Week 231 Depigmented patches, said to look like "ash leaves" are an indication of which condition? They also have periventricular calcification.
Tuberous sclerosis
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Week 231 Tubosclerosis is caused by mutations in **one of which two genes**?
* Either TSC1 (codes for hamartin) or * TSC2 (codes for tuberin) gene * These code for cell growth - hence tumours in odd places!
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Week 231 What is meant by the term **penetrance**?
Proportion of individuals with a particular mutation who exhibit clinical symptoms..
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Week 231 A **short** seiezure, accompanied by **no automatisms** (like continuous blinking, or clenching of fists etc), that was **precipitated by hyperventilation**...patient described "not as having collapsed, but **seeming to have impaired consciousness**, not responding to stimuli during the event...." What type of seizure is this?
This is an **absence** seizure most likely, a form of generalised seizure. * short * no automatisms * hyperventilation prior to * impaired consciousness
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Week 231 In genetics, What is meant by the term **anticipation**?
The tendency of some variable dominant conditions to become more severe or occur earlier in successive generations.
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Week 231 A **CAG repeat blot with threshold 35** is the diagnostic (genetic) criteria for which **autosomal dominant** disease?
Huntindons
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Week 231 90% of patients with juvenile huntingdons have inherited their expanded triplet repeat sequence from which family member?
Father.
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Week 231 What is the **mode of inheritance of Kennedy** disease?
This is an X-linked recessive condition This means: * Males can get it more often - only have one X on chromosome * Females need both copie to be faulty to get condition
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Week 231 Name some common **autosomal recessive** diseases.
* Tay Sachs * Neuronal ceroid lipofuscinoses * Mucopolysaccharidosis I-VII (except II)
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Week 231 A **cherry red spot on the macula** is classical in which AS recessive neurodegenerative disease?
Tay Sachs
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Week 231 Give some examples of x-linked conditions
* HAemophillia * Duchennes * Fragile X syndrome
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Week 231 What are the clinical signs of **muscular dystrophy**?
* Lordosis * Poor posture * Moving onto "toes"
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Week 231 What is exon skipping?
In molecular biology, exon skipping is a form of RNA splicing used to cause cells to “skip” over faulty or misaligned sections of genetic code, leading to a truncated but still functional protein despite the genetic mutation.
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Week 231 In duchennesm uscular dystrophy, the commonest abnormality is that ____ \_\_\_\_ is missing.
Exon 45.
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Week 231 What is the impact of "mitochondrial transmission"?
If mother is affected,all children are affected. If father affected, no children affected.
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Week 231 Give an example of mitochondrial diseases?
* Lebers * hereditary optic neuropathy * Kearns Sayres syndrome
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Week 231 What is meant by the term "**epigenetics**"?
The study of the system that turns genes on and off, and its influences.
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Week 231 Aicardi syndrome is a (rare) disease (only occurring in girls), that is caused by what?
Skewed x inactivation
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Week 231 De novo deletion of 15q11-13 (PATERNALLY inherited) is the causative factor of which disease?
Prader willi
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Week 232 De novo deletion of 15q11-13 **maternally inherited** is the causative factor of which disease?
Angelman's syndrome.
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Week 231 What is meant by the term "copy number variation"?
This is descriving the fact that "The number of copies of a gene or part of a gene varies from one individual to the next".
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Week 232 Huntingdons, DRPLA, Spinocerebellar ataxias and myotonic dystrophy are all transmitted how?
Autosomal dominant
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Week 232 What is the most common type of genetic variation?
Single nucleotide polymorphism.
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Week 232 What is meant by the term "uniparental disomy"?
Both chromosomes of a pair originate from one parent.
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Week 232 What are **myoclonic seizures**?
**Brief, arrhythmic, high-amplitude jerking movements** o Last \<1 second with no pre-warning & no post-ictal phase o BUT often cluster within a few minutes  May evolve into tonic-clonic seizures o Not followed by post-ictal phase  Classic EEG pattern o **Fast, high amplitude spikes of \>5 Hz**
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Week 231 What are **tonic seizures**?
**Sudden onset rigidity, often noticeable due to flexion/extension** o Usually last several seconds o Often occur during sleep or when drowsy
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Week 231 What is **West syndrome**?
Infantile spasms. Onset is usually at 4-6 months of age.
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Week 232 What are the aetiology, features and treatment of West syndrome?
**Aetiology** Associated with structural brain disorders (e.g. Sturge-Weber), perinatal asphyxia, hx of meningitis etc. **Features** Salaam/Jackknife Spasms- Flexor spasms of head, trunk & limbs Last 1-2s & occur in bursts of 20-30 spasms Typically occur on waking, but may occur throughout the day Regression in development -Adults typically have severe LDs EEG **Hypsarrhythmia** - High amplitude ‘sharp’ waves on background of arrhythmic slow waves **Treatment** * *ACTH - Given as IM injection** **Prednisolone**
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Week 232 What is **Lennox-Gastaut syndrome**?
Onset at 1-4years of age Often proceeded by infantile spasms **Features** Children may have absences, atonic ‘drop’ attacks, or myoclonic jerks Regression in development occurs **EEG** Slow (2Hz) spike & wave activity **Prognosis** Poor - tends to continue into adulthood. Adults ten d to have severe LDs
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Week 231 What is **Benign Rolandic Epilepsy Of Childhood**?
Most common benign epilepsy of childhood o Onset at **3-10 years**, declining in frequency past 10. Pts usually seizure free by 15 o **Family history common** - Dominant inheritance pattern o Features  **Simple partial seizures whilst awake**  Facial sensory symptoms e.g. numbness/tingling in face  Preserved memory - Th. partial  Generalised tonic-clonic seizures whilst asleep o EEG  Spike & Wave activity in Centro-Temporal Region o Treatment  **No treatment necessary - Child will grow out of it**
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Week 232 What are **childhood absence seizures**?
**Onset at 4-12 years** Usually seizure free by adulthood **Features** Brief Absence Seizures - Last \<20 seconds Child suddenly stops & stares into space - may twi tch eye/hand etc. Child able to continue interrupted conversation/ action afterwards Can be induced by hyperventilation **EEG** 3 Hz (i.e. fast) spike & wave activity - Classic
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Week 232 What is **Juvenille Monoclonic epilepsy**?
_a.k.a. Myoclonic Epilepsy of Adolescence_ Onset at 10-20 y.o., with Females \>Males - 2:1 ratio Family history of disease common **Features** Brief Myoclonic Jerks, usually occurring in morning after waking e.g. Throwing Cornflakes & milk out of cereal bowl No developmental delay, responsive to treatmen
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Week 232 What are febrile convulsions?
These are NOT epilepsy. Usually just occur for short periods if the child has a fever. They are generalised tonic clonic seizures.
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Week 232 Name **two** symptoms that are suggestive of **true seizures**.
Pupil dilation (Miosis) Peripheral cyanosis
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Week 232 What is the acute management of a **Tonic-Clonic** seizure?
Place pt into recovery position If seizure persists for 5 minutes: * Give **IV Lorazepam** if possible * Give **buccal midazolam or rectal diazepam** if IV access not possible
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Week 232 **Sodium valproate** is the **first line** treatment for what?
* **All generalised seizures and some partials.** * Side effects include \< apetite and weight gain, hair loss and liver failure.
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Week 232 **Carbamazapine** is the **first line** treatment for what?
* **Partial seizures** * SE include lupus, dizziness and visual disturbances.
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Week 232 Which antixonvulsant could you give to teenage girls?
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Week 232 Which two **epilepsy medications are contraindicated in pregnancy**?
Valproate, and Carbamazepine cause neural tube defects.
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Week 232 After a **one-off siezure**, **how long** must patients no drive for?
6 months
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Week 232 After more than one siezure, how long must aptients not drive for?
At least one year seizure free before allowed to drive. 6 months after med change OK to drive if last 3 years all seizures have been whilst asleep.
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Week 232 What is **Catamenial** epilepsy?
EPilepsy that is influenced by hormones (in women)
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Week 232 Spatial memory is a function of which part of the brain?
The Hippocampus
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Week 232 The fear response is related to which part of the brain?
The Amygdala
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Week 232 List some personality traits of patients with temporal lobe epilepsy
* Hypergraphia * Hyposexuality * Emotional viscosity * mood swings * hyper religosity *
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Week 232 27 year old woman with an episode of loss of consciousness while standing on the bus, preceded by nausea and sweating. Witnesses report some limb jerks with rapid recovery. **What is the most likely diagnosis**?
Vaso-Vagal Syncope
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Week 232 70 year old lady with a background of ischaemic heart disease and cerebrovascular disease felt disorientated and exhausted on waking one morning. Her husband reports being woken in the night by a loud noise and described rhythmic jerking movements of all 4 limbs. Her lips turned blue and she bit the side of her tongue. She was not incontinent of urine. **What is the most likely diagnosis?**
This is most likely a generalised tonic clonic seizure Why? Rhythmic Bit SIDE of tongue Cyanosis
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Week 232 65 year old man with a sudden onset of collapse while hill walking. There was brief loss of consciousness with rapid recovery. He has a past history of type 2 diabetes, depression and ischaemic heart disease. **What is the most likely diagnosis?**
Cardiac syncope Why? IHD Happened during exhertion Rapid recovery
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Week 232 21 year old lady with recurrent attacks consisting of abnormal movements of all 4 limbs. The attacks vary in description and can last up to an hour. She has a past history of asthma and irritable bowel syndrome. **What is the most likely diagnosis?**
This is most likely a non-epileptic attack Why? Young female Non rhythmical movements of limbs Long duration
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Week 232 ## Footnote 18 year old man with a new onset of stereotyped attacks preceded by an unpleasant smell, an odd sensation in his stomach and a feeling of fear and anxiety. Witnesses report how he loses awareness, fiddles with his clothing and smacks his lips. **What is the most likely diagnosis?**
**This is most likely a complex partial seizure** Why? Partial - Prodrome sensations Partial - not generalised symptoms, fear suggests focal around limbic system (?) Complex - HE loses awareness - therefore impairment of consciousness Autonomous behaviour (fiddles with clothing and smacks lips)
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Week 232 A 20 year old student is referred to a first seizure clinic following a generalised tonic clonic seizure while on a night out. Over previous months she has noticed some involuntary jerks of her arms, particularly in the morning. **What advice should you give to this patient?**
Should advise to refrain from driving, and also advise that they contact the DVLA
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Week 232 Which of the anti -epileptic drugs is associated with the greatest risk of developmental malformations to an unborn child?
Sodium Valproate
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Week 232 Which of the anti-epileptic drugs does **not reduce the efficacy of the oral contraceptive pill**?
Clobazam
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Week 232 What is **phenytoin**?
This is an anticonvulsant drug anticonvulsant drug used primarily in the management of complex partial seizures and generalized tonic-clonic seizures.
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Week 232 What is the obvious abnormality in this image?
Brain tumour
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Week 232 What is the name of this condition?
This is a cavernoma.
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Week 232 What is the name given to this condition?
Heterotopic gray matter (around the ventricles) - very epileptigenic
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Week 232 What is the abnormality in this image?
Hippocampal sclerosis
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Week 232 What is the name given to the condition shown here?
Neurocysticercosis
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