Neurology Flashcards
What is Wallenberg syndrome ??
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
What is Dejerine Syndrome ??
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
-
What is Foville Syndrome ??
Occlusion of Paramedian branch of Basilar artery
- I/L Facial weakness
- C/L Hemiparesis
- Conjugate gaze palsy
- Involves the Abducens nucleus & Parapontine reticular formation
What is Thalamic Stroke syndrome ??
Small vessel occlusion or Poste. Cerebral Artery issues
- C/L Sensory loss (Pain, Temp., Touch)
- Thalamic pain syndrome (Burning pain)
- Hemianopia
What is Locked in Syndrome ??
PONTINE stroke
- Complete paralysis with preserved Veritical eye movt.
- Preserved Cognition but unable to speak or move
What is Top of the Basilar Artery Synd. ??
Stroke of Top of Basilar artery => Ischaemia in Brainstem + Occipital lobe
- Altered consciousness/ Coma
- Visual disturbance (eg. blindness)
- Diplopia & Ataxia
What is Millard-Gubler syndrome ??
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
What is Striatocapsular Infarct ??
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.
What is Posterior Cerebral Artery Syndrome ??
Posterior Cerebral artery occlusion => Visual & Memory disturbance
- Homonymous Hemianopia
- Visual agnosia (inability to recognize objects)
- Memory deficits
What is Claude Syndrome ??
Posterior Cerebral Artery occlusion => Stroke of Midbrain
- C/L Hemiparesis
- I/L Oculomotor Nerve Palsy (Ptosis, Mydriasis)
- C/L Ataxia
What is Anterior Inferior Cerebellar Artery syndrome ??
Occlusion of AICA => Cerebellar ischaemia
- Vertigo, Nausea
- U/L Hearing loss
- Ataxia & Dysarthria
What is Weber’s Syndrome ??
Posterior Cerebral Artery occlusion => Midbrain Stroke
- C/L Hemiparesis
- I/L Oculomotor nerve palsy (Ptosis, Mydriasis)
What is MCA syndrome ??
Occlusion of MCA => supplies a large portion of Cerebral cortex
- C/L Hemiparesis Face, Arm»_space;> leg)
- C/L Sensory loss
- Aphasia (if Left MCA) & Hemineglect (if Right MCA)
- Homonymous Hemianopia
What is ACA syndrome ??
Occlusion of ACA => affects MEDIAL aspects of FRONTAL & PARIETAL Lobes
- C/L Leg weakness»_space;> Arm/ Face
- Personality changes, Confusion & Disinhibition
- Urinary Incontinence
- C/L Sensory loss (primarily in Legs)
What is PCA syndrome ??
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)
What is Lacunar Stroke ??
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
Name the Posterior Fossa Malformations
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
What is Syringomyelia ??
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
What other conditions are a/w
- Chiari 2 M ??
- Dandy-Walker M ??
- Meningomyelocele (usually Lumbo-sacral)
- Spina bifida
What is the RMP of Neurons ??
-75 mV
What are the Watershed zones of the brain ??
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
Mention the C/F of strokes affecting Watershed zones
ACA-MCA watershed infarct
- Proximal ULs & LLs weakness (Man-in-a-Barrel syndrome)
PCA-MCA watershed infarct
- Higher order Visual Dysfunction
Name the Anterior Circulation Strokes
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
Symptoms of ACA stroke ??
C/L paralysis & Sensory loss of LLs & Urinary incontinence
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
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
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
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
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)
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
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
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
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)
Why does Brain Herniation occur ??
Increased ICP
- Mass effect (Haemorrhage)
- Cerebral edema (Stroke or Trauma)
- Hydrocephalus
Divided into 2 categories
- Supratentorial
- Infratentorial
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
Artery involved in Central/Downward Transtentorial Herniation ??
Brainstem Caudal displacement
- Rupture of Paramedian Basilar artery branches => DURET Haemorrhages
- Decorticate & Decerebrate Posturing
- Fatal
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)
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
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.
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
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
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
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.
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.
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)
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.
Acute Rx. of
- SUNCT or SUNA ??
- Hemicrania Continua ??
1) IV Lidocaine
- Lamotrigine & Topiramate (Short-term Rx.)
2) ToC: INDOMETHACIN
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
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)
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
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
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
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
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
General rule in the Rx. of Migraine ??
5-HT r (+) : used for Acute Rx.
5-HT r (-) : used for Prophylaxis
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.
Prophylaxis of Migraine ??
Given IF >= 2 attacks/ month
1) 1st line: Propranolol or Topiramate
Women of Child-bearing age
- PROPRANOLOL»_space; 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.
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
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)
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
Which type of Contraception are NOT affected by Topiramate ??
Depo-Provera Injection
Intrauterine System
Inducer of P450 enzyme CYP3A4
S/E of Topiramate ??
Reduced appetite & Wt. loss
Dizziness
Paraesthesia
Lethargy & Poor conc.
Rare: Acute Myopia & 2ndary Angle Closure Glaucoma
Teratogenic (malformations)
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
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
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.)
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
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
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
Rx. of Trigeminal Neuralgia ??
1st line: CARBAMAZEPINE
Refer to Neurology if failure to respond to Rx. or Atypical features (< 50yrs old)
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
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
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
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.
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
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)
Rx. of P Apoplexy ??
IoC : MRI is diagnostic
Urgent Steroid replacement (due to ACTH loss)
Careful Fluid balance
Surgery
Features of Occipital Lobe lesions ??
Homo. Hemianopia with Macular sparing
Cortical Blindness
Visual Agnosia
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)
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
Features of Cerebellum lesions ??
Midline lesion: Gait & Truncal Ataxia
Hemisphere Lesions
- Intention tremor, - Nystagmus
- Past-pointing
- Dysdiadokinesis
Kluver-Bucy Syndrome ??
Hypersexuality, Hyperorality, Hyperphagia, Visual Agnosia
- AMYGDALA is affected
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
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
Conditions where Lymphocytes are raised in CSF ??
Viral Meningitis/ Encephalitis
TB meningitis
Partially treated Bacterial Meningitis
Lyme’s disease
Behcet’s
SLE
Lymphoma, Leukaemia
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
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)
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
Hallmarks of Anti-NMDA Receptor Encephalitis ??
PARANEOPLASTIC Syndrome
Features-
Prominent Psychiatric C/F
- Agitation, Hallucinations
- Delusions, Disordered thinking
Seizures, Insomnia, Dyskinesia
Autonomic instability
Rx. of Anti-NMDA Receptor Encephalitis ??
Immunosuppression
- Steroids, IGs, Cyclophosphamide, Rituximab alone or in combination
Plasmapheresis
Resection of Teratoma is also therapeutic
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
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
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
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
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
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
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
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
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
Name other Prion diseases ??
Kuru
Fatal Familial Insomnia
Gerstmann Straussler-Scheinker disease
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)
What is CADASIL ??
Cerebral A D Arteriopathy with Subcortical Infarcts & Leukoencephalopathy
- Rare cause of Multi-Infarct DEMENTIA
- Pt. often presents with Migraine
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
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
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
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
Global Aphasia ??
Lesion affecting ALL areas ie.
- Wernicke’s, A Fasiculus & Broca’s
- Severe Receptive & Expressive Aphasia
- Able to communicate using Gestures
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)
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
Absence Seizures ??
Form of Generalised Epilepsy
- MC in Children (3- 10 yrs old)
- Girls»_space; 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
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
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)
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
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
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)
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
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
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
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
How to localise features of Focal Seizures ??
PARIETAL Lobe (Sensory)
- Paraesthesia
OCCIPITAL Lobe (Visual)
- Floaters or Flashes
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
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
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
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
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
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
Hallmarks of Sodium Valproate ??
Works by Increasing GABA activity
S/E
- Teratogenic
- Neurodevt. delay
Hallmarks of Ethosuximide ??
DoC in Absence seizures
MoA: Blocks T-type Ca2+ channels in Thalamic neurons
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
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
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
Hallmarks of Carbamazepine ??
Chemically similar to TCAs
MoA: Binds to Na+ channels => Increase Refractory period
Uses: Trigeminal N & Bipolar disease
Exhibits Autoinduction
S/E of Carbamazepine ??
P450 inducer
Dizziness, Ataxia
Drowsiness, Headache
Visual disturbances (DIPLOPIA mc)
SJS
LEUCOPENIA & Agranulocytosis
Hypo[Na+]: 2ndary to SIADH
Autoinduction in Carbamazepine ??
When a pt. starts Carbamazepine, they may see a return of seizures after 3- 4 wks of Rx.
Hallmarks of Lamotrigine ??
MoA: Na+ channel Blockers
S/E: SJS
Hallmark of Vigabatrin ??
40% pts. develop visual field defect which may be IRREVERSIBLE
- Check Visual Fields every 6 months
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
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
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
Features of Drug induced Parkinson’s ??
MOTOR c/f are geberally
- RAPID onset
- B/L onset
- Rigidity & Tremors are Uncommon
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
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
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
MoA of Midodrine ??
Used in Orthostatic Hypotension due to Parkinson’s disease
- Acts on Peripheral Alpha-adrenergic receptors => Increase Arterial resistance
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
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
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
Hallmarks of DA r agonists ??
Ergot derived: Bromocriptine, Cabergoline
Ropinirole, Apomorphine
Prior to Rx. obtain
- Echo, CXR, ESR, Cr.
- Pts. should be closely monitored
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
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
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,
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
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
Anti-Muscurinics in PD ??
Blockes Cholinergic r
- used in Drug-induced Parkinsonism rather than Idiopathic PD
- Helps tremor & rigidity
Eg: Procyclidine, Benzotropine, Trihexyphenidyl (Benzhexol)
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
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
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
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
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
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
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
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.
Rx. of Hemiballism ??
Symptoms may decrease in Sleep
Anti-DA agents
- HALOPERIDOL
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
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
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
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
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
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
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
Cushing’s Reflex ??
HTN + Bradycardia, often occurs late & is usually a Pre-Terminal event
MC site of Epidural Haematoma ??
Temporal region
- Skull # cause rupture of MIDDLE MENINGEAL Artery
- Lucid Interval is experienced
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
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
Rx. of Acute SDH ??
Small or Incidental Acute SDH
- Observed conservatively
Sx. options
- Monitor ICP
- Decompressive Cranieotomy
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
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
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
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)
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
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)
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)
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
Drug Rx. in SAH ??
To prevent Vasospasm,
- NIMODIPINE for 21 days
- CCBs which targets brain vessels
Treated with Hypervolaemia, Induced HTN & Haemodilution
What are the important Predictive factors of SAH ??
Conscious level on Admission
Age
Amount of Blood seen on CT Head
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
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
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
Lateral Sinus Thrombosis ??
CN- 6 & 7 palsy
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
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
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
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
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
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
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
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%
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.
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
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
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
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
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
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)
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)
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
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
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)
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
Total Anterior Circulation Stroke ??
TACI (15% )
- Involves MCA + ACA arteries
- All 3 of Bamford criteria (+)ve
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
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
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
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
Weber’s Syndrome ??
I/L CN-3 palsy
C/L Weakness
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
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
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
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 ——
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
Who are the pts. for Thrombectomy ??
Pts. who cannot receive Thrombolysis
eg.- CI like Recent Sx., Current AC use
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)
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
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
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
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.
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
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
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
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
Homo. Hemianopia with
- INCONGROUS defect ??
- CONGROUS defect ??
- MACULA Sparing ??
- Optic TRACT lesion
- Optic RADIATION or Occipital Cortex
- Occipital CORTEX lesion
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
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
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
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
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
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’
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
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
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)
Causes of Miosis ??
SMALL Pupils
- Horner’s syndrome
- Argyll-Robertson pupil
- SENILE Miosis
- PONTINE Haemorrhage
- Congenital
Drug Causes
- Opiates, - OP Toxicity
- Parasympathomimetics: Pilocarpine
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
Nystagmus types ??
UPBEAT Nystagmus
- Cerebellar VERMIS Lesions
DOWNBEAT Nystagmus
- Foramen Magnum Lesion
- Arnold-Chiari malformation
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
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%
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
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
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
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
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)
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
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
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
Rx. of Oscillopsia in MS ??
Visual Field appear to Oscillate
1st line Rx.- GABAPENTIN
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)
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
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
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
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
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
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
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
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
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)
Tell the following about Friedreich’s Ataxia
- MCC of death ??
- Earliest feature ??
- HOCM (90% cases)
- Telangiectasia
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
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
Brown-Sequard Syndrome ??
Lateral Hemisection if SC
- I/L weakness below lesion
- C/L loss of pain & temp. sensation
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)
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
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
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
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
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
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
