Neurology Flashcards

1
Q

What is Wallenberg syndrome ??

A

Lateral Medullary Syndrome
-Vertebral A occlusion => Lateral Medulla stroke
- I/L Face Sensory loss (pain & temperature)
- C/L Body Sensory loss
- Horner’s syndrome (Ptosis, Miosis, Anhidrosis)
- Ataxia, Dysphagia & Dysphonia

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2
Q

What is Dejerine Syndrome ??

A

Central Pain Syndrome (CPS)
Thalamic Syndrome; occlusion of Thalamogeniculate artery, a branch of Posterior Cerebral Artery
Thalamus is the Relay centre for sensory pathway
- Body becomes Hypersensitive to pain
-

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3
Q

What is Foville Syndrome ??

A

Occlusion of Paramedian branch of Basilar artery
- I/L Facial weakness
- C/L Hemiparesis
- Conjugate gaze palsy
- Involves the Abducens nucleus & Parapontine reticular formation

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4
Q

What is Thalamic Stroke syndrome ??

A

Small vessel occlusion or Poste. Cerebral Artery issues
- C/L Sensory loss (Pain, Temp., Touch)
- Thalamic pain syndrome (Burning pain)
- Hemianopia

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5
Q

What is Locked in Syndrome ??

A

PONTINE stroke
- Complete paralysis with preserved Veritical eye movt.
- Preserved Cognition but unable to speak or move

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6
Q

What is Top of the Basilar Artery Synd. ??

A

Stroke of Top of Basilar artery => Ischaemia in Brainstem + Occipital lobe
- Altered consciousness/ Coma
- Visual disturbance (eg. blindness)
- Diplopia & Ataxia

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7
Q

What is Millard-Gubler syndrome ??

A

Stroke due to occlusion of Short Circumferential branch of Basilar Artery
- I/L Facial Palsy
- C/L Hemiparesis
- I/L Abduction deficit
- Involves 6th & 7th CN, Corticospinal tract

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8
Q

What is Striatocapsular Infarct ??

A

Occlusion of Lenticulostraite arteries (branches of MCA) causing stroke of Striatum & Internal capsule
- C/L Pure Motor Hemiparesis (affecting face, arm & leg; more severe in Face & Arms)
- Sensory deficit may be absent or minimal
- NO Cortical signs (eg., Aphasia or neglect), distinguishing it from Cortical stroke.

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9
Q

What is Posterior Cerebral Artery Syndrome ??

A

Posterior Cerebral artery occlusion => Visual & Memory disturbance
- Homonymous Hemianopia
- Visual agnosia (inability to recognize objects)
- Memory deficits

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10
Q

What is Claude Syndrome ??

A

Posterior Cerebral Artery occlusion => Stroke of Midbrain
- C/L Hemiparesis
- I/L Oculomotor Nerve Palsy (Ptosis, Mydriasis)
- C/L Ataxia

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11
Q

What is Anterior Inferior Cerebellar Artery syndrome ??

A

Occlusion of AICA => Cerebellar ischaemia
- Vertigo, Nausea
- U/L Hearing loss
- Ataxia & Dysarthria

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12
Q

What is Weber’s Syndrome ??

A

Posterior Cerebral Artery occlusion => Midbrain Stroke
- C/L Hemiparesis
- I/L Oculomotor nerve palsy (Ptosis, Mydriasis)

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13
Q

What is MCA syndrome ??

A

Occlusion of MCA => supplies a large portion of Cerebral cortex
- C/L Hemiparesis Face, Arm&raquo_space;> leg)
- C/L Sensory loss
- Aphasia (if Left MCA) & Hemineglect (if Right MCA)
- Homonymous Hemianopia

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14
Q

What is ACA syndrome ??

A

Occlusion of ACA => affects MEDIAL aspects of FRONTAL & PARIETAL Lobes
- C/L Leg weakness&raquo_space;> Arm/ Face
- Personality changes, Confusion & Disinhibition
- Urinary Incontinence
- C/L Sensory loss (primarily in Legs)

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15
Q

What is PCA syndrome ??

A

Occlusion of PCA => supplies OCCIPITAL lobe & Parts of TEMPORAL & PARIETAL lobes
- Homonymous Hemianopia (Visual field loss on Opposite side)
- Visual Agnosia (inability to recognise objects despite normal vision)
- Memory impaired (due to Hippocampal involvement)
- Sensory deficits & C/L Hemiparesis (if additional areas are affected)

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16
Q

What is Lacunar Stroke ??

A

Small, deep infarction, usually caused by occlusion of a Single Small artery, often in the BASAL
- Pure Motor Hemiparesis (without sensory loss)
- Pure Sensory stroke (without motor weakness)
- Ataxic Hemiparesis or Dysarthria/ Clumsy Hand syndrome

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17
Q

Name the Posterior Fossa Malformations

A

CHIARI 1 Malformation:
- Cerebellar tonsils downward displacement via F magnum; a/w Spinal C cavitation (eg Syringomyelia)
- Asymptomatic in kids, manifests in ADULTS: headache & cerebellar c/f
CHIARI 2 M:
- Cerebellum (Tonsil & Vermis) + Medulla downward displacement via F magnum
- Non-communicating Hydrocephalus more severe than Chiari 1
- Presents early in life- Dysphagia, Stridor, Apnea, limb weakness.
DANDY-WALKER M
- Agenesis of Cerebellar vermis
- Cystic enlargement of 4th ventricle that fills enlarged posterior fossa
- Non-communicating hydrocephalus

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18
Q

What is Syringomyelia ??

A

Fibres crossing the Anterior White Commissure (Lateral Spinothalamic tract) are damaged 1st => Cape-like loss of Pain & Temp in B/L ULs
- If lesion expands, damages Anterior Horns => LMN deficits
- Site: MC b/w C2 to T9
- a/w Chiari 1 M

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19
Q

What other conditions are a/w
- Chiari 2 M ??
- Dandy-Walker M ??

A
  • Meningomyelocele (usually Lumbo-sacral)
  • Spina bifida
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20
Q

What is the RMP of Neurons ??

A

-75 mV

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21
Q

What are the Watershed zones of the brain ??

A

Cortical border zones occur b/w
- Anterior & Middle cerebral arteries
- Posterior & Middle arteries
Internal border zones occur b/w
- Superficial & Deep vascular territories of the MCA
COMMON locations for Brain Metastases

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22
Q

Mention the C/F of strokes affecting Watershed zones

A

ACA-MCA watershed infarct
- Proximal ULs & LLs weakness (Man-in-a-Barrel syndrome)
PCA-MCA watershed infarct
- Higher order Visual Dysfunction

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23
Q

Name the Anterior Circulation Strokes

A

ANTERIOR Cerebral
- Motor & Sensory cortices of LLs
MIDDLE Cerebral
- Motor & Sensory cortices of ULs & Face
- Temporal lobe (Wernicke’s)
- Frontal lobe (Broca’s)
LENTICULO-STRIATE Artery
- Striatum, Internal Capsule
- Basal ganglia, Thalamus

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24
Q

Symptoms of ACA stroke ??

A

C/L paralysis & Sensory loss of LLs & Urinary incontinence

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25
Symptoms of MCA stroke ??
C/L paralysis & Sensory loss of - Lower Face & ULs If Dominant hemisphere involved - Aphasia If Non-dominant hemisphere - Hemineglect Wernicke's is a/w Rt. Superior Quadrant Visual F defect => Temporal lobe involvement - C/L Homonymous Hemianopia
26
Symptoms of Lenticulo-Striate artery stroke ??
C/L paralysis + Absence of Cortical signs (eg., neglect, aphasia, visual field loss) Pure Motor stroke are MC Common site of Lacunar Infarcts due to Microatheroma & Hyaline arterio-sclerosis (Lipohyalinosis) secondary to unmanaged HTN
27
Name the blood supplies of CNs
MEDIAL CNs - CN 3, 4 : PCA - CN 6 : Basilar - CN 12 : Anterior Spinal LATERAL CNs - CN 5, 7, 8 : Labyrinthine & AICA - CN 9, 10, 11 : Vertebral & PICA
28
Name the Posterior Circulation Strokes
PCA - Occipital lobe. - Supplies CN 3, 4 BASILAR artery - Pons, Medulla, Lower Midbrain - Corticospinal & Corticobulbar tracts - Ocular CN nuclei. - Paramedian Pontine Reticular Formation (PPRF) - Supplies CN 6 ANTERIOR INFERIOR C A - Facial & Vestibular nuclei - Spinothalamic tract, Spinal Trigeminal nucleus - Sympathetic fibres, Middle & Inferior Cerebellar peduncles. - Inner ear POSTERIOR INFERIOR C A - Nucleus ambigus (CN 9, 10) - Vestibular Nucleus, Lateral S T tract, Spinal Trigeminal nucleus - Sympathetic fibres - Inferior Cerebellar Peduncle ANTERIOR SPINAL Artery - Corticospinal tract - Medial leminiscus - Caudal Medulla- Hypoglossal nerve
29
Symptoms of PCA stroke ??
C/L Hemianopia with Macular Sparing DOMINANT Hemisphere, extending to Splenium of Corpus callosum - Alexia without Agraphia NON-Dominant H involved - Prosopagnosia: difficulty to recognise people face WEBER's Synd. (Midbrain stroke) - Paramedian PCA branch occlusion - I/L CN 3 palsy + C/L Hemiplagia (damage to I/L cerebral peduncle)
30
Symptoms of Basilar Artery stroke ??
Locked-in syndrome Consciousness preserved IF RAS spared Quadriplegia Loss of voluntary - Facial (except blinking); Mouth & Tongue movements - Loss of Horizontal but NOT Vertical eye movements
31
Symptoms of AICA stroke ??
Lateral Pontine Syndrome - I/L Paralysis of face (UMN lesion in Cortical stroke - Decrease lacrimation, salivation, Taste in anterior 2/3 of tongue - I/L SNHL, vertigo (supplied by labyrinthine A, a branch of AICA) PICA c/f : Decrease in pain & temperature from C/L body, I/L face - I/L Horner's, I/L ataxia & dysmetria
32
Symptoms of PICA ??
Lateral Medullary/ Wallenberg's - Dysphagia, Hoarseness, Decreased Gag reflex, Hiccups (Nucleus ambigus effects are specific to PICA lesions) AICA c/f - Vomit, Vertigo, Nystagmus- Decrease pain & temp. from C/L body & I/L face - I/L Horner's - I/L Ataxia & Dysmetria
33
Symptoms of Anterior Spinal artery ??
MEDIAL Medullary Syndrome (Paramedian branch of ASA &/or Vertebral artery occlusion) C/L paralysis of ULs & LLs Decreased C/L Proprioception I/L hypoglossal dysfunction (Tongue deviates I/L)
34
Why does Brain Herniation occur ??
Increased ICP - Mass effect (Haemorrhage) - Cerebral edema (Stroke or Trauma) - Hydrocephalus Divided into 2 categories - Supratentorial - Infratentorial
35
Types of Herniation syndromes ??
Cingulate (Subfalcine) H under Falx ceribri - Can compress ACA Central/Downward Transtentorial H - Caudal displacement of Brainstem Uncal Transtentorial H - Uncus= Medial Temporal lobe Cerebellar Tonsillar H into F Magnum - Brainstem compression => Coma & death Upward Cerebellar - Ataxia, Nystagmus
36
Artery involved in Central/Downward Transtentorial Herniation ??
Brainstem Caudal displacement - Rupture of Paramedian Basilar artery branches => DURET Haemorrhages - Decorticate & Decerebrate Posturing - Fatal
37
Features of Uncal Transtentorial H ??
Compress 2 key structures - PCA & CN 3 Early H => I/L Down out Blown pupil (U/L CN3 compression) + C/L Hemiparesis Late H => Coma + Kernohan phenomenon (Misleading C/L Blown pupil + I/L Hemiparesis due to C/L compression against Kernohan notch)
38
39
Causes of the following Headache - Acute Single episode ?? - Chronic Headache ??
Meningitis, Encephalitis SAH, Head injury Sinusitis Glaucoma Tropical illness eg- Malaria Chr. Raised ICP Paget's disease Psychological
40
What is Cluster Headache (CH) ??
Dx. Criteria (ICHD-3) Criterion A: At least 5 attacks fulfilling B to D criterias Criterion B: Severe/ Very severe U/L Orbital, Supraorbital, &/or Temporal Pain lasting 15- 180 min (when not Rx Criterion C: Either or Both of - At least one of the following C/F or Signs, I/L to Headache - Conjunctival injection &/or Lacrimation - Nasal congestion &/or Rhinorrhea - Eyelid Edema - Forehead & Facial Sweating - Miosis &/or Pupils - Sense of Restlessness or Agitation Criterion D: Attacks have a frequency b/w 1 every other day & 8/day Criterion E: Not better accounted for by another ICHD-3 dx.
41
What is Trigeminal Autonomic Cephalalgia ??
Represents a grp. of Primary Headache disorders characterised by - Severe U/L Pain in CN-5 distribution - Accompanied by I/L Autonomic C/F This includes - Cluster Headache - Paroxysmal Hemicrania - Short-Lasting U/L Headache attacks (SUNCT & SUNA) - Hemicrania Continua - Other less common variants
42
Paroxysmal Hemicrania (PH) ??
Criterion A: At least 20 attacks fulfilling criteria B- E Criterion B: Severe U/L Orbital, Supraorbital, or Temporal pain lasting 2- 30 min Criterion C: At least 1 of the following C/F or Signs, I/L to Headache: - Conjunctival Injection &/or Lacrimation - Nasal Congestion &/or Rhinorrhea - Eyelid Edema - Forehead & Facial Sweating - Miosis &/or Ptosis Criterion D: Attack Frequency or > 5/day for ,ore than 1/2 of the time Criterion. E: Complete response to therapeutic INDOMETHACIN doses
43
What is SUNCT & SUNA ??
SUNCT: Short-lasting U/L Neuralgiform headache attacks with Conjunctival injection & Tearing SUNA: Short-lasting U/L Neuralgiform headache attacks with cranial Autonomic symptoms - BOTH involve brief attacks of moderate to severe U/L head pain, typically lasting 1- 600 sec. & occurs at a frequency of at least 1/day
44
Sx. Criteria of SUNCT & SUNA (ICHD-3) ??
A : At least 20 attacks fulfilling B- D B : Moderate or severe U/L Head pain lasting for 1- 600 sec. C : At least 1 of the following C/F or signs, I/L to headache For SUNCT : Conjunctival injection &/or Lacrimation For SUNA: - C injection &/or Lacrimation - Nasal Congestion &/or Rhinorrhoea - Eyelid Edema - Forehead & Facial Sweating - Miosis &/or Ptosis D : Attack Freq.- At least 1/day E : Not better accounted for by another ICHD-3 dx.
45
Dx. Criteria of Hemicrania Continua (HC) ??
A : U/L Headache fulfilling B- D B : Presents for > 3 months, with exacerbations of moderate to severe intensity C : At least 1 of the following C/F & Signs, I/L to headache- - C Injection &/or Lacrimation - Nasal congestion &/or Rhinorrhea - Eyelid Oedema - Forehead & Facial Sweating - Miosis &/or Ptosis D : Complete response to theurapeutic dose of INDOMETHACIN E : Not better accounted by another ICHD-3 dx.
46
Acute Rx. of - Cluster Headache ?? - Paroxysmal Hemicrania ??
1) 1st line: High-flow O2 (12-15l/min) + SC Sumatriptans 2nd line: Intranasal Zolmitriptan & IM Dihydroergotamine 2) ToC: INDOMETHACIN - Celecoxib/ Meloxicam (if 1st line CI)
47
Pathophysiology of Trigeminal Autonomic Cephalgia ??
Dysfunction in the Hypothalamus & its connections with the CN-5-Autonomic reflex pathways - Neuroimaging: Posterior Hypothalamic Gray matter during attacks - Genetic factors, Neuropeptide signalling & Hormonal influences may also play a role.
48
Acute Rx. of - SUNCT or SUNA ?? - Hemicrania Continua ??
1) IV Lidocaine - Lamotrigine & Topiramate (Short-term Rx.) 2) ToC: INDOMETHACIN
49
Preventive Rx of - Cluster Headache ?? - Paroxysmal Hemicrania ??
1) 1st line: VERAPAMIL - Lithium, Topiramate as alternatives Corticosteroids (Short-term prevention during episode) 2) INDOMETHACIN - Alternatives: Gabapentin/ Topiramate
50
Preventive Rx. of - SUNCT & SUNA ?? - Hemicrania Continua ??
1) 1st line: Lamotrigine, Topiramate & Gabapentin - Carbamazepine, Oxcarbamazepine can also be considered 2) INDOMATHACIN - Celecoxib, Meloxicam (if 1st line CI)
51
Dx. Criteria of Migraine without Aura ??
A : At least 5 attacks fulfilling B- D B : Headache for 4- 72 hrs (untreated or unsuccessfully Rx.) - Children: Shorter lasting + more commonly B/L + GI c/f MC C : Headache has >=2 of - U/L location - Pulsating quality - Moderete or Severe pain - Aggravation by/ Causing avoidance of routine physical activity (walking or climbing stairs) D : During Headache at least 1 of - N & V - Photophobia & Phonophobia E : Not attributed to another disorder
52
Features of Migraine ??
3x more common in Women - Prevelence in Men: 6% & Women: 18% Common TRIGGERS - Tiredness, Stress - Alcohol, - COCPs - Lack of food or Dehydration - Cheese, Chocolate, Red wine, Citrus - Menstruation - Bright lights
53
What Aura symptoms prompts a further Ix. or Referral ??
Atypical Aura features like - Motor weakness - Double vision - U/L Visual symptoms - Poor Balance - Decreased level of Consciousness
54
Auras in Migraine ??
M with Aura (25% cases) is easier to Dx.. a TYPICAL aura is - Progressive in nature -Seen Hours prior to Headache - Visual - Transient Hemianopia disturbance (or) Spreading Scintillating Scotoma (Jagged crescent) - Sensory C/F can also occur Auras are seen +/- Headache & - Fully Reversible - Develop over at least 5 min. - Lasts 5- 60 min
55
Dx. of Migraine ??
NICE suggests the following along with the International Headache Society Dx. Criteria - Migraines can be U/L or B/L - NICE gives more details about Typical Auras
56
General rule in the Rx. of Migraine ??
5-HT r (+) : used for Acute Rx. 5-HT r (-) : used for Prophylaxis
57
Acute Rx. of Migraine ??
1st line: Oral Triptan + NSAIDs (or) Oral Triptan + PCM - Age 12- 17yrs: Nasal Triptans (Not Oral) 2nd line: Non-Oral preparation of [Metoclopramide/ Prochlorperazine] +/- [NSAIDs or Triptans] NOTE: Acute Dystonic reaction in Young pts.
58
Prophylaxis of Migraine ??
Given IF >= 2 attacks/ month 1) 1st line: Propranolol or Topiramate Women of Child-bearing age - PROPRANOLOL >> Topiramate (Teratogenic & Reduced Hormonal Contraceptive's effectiveness) 2) 2nd line: course of upto 10 sessions of Acupuncture over 5- 8 wks 3) Riboflavin: Reduces Frequency & Intensity on attacks in some pts.
59
Rx. of Migraine during - Pregnancy ?? - on COCPs ?? - on HRT ??
1) 1st line : PCM 2nd line: NSAIDs (in 1st & 2nd Trimester) - Avoid: Aspirin & Opioids 2) COCPs use is an Absolute CI in pts of M with Aura (Stroke increased risk) 3) SAFE to give HRT for pts. with H/o M but it may worsen the migraines
60
Prophylaxis of Menstrual Migraine ??
Acute: Mefanamic acid or [Aspirin + PCM + Caffeine] Start from 2 days before until 3 days after Bleeding start - Frovatriptan (2.5mg PO BD) - Zolmitriptan (2.5mg PO TDS)
61
Hallmarks of Topiramate ??
MoA- - Blocks Voltage-gated Na+ channels - Increases GABA action - Carbonic Anhydrase (-) : Results in decreased Urine Citrate excretion & forms Alkaline urine & favours CaPO4 stone formation It is a P450 enzyme CYP3A4 inducer - Can cause Hormone Contraceptives being less effective Topiramate + - COCPs & POPs = UKMEC 3 - Implants = UKMEC 2
62
Which type of Contraception are NOT affected by Topiramate ??
Depo-Provera Injection Intrauterine System Inducer of P450 enzyme CYP3A4
63
S/E of Topiramate ??
Reduced appetite & Wt. loss Dizziness Paraesthesia Lethargy & Poor conc. Rare: Acute Myopia & 2ndary Angle Closure Glaucoma Teratogenic (malformations)
64
Hallmarks of Medication Overuse Headache ??
One of the MCC of Chr. Daily Headache - Present for >= 15 days/ month - Developed or Worsened while taking regular symptomatic Rx. - Pts. on OPIOIDS & TRIPTANS are at more risk - May be a Psychiatric Co-morbidity
65
Rx. of Medication Overuse Headache ??
ABRUPT withdrawal of - Simple Analgesics & Triptans (may initially worsen headache) OPIOID Analgesics withdrawn gradually Withdrawal C/F can occur - N & V, Hypotension, Tachycardia, Restlessness, Sleep disturbances & Anxiety
66
Triptans hallmarks ??
AGONISTS of 5-HT1B & 5-HT1D - Acute Rx. of Migraine - Taken asap AFTER headache starts & NOT after Aura starts S/E - Triptan Sensations: Tingling, heat, Tightness (throat, chest), Heaviness, Pressure CI: IHD or Cerebrovascular Disease - (Previous H/o or At Risk pts.)
67
Hallmarks of Trigeminal Neuralgia ??
U/L severe pain over the distribution of CN-5 - MCC is IDIOPATHIC - Compression of Trigeminal roots by tumour or Vascular probs may occur
68
What are the Red Flag C/F which suggests a serious underlying cause in Trigeminal Neuralgia ??
- Sensory changes - Deafness or Other ear problems - H/o Skin or Oral lesions that could spread Perineurally - Pain ONLY in the Ophthalmic division of Trigeminal nerve (Eye socket, Nose & Forehead) or B/L - Optic Neuritis - FHx of Multiple Sclerosis - Age of Onset before 40 yrs
69
Definition of Trigeminal Neuralgia ??
U/L disorder characterised by brief Shock-like pain, abrupt in onset & termination, limited to >- 1 division of Trigeminal nerve - Pain is evoked by Light touch (Washing, shaving, talking, brushing of teeth) & frequently occurs spontaneously - Trigger area: Small areas in Naso-Labial fold or chin may be particularly susceptible to the ppt. of pain - Pain usually remits for variable period
70
Rx. of Trigeminal Neuralgia ??
1st line: CARBAMAZEPINE Refer to Neurology if failure to respond to Rx. or Atypical features (< 50yrs old)
71
Hallmarks of Post-Lumbar Puncture Headache ??
Seen in 1/3rd pts. Due to leak of CSF following Dural puncture - MC in Young Females + Low BMI Factors that Contribute to Headache - Increased Needle size - Direction of Bevel - Not placing the Stylet - Increased no. of LP attempts
72
Hallmarks of Pituitary Apoplexy ??
Sudden enlargement of Pituitary tumour (usually Non-Functioning Macroadenoma) - 2ndary to Bleeding or Infarction Precipitating Factors - HTN, - Pregnancy - Trauma, - Anticoagulation Ix.oC : MRI is diagnostic
73
Typical features & Rx. of Post-LP Headache ??
Develops within 24-48hrs after LP but can occur up to 1 wk later Can last for Several days Worsens with Upright position Improves with Recumbent position Rx.- - Initially Supportive: Analgesia, Rest If pain for > 72hrs: Specific Rx. is indicated to prevent Sub-Dural H - Blood patch, - Epidural saline & IV Caffeine
74
What factors do NOT contribute to Post LP headache ??
Increased volume of CSF removed Bed rest following procedures Increased fluid intake post LP Opening pressure of CSF Position of pt.
75
Features of Parietal lobe lesion ??
Parietal Lobe lesion - Sensory Inattention, - Apraxias - Astereognosis (Tactile agnosia) - Inferior Homo. Quadrantanopia - Gerstmann's Syndrome (Dominant P lobe lesion) : Alexia Acalculia, Finger agnosia & Rt.-Lt. Disorientation
76
Features & Rx. of Pituitary Apoplexy ??
Sudden onset Headache (similar to the one seen in SAH) Vomiting Neck Stiffness Extraocular Nerve Palsy Bitemporal Superior Quadrantanopia Features of Pituitary Insufficiency - Hypotension, HypoNa+ (2ndary to Hypoadrenalism)
77
Rx. of P Apoplexy ??
IoC : MRI is diagnostic Urgent Steroid replacement (due to ACTH loss) Careful Fluid balance Surgery
78
Features of Occipital Lobe lesions ??
Homo. Hemianopia with Macular sparing Cortical Blindness Visual Agnosia
79
Features of Temporal lobe lesions ??
1) Wernicke's Aphasia - Forms speech before sending it to Brocas ares - Lesion: Substitution, Neologism, FLUENT Speech 2) Superior Homo. Quadrantanopia 3) Auditory Agnosia 4) Prosopagnosia (difficulty recognising face)
80
Features of Frontal lobe lesions ??
Brocas Aphasia (Expressive) - Site: Posterior aspect of FL in the Inferior gyrus - Broken speech, laboured & halting Disinhibition Perversed Anosmia Inability to generate a list
81
Features of Cerebellum lesions ??
Midline lesion: Gait & Truncal Ataxia Hemisphere Lesions - Intention tremor, - Nystagmus - Past-pointing - Dysdiadokinesis
82
Kluver-Bucy Syndrome ??
Hypersexuality, Hyperorality, Hyperphagia, Visual Agnosia - AMYGDALA is affected
83
What are the Cerebral areas affected in - Hemiballismus ?? - Huntington's chorea ?? - Parkinson's ?? - Wernicke & Korsakoff syndrome ?? - Kluver-Bucy Synd. ??
- Subthalamic nucleus of BG - Striatum (Caudate N) of BG - Substantia nigra Pars compacta of BG - Medial Thalamus & Mammillary bodies of HYPOTHALAMUS - Amygdala
84
Normal CSF values ??
Pressure : 10-18 cmH2O (Recumbent) Protein : 0.2- 0.4 g/l Glucose : > 2/3rd BG Cells : RBCs- 0, WBC- < 5/mm3
85
Conditions where Lymphocytes are raised in CSF ??
Viral Meningitis/ Encephalitis TB meningitis Partially treated Bacterial Meningitis Lyme's disease Behcet's SLE Lymphoma, Leukaemia
86
CSF with raised proteins are seen in ??
GBS TB, Fungal & Bacterial Meningitis Viral Encephalitis Froin's Syndrome - Increase in CSF protein below a Spinal canal blockage - eg.- Tumour, Disc, Infection
87
Complications of Meningitis ??
1) SNHL (MC sequelae) 2) Seizures 3) FND 4) Infective: Sepsis Intracerebral Abscess 5) Pressure: - Brain herniation - Hydrocephalus 6) Meningococcal Meningitis are at risk of Waterhouse-Fridrichsen Synd. (Adrenal insufficiency 2ndary to Adrenal haemorrhage)
88
Ix. done in Anti-NMDA Receptor Encephalitis ??
1) MRI can be normal but - FLAIR sequence: Deep Subcortical Limbic Str. abnormalities 2) CSF (Normal Initially) - Later: Pleocytosis 3) Anti-MuSK autoantibodies - No Thymoma & No Antibodies against ACh receptors 4) Anti-GM1 autoantibodies - Specific for Acute Inflam. Demyelinating Polyneuropathy (AIDP) variant of GBS
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Hallmarks of Anti-NMDA Receptor Encephalitis ??
PARANEOPLASTIC Syndrome Features- Prominent Psychiatric C/F - Agitation, Hallucinations - Delusions, Disordered thinking Seizures, Insomnia, Dyskinesia Autonomic instability
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Rx. of Anti-NMDA Receptor Encephalitis ??
Immunosuppression - Steroids, IGs, Cyclophosphamide, Rituximab alone or in combination Plasmapheresis Resection of Teratoma is also therapeutic
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Which tumour is Anti-NMDA Receptor Encephalitis a/w ??
Ovarian Teratomes - Detected in upto 1/2 of ALL Female adult pts. More prevalent in AFRO-CARIBBEANS
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Hallmarks of Acute Disseminated Encephalomyelitis (ADEM) ??
aka Post-Infectious Encephalomyelitis Autoimmune DEMYELINATING disease of the CNS Cause : Occurs after Bacterial or Viral pathogen - Measles, Mumps, Rubella & Varicella + others Ix.- MRI (Supra- & Infra- Tentorial Demyelination) Rx.- - IV Glucocorticoids - IVIG if this fails
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Features of Post-Infectious Encephalomyelitis or ADEM ??
After a gap of Few days to 2 months, there is an Acute onset Multifocal CNS symptom with RAPID deterioration - Headache, Fever, N & V Motor + Sensory deficits Brainstem involvement including Oculomotor defects
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Imaging features of - Anti-NMDA Receptor Encephalitis ?? - ADEM or Post-Infectious Encephalomyelitis ??
1) MRI- FLAIR sequence - Deep Subcortical Limbic Str. abnormalities - Plain MRI head may be normal 2) MRI - Supra- & Infra- Tentorial Demyelination
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Hallmarks of Brain abscess ??
Causes - Extension of Sepsis from middle ear or sinuses, trauma or surgery to scalp Penetrating head injury & Embolic events from Endocarditis Ix.- Pt assessment & CT Scan Rx.- 1) Surgery (Craniotomy + Abscess cavity debridement) - Abscess can reform as because the head is closed after abscess drainage 2) IV 3rd Gen. Cephalosporins + Metronidazole 3) ICP increased: Dexamethasone
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Features of Brain abscess ??
Presenting features depends on the site of abscess - Those in critical areas (eg- Motor complex) will present earlier - Headache (dull, persistent) - Fever (NOT Swinging pyrexia as seen with abscesses at other sites) 2ndary to raised ICP (Mass effect) - FND - Nausea, Papilloedema, Seizures
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Hallmarks of Creutzfeldt-Jakob Disease ??
RAPID Progressive CNS condition caused by PRION Proteins - These proteins induce the formation of Amyloid Folds resulting in tightly packed Beta-pleated sheets resistant to Proteases Ix.- - CSF usually normal - EEG: Biphasic, High Amplitude sharp waves (only in Sporadic CJD) - MRI: Hyperintense signals in BG & Thalamus
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Sporadic CJD features ??
85% cases & 10-15% are Familial Mean age onset is 65yrs EEG: Biphasic, High amplitude sharp waves MRI: Hyperintense signals in BG & Thalamus
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New Variant CJD ??
Young pts. (Average age of onset 25 yrs) Psychological C/F: (MC) - Anxiety, Withdrawal & Dysponea - Prion protein is encoded on Chr.20 Methionine Homozygosity at codon 129 of prion protein is a risk factor for developing CJD- Median survival of 13 months
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Name other Prion diseases ??
Kuru Fatal Familial Insomnia Gerstmann Straussler-Scheinker disease
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Gold features of CJD ??
Sporadic - Old age + Rapid Progressive Dementia + Myoclonus - EEG: Biphasic, High amplitude sharp waves New Variant CJD - Young Age (25 yrs) + Psychological C/F (Anxiety, Withdrawal, Dysphonia)
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What is CADASIL ??
Cerebral A D Arteriopathy with Subcortical Infarcts & Leukoencephalopathy - Rare cause of Multi-Infarct DEMENTIA - Pt. often presents with Migraine
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Classification of Aphasia ??
Speech is FLUENT If Comprehension relatively Intact - Conduction Aphasia If Comprehension Impaired - Wernicke's Aphasia Speech is NOT Fluent If Comprehension relatively Intact - Broca's Aphasia If Comprehension Impaired - Global Aphasia
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Wernicke's Aphasia ??
RECEPTIVE Aphasia ['Forms' speech] Lesion: Superior Temporal Gyrus B Supply: Left MCA (Inferior branch) - Forms speech before sending it to Broca's area C/F: Sentences make NO Sense, Word substitution & Neologism but FLUENT speech 'Word salad' - Comprehension IMPAIRED
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Broca's Aphasia ??
EXPRESSIVE Aphasia Lesion: Inferior Temporal Gyrus B Supply: Left MCA (Superior Branch) - Broken speech (not fluent), Halting, Laboured - Repetition is IMPAIRED - Comprehension is NORMAL
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Conduction Aphasia ??
Stroke affecting Arcuate Fasiculus Connects Wernicke's => Broca's - Speech is FLUENT - Repetition is POOR - Aware of the errors they make - Comprehension is NORMAL
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Global Aphasia ??
Lesion affecting ALL areas ie. - Wernicke's, A Fasiculus & Broca's - Severe Receptive & Expressive Aphasia - Able to communicate using Gestures
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Hallmarks of West's Syndrome or Infantile Spasms ??
Brief spasms beginning in the 1st few months of life - Salaam attack: Flexion of Head, Trunk, limbs; lasts for 1-2 sec, repeats upto 50 times - Progressive Mental handicap - EEG: Hypsarrhythmia Causes- Idiopathic 2ndary to: Serious CNS probs (T Sclerosis, Encephalitis, Birth asphyxia)
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Lennox Gastaut Syndrome ??
May be an Extension of West's Synd. Onset: 1- 5 yrs Features- - Atypical absences, falls, jerks - 90% Moderate-Severe Mental Handicap - EEG: Slow Spikes
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Absence Seizures ??
Form of Generalised Epilepsy - MC in Children (3- 10 yrs old) - Girls >> Boys Features - Absences last for few- 30 sec. - No warning + Quick Recovery - Provoked by Hyperventilation or Stress - Child UNAWARE of seizures - Occur Multiple times in a day EEG: B/L Symmetrical 3Hz Spike & Wave pattern
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Benign Rolandic Seizures ??
Age: 4- 12 yrs Occurs at Night or On waking up Seizures are Partial (eg Paraesthesia affecting Face) Secondary generalization may occur - Tonic-Clonic movt. EEG: Centro-Temporal Spikes
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Janz Syndrome or Juvenile Myoclonic Epilepsy ??
Typical in Teenage yrs, MC in Girls Features - Infrequent generalised seizures - Often in Morning/ after Sleep deprivation - Daytime Absences - Sudden, shock-like seizures (may develop before seizures)
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Classifications of Seizures ??
1) FOCAL Onset Seizures - Motor - Non-Motor 2) GENERALIZED Onset Seizures Motor - Tonic-Clonic (Grand Mal - Tonic, - Clonic, - Atonic Non-Motor Typical Absence 3) FOCAL Onset to B/L Seizure - Starts on 1 side of brain (Specific area) before spreading to Both lobes - Secondary Generalised Seizures 4) UNKNOWN Onset Seizures - Term reserved wen the origin of seizure is unknown
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On what basis are Seizures Classified ??
Is based on 3 key features - Where seizures begin in brain - Level of Awareness during Seizures - Other features of Seizures
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Generalised Onset Seizures ??
These engage or involve networks on both sides of brain at the Onset - All pts LOSE Consciousness immediately Divided into - Motor: Tonic-Clinic (Grand-Mal), Tonic, Clonic, Atonic - Non-Motor Typical Absence (Petit Mal)
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Focal onset Seizures ??
Focal Onset Seizures Starts at specific area on 1 side of brain; Can be further divided into - Motor: eg- Jacksonian March - Non-Motor: eg- Deja vu, Jamais vu Having other features such as Aura The pt. may be - Aware or Impaired Awareness or Awareness Unknown
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Rx. & EEG patterns of - - West's Syndrome ?? - Lennox Gastaut Synd. ?? - Absence Seizures ?? - Benign Rolandic Seizures ?? - Janz Syndrome ??
1) Rx.- Vigabatrin & Steroids - EEG: Hypsarrhythmia 2) Rx.- Ketogenic diet may help - EEG: Slow Spike 3) Rx.- Na Valproate & Ethosuximide - Good Prognosis: 90-95% are seizyre free by adolescence - EEG: B/L Symmetrical 3Hz Spike & Wave pattern 4) Rx.- Excellent Prognosis, stops by Adolescence - EEG: Centro-Temporal Spike 5) Rx.- Na Valproate
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How to manage Seizures in Pregnancy & Breastfeeding ??
1) Folic Acid 5mg/ day well before conception to minimise NTDs 2) Aim for MONOTHERAPY 3) Least Teratogenic among the Older Antiepileptics : CARBAMAZEPINE 4) Newer agents: LAMOTRIGINE - Dose needs to be Increased 5) Breastfeeding is safe (except with Barbiturates) 6) Pregnant + on Phenytoin - - Vit. K at last month of POG to prevent clotting probs. in foetus
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How to Localise features of Focal Seizures ??
TEMPORAL Lobe ("HEAD") - Hallucinations (Auditory/Gustatory/ Olfactory), - Epigastric rising or Emotional - Automatism (Lip smacking/Grabbing or Plucking) - Deja vu or Dysphasia post-ictal FRONTAL Lobe (Motor) - Head/Leg Movt., Posturing - Post-Ictal Weakness, Jacksonian march
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How to localise features of Focal Seizures ??
PARIETAL Lobe (Sensory) - Paraesthesia OCCIPITAL Lobe (Visual) - Floaters or Flashes
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Rx. of Absence Seizures
C/F: Lip Smaking, Involuntary movt. of Hands, Fluttering of Eyelids 1st line: ETHOSUXIMIDE 2nd Line - Males: Sodium Valproate - Females: Lamotri. or Levetira. Carbamezepine may WORSEN absence seizures
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Rx. of - - Focal Seizures ?? - GTCS ??
1) 1st line: Lamatrigine/ Levitiracetam - 2nd line: Carbamazepine/ Oxcarbamezepine/ Zonisamide 2) Males: Na Valproate - Females: Lamotrigine/ Levetiracetam - Girls < 10yrs + Unlikely to need Rx when old enough (OR) Women who are unable to have Kids : Offer SODIUM VALPROATE as 1st line
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Rx. strategy in Epilepsy ??
Rx. is started following SECOND Epileptic Seizure Start Anti-Epileptics after the 1st episode in the following- - FND - Structural abnormalities on Brain Scans - EEG shows definite Epileptic waves Pt. or their Family or Carers consider the risk of having a further seizure unacceptable
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Rx. of - - Myoclonic Seizures ?? - Tonic or Atonic Seizures ?? - Partial Seizures ??
1) Males: Na Valproate - Females: Levetiracetam 2) Males: Na Valproate - Females: Lamotrigine 3) 1st line: Carbamazipine
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What is Status Epilepticus ??
Single seizure for > 5min (OR) >= 2 seizures within a 5 min. period without the person returning to normal b/w them
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Rx. of Status Epilepticus ??
Medical Emergency 1) ABC: Airway adjunct, O2, Check BG 2) 1st line: IV BZPs (Dia-/ Lora- zepam) - Prehospital setting: PR Diazepam or Buccal Midazolam - In Hospital: IV Lorazepam, can be repeated once after 10-20 min 3) If Ongoing/ 'Established' Status start- 2nd line: Phenytoin/ Phenobarbital Infusion 4) If NO Response/ 'Refractory' Status within 45 min of Onset - GA Induction
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Hallmarks of Sodium Valproate ??
Works by Increasing GABA activity S/E - Teratogenic - Neurodevt. delay
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Hallmarks of Ethosuximide ??
DoC in Absence seizures MoA: Blocks T-type Ca2+ channels in Thalamic neurons
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Hallmarks of Phenytoin ??
MoA: Binds to Na+ channels Increasing their Refractory period - P450 enzyme Inducer Trough levels needs to be checked immediately before dose should be checked if - Adjustment of Phenytoin dose - Suspected Toxicity - Detection of Non-Adherence
130
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S/E of Phenytoin ??
ACUTE - Initially: Dizziness, Diplopia, Nystagmus, Slurred speech, Ataxia - Later: Confusion, Seizures CHRONIC - Common: Gingival Hyperplasia (2ndary to increased PDGF expression), Hirsutism, Coarse Facial features, Drowsiness - Megaloblastic anaemia - Peripheral Neurapathy - LNpathy, - Dyskinesia - Enhanced Vit.D metabolism causing Osteomalacia
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What are the Idiosyncrtic & Teratogenic effects of PHENYTOIN ??
IDIOSYNCRATIC - Fever, - Hepatitis - Dupuytren's contracturs - Aplastic anaemia - Drug-induced Lupus - Rashes (including TEN) TERATOGENIC - Cleft Palate & Congenital H Disease
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Hallmarks of Carbamazepine ??
Chemically similar to TCAs MoA: Binds to Na+ channels => Increase Refractory period Uses: Trigeminal N & Bipolar disease Exhibits Autoinduction
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S/E of Carbamazepine ??
P450 inducer Dizziness, Ataxia Drowsiness, Headache Visual disturbances (DIPLOPIA mc) SJS LEUCOPENIA & Agranulocytosis Hypo[Na+]: 2ndary to SIADH
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Autoinduction in Carbamazepine ??
When a pt. starts Carbamazepine, they may see a return of seizures after 3- 4 wks of Rx.
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Hallmarks of Lamotrigine ??
MoA: Na+ channel Blockers S/E: SJS
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Hallmark of Vigabatrin ??
40% pts. develop visual field defect which may be IRREVERSIBLE - Check Visual Fields every 6 months
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Hallmarks of Parkinson's Disease ??
Progressive Neurodegenerative condition caused by degeneration of DA Neurons in S Nigra Triad: Bradykinesia, Tremors, Rigidity Symptoms are ASYMMETRICAL - 2x more common in MEN - Mean age of Dx.- 65 yrs
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Triad of Parkinson's Disease ??
BRADYKINESIA (Hypokinesia) - Poverty of movt. - Short, shuffling steps with reduced arm swinging - Difficulty to INITIATE movt. TREMORS - Most marked at Rest, 3- 5 Hz - Worse: Stressed or Tired - Relieves: VOLUNTARY movt. - 'Pill-Rolling' (Thumb & Index finger) RIGIDITY - Lead-Pipe - COGWHEEL: Rigidity superimposed by Tremors
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Features of Parkinson's ??
Mask-like facies Flexed posture Micrographia Drooling of Saliva Psychiatric C/F:DEPRESSION (mc 40%) - Dementia, Sleep disturbance Impaired Olfaction REM Sleep behaviour disorder Fatigue Autonomic: Postural C/F
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Features of Drug induced Parkinson's ??
MOTOR c/f are geberally - RAPID onset - B/L onset - Rigidity & Tremors are Uncommon
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Dx. of Parkinson's Disease ??
Dx. is usually Clinical - Difficult to differentiate b/w Essential tremor & Parkinson's [(123)I-FP-CIT] SPECT - Discolouration of S nigra due to loss of pigmented nerve cells
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Parkinson's disease Medications ??
TIME_Critical Acute Akinesia or Neuroleptic Malignant Synd. is high if- - NOT taken on time (OR) - Not absorbed properly (eg.- GE) Never have 'Drug Holiday' IMPULSE CONTROL Disorders This can occur with any DA therapy but is more common with - DA Agonist Rx. - H/o Previous impulsive behaviour - H/o [-OH] intake or Smoking
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What are the common problems faced in Parkinson's Rx & how to tackle them ??
EXCESSIVE Sleepiness - If in daytime: Avoid Driving - Use Modafinil if alternative strategy fails (eg.- adjusting dose) ORTHOSTATIC Hypotension - Review medications - MIDODRINE if c/f persists DROOLING of Saliva - Glycopyrronium Bromide
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MoA of Midodrine ??
Used in Orthostatic Hypotension due to Parkinson's disease - Acts on Peripheral Alpha-adrenergic receptors => Increase Arterial resistance
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Rx. of Parkinson's ??
Should ONLY be Dx. & Rx. Initiated by a SPECIALIST 1st Line Rx.- - Motor C/F affecting quality of life: LEVODOPA - Motor C/F NOT affecting quality of life: DA agonist (Non-Ergot derived), Levodopa, MAO-B i Excessive Sleepiness, Hallucinations & Impulse Control Disorder
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Rx. when pt. is symptomatic even after starting optimal Levodapa Rx. or has developed Dyskinesia ??
add DA agonist, MAO-B i or COMT i as an adjunct
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Hallmarks of Levodopa ??
Almost always combined with - Decarboxylase (-): Carbidopa or Benserazide - This prevents peripheral metabolism of Levodopa to DA outside of brain => Reduces S/E S/E : Dry mouth, Anorexia, Psychosis Palpitations, Postural Hypotension
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Hallmarks of DA r agonists ??
Ergot derived: Bromocriptine, Cabergoline Ropinirole, Apomorphine Prior to Rx. obtain - Echo, CXR, ESR, Cr. - Pts. should be closely monitored
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S/E seen due to difficulty in achieving a Steady dose of Levodopa ??
1) End-of-dose wearing off : C/F worsen towards the end of dosage interval => Decline in Motor activity 2) 'On-off' Phenomenon: Large variation in Motor performance, - 'On': Normal function - 'Off': Weakness & restricted mobility 3) Dyskinesia at Peak dose - Dystonia, Chorea, Athetosis (involuntary writhing movt.) 4) Do NOT acutely Stop LD - If can't take drug PO, give a DA agonist patch (Rotigotine patch) as Rescue drug to prevent Acute DYSTONIA
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S/E of DA r agonists ??
- Impulse Control Disorder - Excessive daytime Somnolence - Hallucinations in Older pts. (more common than in Levodapa) - Nasal Congestion & Postural Hypotension
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MoA of - - MAO-B inhibitors ?? - Amantadine ??
1) (-) breakdown of DA secreted by Dopaminergic neurons - Selegiline, Rasageline 2) Increases DA release & (-) its uptake at DA synapses - S/E: Ataxia, Slurred speech, Livido reticularis, Confusion, dizziness,
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MoA of Catecho-O-Methyl Transferase inhibitor (COMT) inhibitors ??
COMT is an enzyme involved in the breakdown of DA - Used in conjunction with Levodopa in pts. with established PD - Entacapone, Tolcapone
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Hallmarks of Steele-Richardson-Olszewski syndrome ??
Progressive Supranuclear Palsy - A 'Parkinson Plus' syndrome Features - Postural instability & Falls - Stiff Broad-based gait - IMPAIRED Vertical gaze (Downgaze worse than Up gaze- Difficulty reading or descending stairs) - Parkinsonism: BRADYKINESIA is prominent - COGNITIVE Impairment (Primary FRONTAL Lobe dysfunction) Rx.- Poor response to L-dopa
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Anti-Muscurinics in PD ??
Blockes Cholinergic r - used in Drug-induced Parkinsonism rather than Idiopathic PD - Helps tremor & rigidity Eg: Procyclidine, Benzotropine, Trihexyphenidyl (Benzhexol)
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Levodapa S/E ??
Usually given with Decarboxylase i (Carbidopa or Benserazide) Reduced effectiveness with time S/E - Reddish discolouration of Urine upon Standing - Psychosis - Dyskinesia - 'On-off' effect, - N & V - Postural Hypotension - Cardiac arrhythmias
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Causes of Parkinsonism ??
PD Drug induced: Anti-Psychotics, Metoclopramide - Domperidone dose not cross BBB, so Extra-Pyramidal S/E NOT seen Progressive Supranuclear Palsy Multiple System Atrophy Wilson's disease Post-Encephalitis Dementia Pugilistica (2ndary to chronic head trauma eg.- Boxing) Toxins: Carbon Monoxide, MPTM
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C/F of Huntington's Disease ??
Typically develops after 35yrs of age - Chorea - Personality changes: Irritabile, Apathy, Depression - Intellectual impairment - Dystonia - Saccadic eye movements Ventricles are enlarged
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Hallmarks of Multiple System Atrophy ??
Two types of MSA MSA-P: Parkinsonian predominant c/f MSA-C : Cerebellar predominant C/F Shy-Drager Synd. is a type of MSA C/F Parkinsonism Autonomic disturbance - Erectile dysfunction: EARLY c/f - Postural Hypotension - Atonic Bladder Cerebellar signs
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Hallmarks of Huntington's Disease ??
Inherited A D Neurodegenerative condition - CAG repeat disorder - Defect in Huntingtin gene on Chr. 4 - Anticipation: Disease presents at an earlier age in successive generations Causes Degeneration of Cholinergic & GABAergic neurons in STRIATUM of Basal Ganglia
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Causes of Chorea ??
Huntington's, Wilson's, Ataxia Telangiectasia SLE, APLS RF: Sydenham's Chorea OCPs, L-dopa, Antypsychotics NeuroAcanthocytosis Pregnancy: Chorea gravidarum Thyrotoxicosis Polycythaemia Rubra vera Carbon Monoxide Poisoning Cerebrovascular accident
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What is Chorea ??
Caused by damage to the - BG specially Caudate nucleus Involuntary, rapid, jerky, movt. which often move from one part of the body to another ATHETOSIS: Slower, sinuous movt. of the limbs
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What is Hemiballism ??
Occurs following damage to the Subthalamic Nucleus - Ballistic movt. are Involuntary, sudden, jerking movt. - Seen C/L to the side of lesion Primarily affects PROXIMAL Limb musculature & Distal muscles may display more Choreiform like movt.
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Rx. of Hemiballism ??
Symptoms may decrease in Sleep Anti-DA agents - HALOPERIDOL
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Features of Neuroleptic M S ??
Seen within Hrs- Days of starting Anti-Psychotics - Pyrexia - Muscle Rigidity - Autonomic lability: HTN, Tachycardia & Tachypnoea - Agitated Delirium with Confusion - Normal pupils Raised CK is common => 2ndary AKI Leukocytosis
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What is Neuroleptic Malignant Syndrome ??
Seen in pts. on ANTI-PSYCHOTICS - Can also occur in Atypical Anti-P - Seen in PD when L-dopa is suddenly stopped or dose reduced Anti-Psychotics => DA blockade => triggers Massive release of Glutamate => Neurotoxicity & Muscle damage
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Rx. of Neuroleptic M S
1) STOP Antipsychotics 2) Transfer to Medical ward if in Psychiatric ward 3) IV Fluid to prevent Renal failure 4) Dantrolene (selected cases) - Binds to Ryanodine r => Decreases Ca2+ release from S reticulum => decreases excitation-contraction coupling in skeletal muscles 5) Bromocriptine, DA agonist may be used
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When should we do a CT Head within 8 hrs of Head injury ??
Any Adult who have experienced some Loss of Conscious or Amnesia since the injury with the following - >= 65 yrs old - H/o Bleeding or Clotting disorders including Anti-Coagulants - Dangerous mechanism of injury (pedestrian or cyclist hit by motor vehicle, an occupant ejected by a motor vehicle or a Fall from a height > 1 mt. or 5 stairs - > 30 min of Retrograde amnesia of events immediately before head injury On Warfarin + Head injury + NO other indications for a CT = Do CT head within 8 hrs of injury
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Conditions where CT head is indicated immediately after Head injury ??
GCS < 13 on initial assessment GCS < 15 at 2 hrs post-injury Suspected Open or Depressed skull # Signs of Basal Skull # : Panda eyes, Haemotympanum, CSF leak from Ear or Nose, Battle's sign Post-traumatic seizures FND > 1 episode of vomiting
171
Types of Traumatic Brain injury ??
Primary Brain Injury - Focal (Contusion/ Haematoma) - Diffuse (diffuse axonal injury) Diffuse Axonal Injury - Due to Mechanical Shearing following deceleration, leads to tear & disruption of Axons INTRA-CRANIAL Haematoma's - Extradural/ Subdural/ Intracerebral CONTUSION: Seen adjacent to Coup or C/L (Contre-coup) to the side of impact SECONDARY Brain Injury: Cerebral Oedema, Ischaemia, Infection, Tonsillar or Tentorial herniation exacerbates original injury
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MC site of Chr. Sub-Dural Haematoma ??
Frontal & Parietal lobes Rupture of Small Bridging VEINS in the SD space RF: Old age, Chr. [-OH] => Brain atrophied => Fragile or Taut bridging veins & Anticoagulation
173
Cushing's Reflex ??
HTN + Bradycardia, often occurs late & is usually a Pre-Terminal event
174
MC site of Epidural Haematoma ??
Temporal region - Skull # cause rupture of MIDDLE MENINGEAL Artery - Lucid Interval is experienced
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Acute Subdural Haematoma ??
Fresh blood collection in SD space - MCC: High-impact Trauma => often a/w underlying brain injury C/F Severity depends on - Size of compressive clot - Associated injuries Presentation: Incidental finding in trauma to severe coma & Coning due to herniation Large Acute SDH => Mass effect => Mid-line shift or Herniation
176
CT Scan of- - Acute SD Haematoma ?? - Chronic SDH ?? - SAH ??
1) Crescentric collection NOT limited by Suture lines - Appears Hyperdense in comparision to brain 2) Very similar to Acute SDH findings BUT- are Hypodense (dark) compared to brain substance 3) Acute blood (Hyperdense) spread in the Basal Cisterns, Sulci & in severe cases- Ventricular system
177
Rx. of Acute SDH ??
Small or Incidental Acute SDH - Observed conservatively Sx. options - Monitor ICP - Decompressive Cranieotomy
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Hallmarks of Chr. SDH ??
Blood collection in SD space for wks. to months - Rupture of Small BRIDGING Veins in the SD space Several week to months Progressive H/o Confusion or Reduced Consciousness or FND
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Hallmarks of SAH ??
Intracranial bleed: presence of blood within the SA space, ie., deep to the SA layer of meninges - MCC: Head Injury called Traumatic SAH - In the absence of Trauma it is called Spontaneous SAH
180
Rx. of Chronic SDH ??
Incidental finding or Small size + Not a/w CNS deficits - Conservative Rx. Pt. confused + a/w FND or Severe Imaging findings - Surgical Decompression with Burr hole
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Causes of SAH ??
1) Intracranial Aneurysm (Saccular Berry Aneurysm) : 85% cases Conditions a/w Berry aneurysm are - APKD, Ehlers-Danlos, Coarctation of Aorta - May be 2ndary to Autonomic Neural stimulation from Hypothalamus (OR) 2) Elevated levels of circulating Catecholamines 3) AV Malformations 4) Pituitary Apoplexy 5) Arterial dissection 6) Mycotic (Infective) aneurysm 7) Perimesencephalic (Idiopathic venous bleed)
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Complications of SAH ??
Re-Bleeding (70% Mortality rate) - In 10% cases & MC in first 12 hrs - If suspected: Repeat CT Head Vasospasm aka Delayed Cerebral Ischaemia : 7- 14 days after onset HYPO[Na+] : MC due to SIADH Seizures Hydrocephalus Death
183
Ix. done in SAH ??
CT head - Hyperdense at Basal cisterns, Sulci & in Severe cases- Ventricular system - (-)ve in 7% cases 2) LP [Used to confirm when CT (-)ve] - Performed >= 12hrs after C/F onset as this allows Xanthochromia to set in - Xanthochromia helps to distinguish b/w True SAH from Traumatic tap (Blood introduced by LP itself) - Normal/ Raised Opening pressure 3) Refer to NeuroSx. as soon as SAH is confirmed 4) CT intracranial Angiogram (finds the vascular lesion) +/- Digital Subtraction Angiogram (Catheter Angiogram)
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Rx. of SAH ??
1) Spontaneous SAH - Treat the cause 2) Intracranial Aneurysm needs Rx. due to risk of Rebleeding - Maj. cases : Coiling (Interventional Neuroradiologist) - Minority cases: Craniotomy + Clipping by a Neurosurgeon - Until Aneurysm treated, pt. should be in Strict Bed-rest, well-controlled BP & avoid straining 3) Hydrocephalus - External V drain (temporary) - VP Shunt (Long term)
185
Features of SAH ??
Thunderclap or Baseball bat or Worst of my life HEADACHE - Sudden onset, Severe, OCCIPITAL N & V Meningism (Photophobia, Neck stiffness) Seizures, Coma, Sudden Death ECG: ST elevation
186
Drug Rx. in SAH ??
To prevent Vasospasm, - NIMODIPINE for 21 days - CCBs which targets brain vessels Treated with Hypervolaemia, Induced HTN & Haemodilution
187
What are the important Predictive factors of SAH ??
Conscious level on Admission Age Amount of Blood seen on CT Head
188
Hallmarks of Intracranial Venous Thrombosis ??
Can cause Cerebral Infarction (much less common than Arterial causes) Types - Isolated Sagittal Sinus Thromboses (50% cases) - Coexistent Lateral Sinus + Cavernous Sinus thromboses Features - Headache (may be sudden in onset) - Reduced Consciousness - N & V
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Ix. and Rx. of IC Venous Thrombosis ??
Gold Std. Ix.- MRI Venogram - CT Venogram (alternative) NCCT is normal in 70% cases D-Dimer may be elevated Rx.- Anti-Coagulation - Acute AC : LMWH - Long term AC : Warfarin
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What is Sagittal Sinus Thrombosis ??
1) Present with Seizures + Hemiplegia 2) Parasagittal biparietal or Bifrontal Haemorrhagic infarction may be seen 3) Venography: EMPTY DELTA Sign 4) CECT: Empty DELTA Sign (look for triangular shaped dural sinus, this should be filled with Contrast => Thrombus fails to enhance & is outlined by Enhanced Collateral channels in Falx
191
Lateral Sinus Thrombosis ??
CN- 6 & 7 palsy
192
Hallmarks of Cavernous Sinus Thrombosis ??
Causes : Local infection (eg Sinusitis), Neoplasia, Trauma - Peri-Orbital Edema - Ophthalmoplegia: CN-6 damage before CN- 3 & 4 - CN-5 involvement: Hyperaesethesia of Upper face & Eye pain Central Retinal Vein Thrombosis
193
Idiopathic IC HTN or Pseudotumour Cerebri ??
Formerly called Benign IC HTN Risk Factors - Obesity - Female Sex, - Young - Pregnancy Drugs: COCPs, Steroids, Tetracyclines, Vit. A, Lithium Features - Headache, Blurred Vision - Papilloedema - Enlarged Blindspot - CN-6 plasy
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Rx, of Pseudotumour Cerebri ??
1) Wt. Loss 2) Diuretics: Acetazolamide 3) Topiramate also used - Added benefit of causing Wt. loss 4) Repeated LP 5) Surgery - Optic N sheath decompression & fenestration (to prevent ON damage) 6) Lumbo-Peritoneal or VP Shunt - To reduce ICP
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Hallmarks of NPH ??
REVERSIBLE cause of Dementia in Elderly pts. - 2ndary to Reduced CSF absorption at Arachnoid villi Cause: Head injury, SAH or Meningitis TRIAD - Urinary Incontinence - Dementia & Bradyphrenia - Gait abnormality (similar to PD) 60% of pts. have all these features These develop over a few months
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Ix. & Rx. ??
Hydrocephalus with Ventriculo-megaly in the absence of, or out of proportion to, sulcal enlargement Rx.- - VP Shunt - 10% with Shunt experience significant complications like: Seizure , Infection & Intracranial Bleed
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Hallmarks of Herpes Simplex Encephalitis ??
HSV-1 (95% cases) typically affects the - TEMPORAL Lobes & - Inferior Frontal Lobes Features - Fever, Headache, Psychiatric C/F, Seizures, N & V - Focal features: APHASIA - Peripheral lesions (eg Cold sores) have NO RELATION to the presence of HSV Encephalitis
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Ix. of HSV Encephalitis ??
CSF: Lymphocytosis, Elevated protein PCR for HSV CT : Medial Temporal & Inferior Frontal changes (eg Petechial haemorrhages)- Normal in 1/3rd pts. MRI is Better : Hyperintensity of the affected White matter & Cortex in the Medial Temporal lobes & Insular Cortex EEG : Lateralised Periodic discharges at 2 Hz
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Rx. of HSV Encephalitis ??
IV Acyclovir - Prognosis is dependent on whether Acyclovir is started Early - If Rx. started promptly: Mortality is 10- 20% - Left untreated: Mortality is 80%
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What is Transient Global Amnesia ??
Acute onset of Anterograde Amnesia (inability to form NEW memories) - Due to Transient ischaemia to THALAMUS (particularly Amygdala & Hippocampus) Features - Pt. appears Anxious & repeatedly asks the same questions - Episodes are self-limiting & resolve within 24hrs.
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What is TIA ??
Tissue based definition (now used) - A transient episode of neurological dysfunction caused by Focal brain, Spinal C, or Retinal ischaemia without Acute infarction Time based definition (formerly used) - Sudden onset of focal neurological symptom &/or sign lasting , 24hrs, due to a transient decrease in blood flow
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C/F of TIA ??
Sudden onset FND but rather than persisting, the features resolve within 1 hour - U/L weakness or Sensory loss - Aphasia or Dysarthria - Ataxia, vertigo or loss of balance Visual problems - Sudden transient loss of vision in 1 eye (Amaurosis fugax) - Diplopia - Homonymous hemianopia
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Ix. done in TIA ??
1) NEUROIMAGING CT Brain should NOT be done UNLESS there is a clinical suspicion of an alternate dx. that CT could detect MRI (with Diffusion weighted & Blood sensitive sequence) - Preferred to determine Ischaemic Territory or to detect Haemorrhage or alternative pathologies Done on the same day as specialist assessment if possible 2) CAROTID Imaging - Atherosclerosis in carotids may be a source of Emboli in some pts. - ALL pts. must have an URGENT Doppler unless they are not a pt. for Carotid Endarterectomy
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Assessment & Referral of TIA ??
Treat TIA urgently Aim: Reduce risk of Recurrent stroke - 10% risk in first 7 days after TIA ASPIRIN 300mg immediately (unless CI) in suspected cases PPIs if dyspepsia a/w aspirin use If Aspirin can't be used (allergic or intolerant) give CLOPIDOGREL Refer the pt. within 24 hrs to Specialist assessment & Ix. to a TIA clinic
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Specialist Review of TIA ??
If >1 episode of TIA (Crescendo TIA) has occurred (OR) has a suspected Cardio-Embolic source (OR) Severe Carotid Stenosis - Admission or Observation with a Stroke specialist If TIA suspected in last 7 days : - Urgent assessment (in < 24hrs) by a Specialist Stroke physician If TIA suspected to have occurred > 7 days previously - Specialist Assessment within 7 days Advise NOT to drive
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Further Rx. of TIA ??
ANTI-THROMBITIC Therapy 1) Start DAPT in pts. presenting in < 24hrs of TIA + Low risk of bleeding - [Aspirin + Clopidogrel]*21 days (OR) - [Aspirin + Ticagrelor[*30 days - If pt. NOT appropriate for DAPT, give Clopidogrel loading dose followed by Daily maintenance dose 2) PPIs if risk of GI bleed or Dyspepsia 3) Single Anti-P Rx. should be used with Clopidogrel recommended as std. Rx. :- - After DAPT completion - For long-term prevention of vascular events in people with TIA without paroxysmal or permanent AF - ASPIRIN used if Clopidogrel is not tolerated 4)
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Further Rx. of TIA ??
4) AC with a Rapid onset action to pts. with Paroxysmal, persistent or permanent AF or A Flutter asap after IC bleed or other CI (severe HTN, >=180/120, should be treated 1st) are Excluded 5) High Intensity STATIN Therapy immediately unless CI - Start in ALL pts irrespective of baseline LDL (2ndary prevention) - If alternate Statin at max. tolerated dose (if high intensity statin not suitable or not tolerated)
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Carotid Artery Endarterectomy ??
Degree of CA Stenosis is reported using NASCET (North American Symptomatic C Endarterectomy Trial) 1) TIA or Acute non-disabling ischaemic stroke + STABLE neuro symptoms + Symptomatic Severe Carotid stenosis 50-99% - Assessed & referred for CAE to be performed in < 7 days of C.F onset - Receive optimal medical Rx.: Control BP, Anti-P Rx., Cholesterol reduction by diet & drugs, lifestyle advice, Smoking cessation 2) TIA or Acute Non-disabling Ischaemic stroke + Mild-moderate carotid stenosis < 50% should: - NOT undergo CAE - Receive optimal drug Rx. as above 3) Meet the CAE criteria + Unsuitable for Open Sx. (inaccessible Carotid bifurcation, Restenosis after CAE, Radiotherapy associated carotid stenosis) - Carotid Angioplasty + Stenting
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Stroke Assessment ??
ROSIER Score Exclude Hypoglycaemia first & then LoC or Syncope : [-1] Seizure activity : [-1] New, Acute Onset of : - Asymmetric Face weakness : [+1] - Asymmetric Arm weakness : [+1] - Asymmetric Leg weakness : [+1] - Speech disturbance ; [+1] - Visual Feild Defect [+1] Stroke is likely if score is > 0
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First Ix. of Stroke ??
NCCT of Head - Ischaemic or Haemorrhagic Acute Ischaemic Stroke - Areas of low density in Grey or White matter of the territory (These changes may take time to develop) - Hyperdense artery sign corresponds to the responsible Arterial clot- visible immediately Acute Haemorrhagic Stroke - Area of Hyperdense material (Blood) surrounded by low density (oedema)
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TYPES of Stroke ??
Oxford Stroke Classification (aka Bamford Classification) classifies stroke based on Initial C/F The follo. criteria is assessed - U/L Hemiparesis &/or Hemisensory loss of the Face, arm & leg - Homonymous Hemianopia - Higher Cognitive dysfunction eg.- Dysphagia
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Total Anterior Circulation Stroke ??
TACI (15% ) - Involves MCA + ACA arteries - All 3 of Bamford criteria (+)ve
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Partial Anterior Circulation Stroke ??
PACI (25%) - Involves Smaller arteries of Anterior circulation, eg- Upper or Lower division of MCA - 2 of the 3 Bamford criteria (+)ve
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Lacunar Infarcts ??
LACI (25% cases) Involves perforating arteries around Internal capsule, Thalamus, BG Presents with one of the following- - U/L weakness (&/or Sensory deficit) of Face & arm, Arm & leg or ALL 3 - Pure Sensory Stroke - Ataxic Hemiparesis
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Posterior Circulation Strokes ??
POCI (25% cases) Involves Vertebro-basilar artery Presents with 1 of the following - Cerebellar or Brainstem syndromes - Loss of Consciousness - Isolated Homo. Hemianopia
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Wallenberg Syndrome ??
Lateral Medullary Syndrome [Poste. Inferior Cerebellar Artery] - Ataxia, Nystagmus - I/L : Dysphagia, Facial numbness, CN palsy (Horner's) - C/L : Limb Sensory loss
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Weber's Syndrome ??
I/L CN-3 palsy C/L Weakness
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Initial Rx. of suspected Stroke ??
Stabilise + Refer to Hyperacute or Acute stroke unit ABC Consider ET Intubation in - Unable to protect airway - GCS <= 8 Give O2 only if sats. < 93% - Target: 96% in acutely ill + not at risk of Hypercapnia - Target: 88-92% If at risk of Hypercapnia
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Rx. of Confirmed Stroke ??
IV Alteplase (Recombinant TPA) - IC Bleed must be excluded before Thrombolysis Window < 4.5 hrs of C/F onset (OR) 4.5 to 9 hrs of known onset or in < 9 hrs of midpoint of sleep when they have woken up with C/F & - Evidence of Potential to salvage brain tissue on CT perfusion or MRI (DWI-FLAIR mismatch) - Whom Mechanical Thrombectomy is either not indicated or not planned IV Tenecteplase is an alternative BUT is NOT recommended in - Ischaemic stroke on awakening from sleep (OR) - Unknown onset who undergo no brain imaging other than CT IV Thrombolysis should not delay Mechanical Thrombectomy
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Criteria for extending Thrombolysis to 4.5 to 9 hrs & wake-up stroke ??
Wake-up stroke - > 4.5 hrs from last seen well, no upper limit - MRI DWI-FLAIR mismatch - DWI lesion + No FLAIR lesion Wake up stroke/ Unknown onset time - > 4.5 hrs from last seen well & < 9hr of the midpoint of sleep (time 1/2way b/w going to bed & waking up) - CT or MRI Core-perfusion mismatch - Mismatch ratio >1.2, mismatch volume > 10ml & Ischaemic core volume < 70ml Known Onset Time (4.5 to 9 hrs) - CT or MRI Core perfusion mismatch - Mm ratio > 1.2, Mm Vol. > 10ml & Ischaemic Core vol < 70ml
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Hallmarks of Mechanical Thrombectomy ??
Eligible MT pts. should receive prior IV Thrombolysis asap (unless CI) irresepective of if they presented to Acute Strike Unit or Thrombectomy Centre ASAP in < 6 hrs of C/F onset (together with IV Thrombolysis) if not CI to - - With NO previous disability (Mod. Rankin Score 0 or 1) - With confirmed occlusion of the Proximal Anterior Circulation & Proximal IC Large Artery Occlusion causing a disabling neuro deficit (NIHSS of >=6) ASAP in pts. last known to be well b/w 6-24 hrs previously (including wake-up-stroke) & With No Previous Disabilities (Mod. Rankin S 0 or 1) Combined with Thrombolysis, if eligible ------
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Hallmarks of Mechanical Thrombectomy ??
ASAP in pts. last known to be well b/w 6-24 hrs priorly (including wake-up-stroke) & With no previous disabilities (Mod. Rankin S 0 or 1) Combines with Thrombolysis, if eligible 1) Who have Confirmed Proximal IC Large Artery Occlusion (ICA &/or MI) causing a disabling Neuro. deficit (NIHSS score of >= 6) 2) If there is a Potential to Salvage Brain Tissue, as shown by Perfusion imaging - B/w 6- 12 hrs: ASPECTS Score >= 3, irrespective of the Core Infarct size - B/w 12- 24 hrs: ASPECTS Score >=3 & CT or MRI Perfusion Mismatch of >15 ml irrespective of Core I size Consider MT + IV thrombolysis (IF not CI & within licensed time window) in pts/ with - Confirmed occlusion of Proximal Anterior Circulation (By CTA or MRA) - If there is potential to Salvage brain tissue as shown by perfusion imaging
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Who are the pts. for Thrombectomy ??
Pts. who cannot receive Thrombolysis eg.- CI like Recent Sx., Current AC use
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Anti-Platelets use in Stroke ??
Offer ASAP but certainly within 24hrs (unless CI) in Acute Stroke + IC Bleed has been excluded by imaging - In practice, administration of Aspirin (or alternative) is usually delaye 24hrs after Alteplase or Tenecteplase, once further CT scan has excluded significant bleed - Anti-P Rx. is CI or Delayed in pts with active bleeding (eg GI bleed)
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Anti-Platelet of choice in Stroke ??
ASPIRIN (only) in pts. with Disabling Ischaemic stroke - Continue daily for 2 wks - Then start definitive long-term Anti-Thrombotic Rx. DAPT with either Aspirin & Clopidogrel for 21 days (OR) Aspirin + Ticagrelor for 30 days In pts. presenting within 24hrs of minor stroke & with a low risk of bleeding - PPI to reduce risk of GI Bleed - Minor Ischaemic stroke who are not appropriate for DAPT, give Clopidogrel monotherapy SECONDARY Prevention - After 21 or 30 days, for long term prevention of vascular events in people with Ischaemic stroke without Paroxysmal or Permanent AF, single Anti-Platelet Rx. should be used
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Aspirin route of a administration in Stroke ??
Orally (if no Dysphagia) or Rectally or by Enteral tube (for those with dysphagia) Aspirin allergy/ Intolerance/ S/Es - CLOPIDOGREL is used as an alternative - Offer PPIs in addition to Aspirin if any pt. reports dyspepsia a/w Aspirin
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VTE Prophylaxis in Acute Stroke ??
Intermittent Pneumatic Compression within 3 days of admission - Do NOT use LMWH or Graduated compression stockings In Practice, LMWH is considered if IPC is CI or not possible Do NOT use AC routinely Indications of AC in stroke include - Cardiac source of Embolism - Cerebral Venous Thrombosis - Some cases of Arterial dissection If already on AC (prosthetic HV) + Disabling Ischaemic stroke + At risk of Haemorrhagic transformation - Stop AC for 7 days + substitute with ASPIRIN
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High intensity Statin Therapy in Acute Stroke ??
Do NOT start Rx. immediately - Appropriate to start once the pt. can swallow - Consensus states: Safe to start after 48 hrs Moderate or Low intensity statin Rx. at max. tolerated dose if High intensity statin is unsuitable or not tolerated Continue statin if already on it.
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Glycaemic Control in Acute Stroke ??
Post stroke + Hyperglycaemia = Increased risk of mortality independent of their age & severity of stroke; due to - - Increased Tissue Acidosis from Anaerobic metabolism, - Free radical generation, - Increased BBB permeability BG maintained at 4 to 11 mmol/L - DM: Insulin + Glucose to all adults - Hypoglycaemia (can cause Neuronal injury) Exclude Hyper- or Hypo- glycaemia before Thrombolysis as - Hypo-G : is a Stroke mimic - Hyper-G : a/w IC Bleed & worse clinical outcome
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What is Reye's Syndrome ??
Severe progressive encephalopathy affecting children that is a/w Fatty infiltration of Liver, Kidneys & Pancreas Cause- A/W Aspirin use & Viral cause Peak Incidence at 2 yrs of age - H/o preceding Viral illness - Encephalopathy: Confusion, Seizures, Cerebral Oedema, Coma - Fatty infiltration of .... - Hypoglycaemia Rx.- SUPPORTIVE
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Visual Field defects (VFD) ??
1) Lt. Homo. Hemianopia : VFD to left ie. lesion of Rt. Optic tract 2) Homo. Quandrantanopias (PITS) - Parietal-Inferior, Temporal-Superior 3) INCONGROUS defects: Optic tract defect 4) CONGROUS defect: Optic Radiation or Occipital Cortex lesion
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Homo. Quandrantanopias with - Superior part affected ?? - Inferior part affected ??
Lesion: Inferior Optic Radiations in Temporal lobe (MEYER'S Loop) Lesion: Superior Optic Radiations in Parietal lobe
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Homo. Hemianopia with - INCONGROUS defect ?? - CONGROUS defect ?? - MACULA Sparing ??
- Optic TRACT lesion - Optic RADIATION or Occipital Cortex - Occipital CORTEX lesion
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Bitemporal Hemianopia ??
Lesion: Optic CHIASM Upper Quadrant defect>>Lower Q D - INFERIOR Chiasmal compression => PITUITARY Tumour Lower Quadrant defect>>Upper Q D - SUPERIOR Chiasmal compression => CRANIOPHARYNGIOMA
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Internuclear Ophthalmoplegia ??
INO is caused by MLF Lesion - MLF coordinates eye movt. by linking CN-6 nucleus of one side of brainstem with the CN-3 of opposite side Causes - MS (MCC in younger pts.) - Stroke (common in Older pts.) - Trauma - Tumour & Infections: Less Common cause
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What are INO-Plus Syndrome ??
INO a/w additional Neuro. deficits 1) WEBINO: Wall-Eyed B/L INO - B/L INO + Wall-Eyed Appearance 2) One & a Half Syndrome - INO + I/L Horizontal gaze palsy 3) Eight & a Half Syndrome - One & a Half synd. + CN-7 palsy 4) Half-&-Half Syndrome - INO + CN-4 Palsy
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C/F of INO ??
Adduction on I/L side of MLF lesion - Affected eye shows limited or no movt. towards nose ABDucting Nystagmus. of C/L eye - Opposite eye on looking outwards often exhibits Nystagmus Diplopia
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What is Wall-Eyed B/L INO ??
B/L INO with the following C/F - B/L ADDuction deficit - B/L ABDucting Nystagmus - Wall-Eyed appearance: Eyes appear to be turned Outwards (Exotropia) at rest giving a 'Wall-Eyed' look CAUSE: Severe Brainstem lesion due to - MS, Stroke or Trauma affecting both MLFs
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C/F of One & a Half Syndrome ??
I/L Horizontal Gaze Palsy C/L INO : Other eye shows ADDuction deficit Residual movt.- - ONLY remaining Horizontal movt. is ADDuction of C/L eye; hence the term '1.5'
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What is One & a Half Syndrome ??
INO + Horizontal gaze palsy Lesion: Affects both MLF + Adjacent Pontine str. such as Paramedian Pontine Reticular Formation (PPRF) (OR) CN-6 nucleus Causes - Stroke in the Pons - MS : Especially in Young pts. - Tumours & Other Brainstem Pathologies: LESS Commonly
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What is Eight-&-a-Half Syndrome ??
1.5 synd. + CN-7 palsy Features - C/F of 1.5 syndrome - CN-6 Palsy: Weakness or Paralysis of the muscles on 1 side of the face CAUSES - Brainstem lesion: Stroke, MS, or other Focal Brainstem pathology - Affects both MLF & CN-7 Nucleus or Fascicle
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Hallmarks of Neuromyelitis Optica ??
NMO is a Monophasic or Relapsing-remitting Demyelinating CNS disorder - Distinct disease (Not a part of MS) - MC in Asians Typically involves Optic nerves & Cervical Spine - Vomiting (common presenting c/f)
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Causes of Miosis ??
SMALL Pupils - Horner's syndrome - Argyll-Robertson pupil - SENILE Miosis - PONTINE Haemorrhage - Congenital Drug Causes - Opiates, - OP Toxicity - Parasympathomimetics: Pilocarpine
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What is Half-&-Half Syndrome ??
INO + CN-4 (Trochlear N) palsy Features - INO features - CN-6 Palsy: VERTICAL Diplopia due to SO muscle weakness => UPWARD Deviation of affected Eye CAUSES: Brainstem lesions - Stroke, MS, other Focal Brainstem pathology affecting both the MLF & CN-6 or its Nocleus
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Nystagmus types ??
UPBEAT Nystagmus - Cerebellar VERMIS Lesions DOWNBEAT Nystagmus - Foramen Magnum Lesion - Arnold-Chiari malformation
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Dx. of Neuromyelitis Optica ??
B/L Optic Neuritis & Myelitis + 2 of the following 3 criteria - SC lesion involving >= 3 spinal levels - Initially normal MRI brain - AQUAPORIN 4 (+)ve serum Antibody
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Hallmarks of Multiple Sclerosis ??
Chronic Cell mediated Autoimmune disorder characterised by Demyelination of CNS - 3x MC in Women - 20- 40 yrs old - MC in Higher latitudes (5x MC than in Tropics) Monozygotic Twin Concordance: 30% Dizygotic Twins Concordance: 2%
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Dx. of MS ??
Made on the basis of >= 2 relapses & either objective clinical evidence of >= 2 lesions (OR) objective clinical evidence of 1 lesion together with reasonable historical evidence of a previous relapse VISUAL - Optic Neuritis (MC presenting c/f) - Optic atrophy - Uhthoff's phenomenon: Vision deteriorates on rising body temp. - INO SENSORY - Pins/ Needles, - Numbness - Trigeminal Neuralgia - Lhermitte's Synd.- Limb paraesthesia on neck flexion MOTOR, CEREBELLAR & OTHERS
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Types of Multiple Sclerosis ??
RELAPSING-REMITTING disease - MC form (83% cases) - Acute attacks (eg, 1-2 months): Followed by period of Remission SECONDARY PROGRESSIVE Disease - Describes R-R type pts. who have deteriorated & have developed neuro signs & c/f b/w relapses - 65% of pts. with R-R type do to develop 2ndary progressive disease within 15yrs of Dx. - GAIT & BLADDER disorders seen PRIMARY PROGRESSIVE Disease - 10% of pts. - Progressive deterioration from start - MC type in Older adults
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Motor, Sensory & Other features of MS ??
MOTOR - Spastic weakness: MC seen in legs CEREBELLAR - Ataxia : MC during an Acute Relapse than as a presenting feature - Tremor OTHERS - Urinary Incontinence - Sexual dysfunction - Intellectual deterioration
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Ix. done in MS ??
Dx. requires demonstration of lesions disseminated in Time & Space MRI - High signal T2 lesion - Periventricular plaques - Dawson Finger: Often seen on FLAIR images: Hyperintense lesion perpendicular to C Callosum CSF - Oligoclonal bands (& NOT in serum) - Increased Intrathecal synthesis if IgG Visual Evoked Potentials - Delated, but well-preserved waveform
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Rx. of MS ??
Aim: Reducing the frequency & duration of relapses. NO cure Acute Relapse - High dose STEROIDS may be given for 5 days to shorten the length of an acute relapse - Steroids SHORTENS the duration of relapse & do NOT alter the degree of recovery (ie. whether a pt. returns to baseline functions)
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PROGNOSTIC Features of MS ??
GOOD Prognosis features - Female sex - Young age of onset (20- 30) - R-R disease - Sensory symptoms ONLY - Long interval b/w first 2 episodes - Complete recovery b/w relapses
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Specific problems encountered in MS ??
FATIGUE - Exclude other causes (anaemia, TFT) - Trial of AMANTIDINE - 2nd line: CBT, Mindfulness training SPASTICITY - 1st line: BACLOFEN & GABAPENTIN - 2nd line: Diazepam, Dantrolene & Tizanidine - Physiotherapy BLADDER DYSFUNCTION - Urgency/ Incontinence/ Overflow - Do an USS first to assess Bladder emptying- Anticholinergics may worsen c/f in some pts. - Significant Residual V: Intermittent Self catheterisation - NO significant Residual V: Anticholinergics OSCILLOPSIA
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What are the Drug options for reducing the risk of Relapse in MS ??
NATALIZUMAB (1st line) given as IV - Recombinant monoclonal Anti-B - Antagonises Alpha-4-Beta-1-integrin found on Leucocytes surface - (-) Leucocyte migration across the endothelium across BBB - Has the Strongest evidence for PREVENTING Relapse FINGOLIMOD BETA- INTERFERONS GLATIRAMER ACETATE
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Rx. of Oscillopsia in MS ??
Visual Field appear to Oscillate 1st line Rx.- GABAPENTIN
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Rx. of MS ??
Typical Indication of Disease-Modifying Drugs (DMDs) include - R-R disease + 2 relapses in past 2 yrs + able to walk 100m unaided - 2ndary Progressive D + 2 relapses in past 2 yrs + able to walk 10m (aided or unaided)
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Hallmarks of - Fingolimod ?? - Beta-IFN ??
FINGOLIMOD - Sphingosine 1-phosphate (SIP) receptor modulator - Prevents Lymphocytes from leaving LNs - Oral formulations available BETA-IFN (IM or SC route) - Not considered to be as effective as DMDs
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Features of MS ??
Lethargy Double vision, pain behind eyes, decrease colour vision (red desaturation), INSULAR = Intention tremor, Nystagmus, Slurred speech, Urinary probs, Labile mood, Ataxia, Retrobulbar neuritis
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Hallmarks of Glatiramer Acetate ??
Immunomudulating drug: acts as 'IMMUNE Decoy' Given SC Along with Beta-IFN considered an 'Older drug' with less effectiveness compared to Monoclonal Anti-B & SIP receptor modulators
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Third nerve palsy ??
Eye deviated 'Down & Out' Ptosis Pupil may be dilated (sometimes called a 'surgical' 3rd N palsy) Causes : DM, - Vasculitis (T arteritis, SLE) False Localising sign (due to Uncal herniation through tentorium if raised ICP Poste. Communicating A Aneurysm - Pupils dilated - a/w Pain Cavernous sinus thrombosis Weber's Synd: I/L 3rd N palsy + C/L Hemiplegia (Midbrain stroke) Amyloid, MS
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4th nerve Palsy ??
Supplies Superior Oblique (depresses eye, moves inward) SO4 FEATURES - Vertical Diplopia : Classic when reading a book or going downstairs - Torsional Diplopia: Subjecting tilting of objects - Pt. may develop Head tilt (Lt. CN4 palsy => Rt head tilt), which they may or may not be aware of - When looking straight: affected eye Deviates upwards & outwards
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Cerebellar signs ??
U/L cerebellar lesions causes I/L signs "DANISH" - Dysdiadochokinesia, Dysmetria (past-pointing), pt. may appear drunk - Ataxia (limb, truncal) - Nystagmus (Horizontal=I/L Hemisphere) - Intention Tremor - Slurred staccato speech, Scanning dysarthria - Hypotonia
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Causes of Cerebellar signs ??
Friedreich's Ataxia, Ataxia telangiectasia Neoplasia: Cerebellar Hemangioma Stroke Alcohol MS Hypothyroidism DRUGS: Phenytoin, Lead poisoning Paraneolpastic: eg.- 2ndary to Lung Cancer
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Hallmarks of Ataxia Telangiectasia ??
A R Condition : Defect in ATM Gene (encodes DNA repair enzyme) One of the Inherited Combined Immunodeficiency disorders - Early childhood, 1- 5 yrs old with abnormal movt. - Cerebellar ataxia - Telangiectasia - IgA deficiency => Recurrent Chest infection - 10% risk of Malignancy, Lymphoma, Leukaemia - Also Non-Lymphoid tumours
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Hallmarks of Friedreich's Ataxia ??
A R condition - GAA repeat disorder [in X25 gene on Chr. 9 (Frataxin)] - Do NOT show anticipation Onset: 10- 15 yrs - MC presenting C/F: Cerebellar ataxia & Kyphoscoliosis - HOCM (90%)- MCC of death - Absent ankle jerks/ Extensor plantar - Optic Atrophy - Spinocerebellar tract degeneration - DM (10-20%) - High-arched palate - Telangiectasia (at 1- 2 yrs)
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Tell the following about Friedreich's Ataxia - MCC of death ?? - Earliest feature ??
- HOCM (90% cases) - Telangiectasia
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Hallmarks of Autonomic Neuropathy ??
Impotence, Inability to sweat, Postural hypotension (drop of 30/15 mmHg) Loss of decrease in HR following Deep breath Pupils: DILATES after Adrenaline instillation CAUSES - DM, - GBS, - PD - MSA (Shy-Drager's syndrome) - HIV, Chaga's, Neurosyphilis Drugs: Anti-HTN, TCAs - Craniophatyngioma
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Hallmarks of Spastic Paraparesis ??
UMN pattern of weakness in LL Causes - Demyelination (MS) - Cord compression: Trauma, Tumour - Parasagittal Meningioma - Tropical Spastic Paraparesis - Transverse myelitis (eg,- HIV) - Syringomyelia - Hereditary Spastic Paraplegia - Osteoarthritis of Cervical spine
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Brown-Sequard Syndrome ??
Lateral Hemisection if SC - I/L weakness below lesion - C/L loss of pain & temp. sensation
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Hallmarks of Transverse Myelitis ??
Inflammation of Myelin, extends transversly across SC - Damages myelin sheath Causes - Virus: VZV, HSV, CMV, EBV, HIV, Influenzae, Echovirus - Bacterial: Syphilis, Lyme's - Post-infectious: Immune mediated - First C/F of MS or Neuromyelitis optica (NMO)
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Subacute Combines Degeneration of SC ??
Due to B12 deficiency Dorsal Column + Lateral CS tract affected - Joint position & Vibration sense lost first UMN signs develops in leg, classically- Extensor plantar, brisk knee reflex, Absent ankle jerks In untreated: Stiffness & Weakness persists
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Brachial Neuritis
Acute onset U/L (occasionally B/L) severe pain - Followed by Shoulder & Scapular weakness several days later - Sensory changes are minimal Rapid wasting of Arm muscles in accordance to the nerve involved RFs : Recent Trauma, Infection, Sx., Vaccination Prognosis: Good except when PHRENIC Nerve is involved => Breathlessness
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Cervical Spondylitic Myelopathy ??
Degenerative condition of C Spine, essentially OA of Cervical vertebral bodies - Spinal canal narrowed => press on SC => Neuro dysfunction - Seen in 5- 10% of pts. who have cervical spondylosis - Neck pain - UMN weakness in LLs => Increased reflexes, tone & upgoing plantars - Motor weakness, Sensory loss & Bladder/ Bowel dysfunc. - Wide-based, Ataxic or Spastic gait - Bladder: Urgency, retention
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Basilar Migraine ??
Aura : Posterior territory vascular disturbance (Acute-onset vertigo, tinnitus, slurred speech, double vision) lasting for a few hrs Followed by Headache a/w vascular migraine
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Tourette syndrome ??
Typical Behaviour Vocal Tics (Bad language, shouting in class) Motor tics (eg.-involuntary hand movt.) Rx.- Low dose RISPERIDONE High dose can cause tardive dyskinesia
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Anterior Spinal artery occlusion ??
Supplies Anterior 2/3rd of SC - Loss of B/L Motor function - Loss of B/L Pain & Temp. sensation Vibration & Proprioception are preserved
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