Neurology Flashcards

Question

Ataxia Telangiectasia

Answer 1

**Autosomal recessive neurodegenerative disease** Defect in DNA repair Onset in childhood / adolescents **Features** Progressive ataxia Telangiectasia: conjunctivae, eyes, nose, skin creases Immunocompromised: defective cell-mediated immunity Lymphoproliferative disease

Answer 2

Autosomal recessive of ATP7B gene on chromosome 13 **CLANK:** **C**ornea: Keiser-Fleischer rings **L**iver: CLD **A**rthritis **N**euro: Parkinsonism, Ataxia, Psychiatric problems **K**idney: Fanconi Syndrome

Answer 3

**Inspection:** walking aids, disuse atrophy, contractures **Limb posiiton** * Leg: extended, internally rotated with root plantar flexed * Arm: flexed, internally rotated, supinated **Gait** * Unilateral lesion ---\> circumducting * Bilateral lesion --\> scissoring **UMN signs** * Increased tone * Pyramidal distrubution of weakness * Leg: extensors stronger than flexors * Arm: flexors stronger than extensors * Hyper-reflexia * Extensor plantars **Sensation** Sensory level = cord lesion Completion: CN (evidence of MS), Cerebellum (evidence of MS)

Answer 4

**Extensor in lower limbs** **Flexors in upper limbs**

Answer 5

**_Bilateral_** **Common causes:** Multiple sclerosis Cord compression COrd trauma Cerebral palsy **Other causes:** Familial spastic paraparesis Vascular: aortic dissection --\> Beck's syndrome Infection: HTLV-1 Tumour: ependymoma Syringomyelia Mixed UMN and LMN = MAST **M**otor neuron disease **At**axia, Friedrich's **S**ubacute degeneration of cord (B12 def) **T**aboparesis (tertairy syphilis)

Answer 6

**Hemisphere** Stroke Multiple sclerosis Space-occupying lesion Cerebral palsy **Hemicord (can be monoparesis)** Multiple sclerosis Cord compression

Answer 7

**Presentation** * Pain * Local, deep * Radicular pain * Weakness * LMN at the level of compression --\> nerve roots affected * UMN below the level of compression --\> cord affected * **Sensory level** * Spinchter disturbance **Causes** * Trauma, vertebral #s * Infection: epidural abscess, TB * Malignancy: breast, thyroid, bronchus, kidney, prostate mets * Disc prolapse: L1/L2 **Invx:** MRI **Mx** Neurosurgical emergency Malignancy: dexamethasome IV, radioterpay Abscess: surgical debridement and IV antibiotics

Answer 8

**Presentation** **Pain:** back pain and radicular pain down legs **Weakness:** bilateral and flaccid **Sensation: s**addle anaesthesia **Spincters:** incontinence / retention / poor anal tone **Causes** Trauma Malignancy Abscess Disc prolapse

Answer 9

**Anterior Spinal Artery Syndrome** Infraction of spinal cord in distribution of anterior spinal artery Ventral 2/3 of cord **Causes:** aortic dissection Para/ quadri-paresis Impaired pain and temperature sensation Preserved touch and propioception

Answer 10

Syrinx: tubular cavity in central canal of the cord **Commonly located in the cervical cord** Syrinx expands ventrally affecting: * Decussating spinothalamic neurons * Anterior horn cells * Corticospinal tracts Can have static background symptoms, worsening on coughing, sneezing **Causes:** * Blocked CSF circulation with decreased flow from posterior fossa * Arnold-Chiari malformation (cerebellum herniates through foramen magnum) * Masses * Spina bifida * Secondary to cord trauma, myelitis, cord tumours and AVMs **Main Features** **Dissociated sensory loss** * Loss of pain and temperature * Preserved touch , proprioception and vibration * Cape distribution **Wasting/ weakness of hands + claw hand** **Loss of reflexes in upper limb** **Charcot joints: shoulder and elbow** **Other Signs** Horner's syndrome UMN weakness in lower limbs with extensor plantars Syringobulbia: cerebellar and lower CN signs Kyphoscoliosis Invx: MRI **Mx** Surgical decompression at foramen magnum for Chiari malformation

Answer 11

**Retrovirus,** prevalent in Japan and Caribbean Adult T cell leukaemoa / lymphoma **Tropical spastic paraplegia / HTLV myelopathy** Slowly progressive spastic paraplegia Sensory loss and paraesthesia Bladder dysfunction

Answer 12

Rapid onset focal neurological deficit of vascular origin lasting \>24 hours

Answer 13

**80% ischaemic** Atheroma: large or small vessel Embolism: cardiac (AF or endocarditis) or atherothromboembolism **20% haemorrhagic** Raised BP, trauma, aneurysm rupture, anticoagulation, thrombolysis

Answer 14

Bamford classification of stroke (based on clinical features) **Total anterior circulation stroke (TACS)** = carotid/ MCA or ACA 1. Unilateral hemiparesis and/or hemisensory loss of the face, arm & leg 2. Homonymous hemianopia 3. Higher cognitive dysfunction e.g. dysphasia, neglect, apraxia 4. **Partial anterior circulation stroke (PACS)** * Involves smaller arteries of anterior circulation e.g. upper or lower division of middle cerebral artery * 2 of the above criteria are present **Posterior circulation infarcts (POCI, c. 25%)** * Involves vertebrobasilar arteries * Presents with 1 of the following: 1. cerebellar or brainstem syndromes 2. loss of consciousness 3. isolated homonymous hemianopia **Lacunar infarcts (LACI, c. 25%)** * Involves perforating arteries around the internal capsule, thalamus and basal ganglia * Presents with 1 of the following: 1. unilateral weakness (and/or sensory deficit) of face and arm, arm and leg or all three. 2. pure sensory stroke. 3. ataxic hemiparesis

Answer 15

**Chronic inflammatory condition of the CNS** characterised by **multiple plaques of demyelination** **separated by time and space**

Answer 16

CD4 cell mediates destruction of oligodendrocytes ---\> demyelination and eventual neuronal death Initial viral inflammation primes humoral antibody response Plaques of demyelination are hallmark finding

Answer 17

Relapsing-remitting = 80% Secondary progressive Primary progressive = 10% Progressive relapsing

Answer 18

**Presentation** **TEAM** **T**ingling **E**ye: optic neuritis (decrease central vision and eye pain on eye movement) **A**taxia + other cerebellar signs **M**otor: spastic paraparesis **Clinical Features** Sensory: dys/paraesthesia, decreased vibration sense, trigeminal neuralgia Motor: spastic paraparesis, transverse myelitis Eye: diplopia, **bilateral intranuclear opthalmoplegia**, optic neuritis Cerebellum: trunk and limb ataxiamscanning dysarthria, falls GI: swallowing

Answer 19

Electric shoch sensation in trunk and limbs after flexion of the neck

Answer 20

**Internuclear opthalmoplegia** Can abduct on convergence but not on lateral gaze

Answer 21

Decrease in visual acuity with heat e.g. hot bath **Sign of optic neuritis**

Answer 22

Pain on eye movement Rapid decrease in central vision Uhthoff's Decreased acuity Decreased colour vision White disc Central sarcoma RAPD

Answer 23

Also known as ataxic nystagmus / conjugate gaze palsy **Disruption of MLF connect CN VI to CN III** Weak adduction of affacted eye Nystagmus in contralateral eye upon gaze **Convergence preserved**

Answer 24

**Bloods** FBC comprehensive metabolic panel thyroid-stimulating hormone (TSH vitamin B12: rule out sub-acute degernation of the cord **MRI brain and spine** Gd-enhanced - image active inflammation Typically in **periventricular white matter** **Lumbar puncture** Glucose, protein, and cell count should be normal Oligoclonal bands and elevated CSF immunoglobulin G (IgG) and IgG synthesis rates are present in 80% of MS cases **Antibodies** Anti-NMO (anti-aquaporin 4 [AQP4]) antibody testing is recommended to rule out **neuromyelitis optica (Devic syndrome)** Anti-MBP **Visual evoked potential** Visual evoked potentials are most commonly abnormal, with somatosensory and auditory evoked potentials less so. **DDx** CNS sarcoidosis SLE Devic's syndrome: neuromyelitis optica: MS variant with transverse myelitis and optic neuritis (NMO +ve) +MRI Brain NORMAL

Answer 25

McDonald Criteria for diagnosing MS

Answer 26

**Acute relapse** Methylprednisolone 1000 mg intravenously once daily for 3 days If severe --\> plasma exhcnage **Relapsing-Remitting MS** **Immunomodulator**: interferon beta-1a: 30 micrograms intramuscularly once weekly **Medical:** amantadine: 100 mg orally in the morning **Conservative:** regular exercise programmes, good sleep hygien,e practicesene mind-body therapies, such as yoga and relaxation Symptomatic Relief Fatigue: modafinil Depression: citalopram Pain: gabapentin Spasticity: physio, baclofen, dantrolene, botulinum Urgency: oxybutynin ED: slidenafil Tremor: clonazepam

Answer 27

Older female Motor signs at onset Many relapses early on Many MRI lesions

Answer 28

**Inspection:** wasting and fasciculation - esp tongue fasciculation **Tone**: spasticity **Power:** Weak **Reflexes:** Absent or brisk Sensation: NORMAL **Speech** Bulbar: nasal Pseudobulbar: hot-potato **Jaw-jerk** Bulbar: absent Pseudo-bulbar: brisk Eye movements: **MND** does **NOT** involve the eyes

Answer 29

**Cervical cord compression** --\> myelopathy Brainstem lesions **Polio:** asymmetrical LMN paralysis Mixed UMN and LMN = **MAST** **M**ND **A**taxia, Friedrich's **S**ub-acute degeneration of the cord **T**aboparesis

Answer 30

Brain cord MRI to exclude myelopathy EMG: fasciculations Lumbar puncture: exlcude inflammatory causes Revised El Eacorial criteria

Answer 31

**Amyotrophic Lateral Sclerosis: 50%** Corticospinal tracts ---\> **UMN** and **LMN** signs + **fasciculations** **Progressive Bulbar Palsy: 10%** Only affects CN IX - XII ---\> bulbar palsy **Progressive Muscular Atrophy: 10%** Anterior horn cell lesion --\> **LMN** signs only Distal to proximal Better prognosis compared with ALS **Primary Lateral Sclerosis** Loss of Betz cells in motor cortex --\> mainly **UMN** signs Marked spastic leg weakness and **pseudobular** palsy No cognitive decline

Answer 32

Typically LMN signs in arms and UMN signs in legs Both the upper motor neurons and the lower motor neurons degenerate Familial cases: chromosome 21 (superoxide dismutase gene) **Presentation** * Fasciculations (muscle twitches) in the arm, leg, shoulder, or tongue * Muscle cramps * Tight and stiff muscles (spasticity) * Muscle weakness affecting an arm, a leg, neck or diaphragm. * Slurred and nasal speech * Difficulty chewing or swallowing When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS. Other individuals first notice speech or swallowing problems, termed “bulbar onset” ALS Individuals with ALS usually have difficulty swallowing and chewing food, which makes it hard to eat normally and increases the risk of choking. They also burn calories at a faster rate than most people without ALS. Due to these factors, people with ALS tend to lose weight rapidly and can become malnourished. individuals may experience problems with language or decision-making, and there is growing evidence that some may even develop a form of dementia over time. **Mx** **Medical: Riluzole** Drooling: Amitriptyline Dysphagia: NG tube Pain: Analgesic ladder Spasticity: baclofen, botulinum **Breathing support:** NIV prolongs life by 7 months **Conservative:** Physical therapy Speech therapy Nutritional support

Answer 33

**Normal Production of Sound** **Phonation: l**ayrnx and vocal cord **Articulation:** Tongue and mouth Larynx - produces vowels and some consonants. Lips - produce m, b and p. Lingula - l and t. Throat and soft palate (guttural) - nk and ng. Bulbar palsy is the result of diseases affecting the lower cranial nerves (VII-XII) A speech deficit occurs due to paralysis or weakness of the muscles of articulation which are supplied by these cranial nerves. Importantly, these lesions do not affect speech in isolation. The bulbar nerves also innervate muscles involved in swallowing and facial muscles. **Presentation** Lips - tremulous. Tongue - weak and wasted and sits in the mouth with fasciculations. Drooling - as saliva collects in the mouth and the patient is unable to swallow (dysphagia). Absent palatal movements. Dysphonia - a rasping tone due to vocal cord paralysis; a nasal tone if bilateral palatal paralysis. Articulation - difficulty pronouncing r; unable to pronounce consonants as dysarthria progresses. **Mx** Drooling: Amitriptyline Dysphagia: NG tube Pain: Analgesic ladder Spasticity: baclofen, botulinum Conservative: Physical therapy Speech therapy Nutritional support

Answer 34

Affects LMN Presentation * weakness and wasting of muscles in the legs, arms, hands and body * fatigue * muscle cramps and pain * fasciculations * clumsiness * breathing difficulties * weight loss

Answer 35

Prevents stimulation of glutamate receptors Used mainly in **amyotrophic lateral sclerosis** Prolongs life by about **3 months**

Answer 36

Loss of Betz cells in motor cortex --\> Mainly UMN Marked spastic leg weakness Pseudobulbar palsy No cognitive decline Presentation * Problems with balance * Weakness of muscles, especially in the legs * Muscle spasms and cramps * Slurring of your speech * Swallowing difficulties, occasionally resulting in drooling * Emotional lability * Fatigue * Bladder urgency

Answer 37

**LMN Signs** Wasting Fasciculation Hypotonia Hyporeflexia Pathology in anterior horn cell --\> muscle

Answer 38

Motor peripheral poly neuropathy +/- sensory disturbance = Mixed Peripheral Polyneuropathy **DDx** **Chronic** Hereditary motor sensory neuropathy (HMSN) aka Charcot-Marie Tooth Paraneoplastic Lead Poisoning **Acute** Guillain barre syndrome Botulism

Answer 39

**_Proximal Myopathy_** **DDx** **Inherited:** Muscular dystrophy **Inflammatory:** * Polymyositis * Dermatomyositis **Endocrine** * Cushing's syndrome * Acromegaly * Thyrotoxicosis * Osteomalacia * Diabetic amyotrophy **Drugs** * Alcohol * Statins * Steroids **Malignancy:** paraneoplastic

Answer 40

Group of muscles with same supply Segmental: nerve roots / plexus Peripheral: mononeuropathy

Answer 41

**Anterior horn** Syringomyelia MND Polio **Roorts: C8, T1:** spondylosis **Brachial plexus** Compression: cervical rib Avulsion: Klumpke's palsy **Neuropathy** Generalised: HMSN Mononeuritis multiplec: Diabetes melitus Compressive mononeuropathy **Muscle** Disuse: Rheumatoid arthritis Distal myopathy: myotonic dystrophy

Answer 42

**Sensory** Bilateral symmetrical Glove and stocking distribution: length dependent Decreased tendon reflexes: loss of ankle jerks in DM Signs of trauma or joint deformity: charcot joint Loss of propioception --\> +ve rhombergs **Motor** Bilateral symmetrical LMN weakness Wasting and fasciculations Decreased tone Hyporeflexia Completion: review drugs, dipstick urine, gait, CN

Answer 43

**Mainly Sensory** _Diabetes Meltius_ _Alcohol_ B12 deficiency Chronic renal failure and cancer (paraneoplastic) Vasculitis Drugs: isoniazid, vincristine **Mainly Motor** HMSN / CMT Paraneoplastic: Lung cancer, Renal cell carcinoma Lead poisoning Acite: GBS and Botulism **Notes on isoniazid:** Isoniazid binds with active form of pyridoxine called pyridoxoal-5-phosphate to form isoniazid-pyridoxal hydrazone which is excreted in the urine causing pyridoxine deficiency. Dose of 10 mg of pyridoxine for each 100 mg dose of isoniazid prevents pyridoxine deficiency Usually occurs after 6 months of therapy - usually reverses on stopping drug

Answer 44

Diabetes melitus Alcohol = bruning sleep with sheets off

Answer 45

Demyelination ---\> decrease in conduction speed **Axon** degeneration ---\> decrease in conduction **Amplitude**

Answer 46

Charcot Marie Tooth

Answer 47

MDT approach: Gp, neurologist, specialist nurse, physio, OT **Conservative** Foot care Shoe consideration Splinting of joints can prevent contractures **Specifc** Optimise glycaemic control Replace nutritional deficiencies Avoid alcohol Steroids and immunosupressants for vasculitis IVIG for GBS Neuropathic pain: amitriptyline and gabapentin

Answer 48

Inspection: fingers pricks from DM monitoring Peripheral vascular disease **Charcot joints** **Motor** **Bilateral loss of ankle jerks** (2º to sensory neuropathy) Mononeuritis multiplex --\> foot drop **Sensory** Distal sensory loss in stocking distribution **Completion:** fundi, upper limbs and cranial nerves, urine dip Sensory neuropathy Mononeuritis multiplex CNIII CNVI Ulnar nerve

Answer 49

**Metabolic** Glycosylation Reactive oxygen species Sorbitol accumulation **Ischaemia** Loss of vasa nervorum

Answer 50

MDT approach: GP, endocrinologist, neurologist, DNS Good glycaemic control Amitriptyline Gabapentin Capsaicin cream ? not in DM

Answer 51

Also known as Peroneal Muscular Atrophy Also known as Hereditary Motor and Sensory Neuropathy **Inspection** Pes cavus (high arch, fixed plantar flexion) Symmetrical **distal** muscle wasting --\> claw hand --\> champagne bottle legs Thickended nerves esp. common peroneal nerve around fibula **Motor** High-stepping gait: **foot drop** Weak foot and toe dorsiflexion **Absent ankle jerks** **Sensory** Variable loss of sensation in a **stocking distribution**

Answer 52

**HMSN 1** **Commonest form** **Demyelinating** AD mutation in the peripheral myelin protein 22 gene **HMSN 2** Second commonest form **Axonal degeneration** Autosomal dominant Nerve conduction studies can discrimate between the two: Decreased velocity = demyelination = Type I Decreased amplitude = axonal degeneration = Type II

Answer 53

**MDT** Footcare Shoe choice ORthoses: ankle brace

Answer 54

**Inspection:** thymectomy scar **Eyes** Bilateral ptosis: worse on sustained upward gaze Complex opthalmopegia **Facial movements** Myasthenic snarl on smiling **Voice** Becomes nasal Deterioration: ask patient to count to 50 **Limbs** Fatigueability: repeatedly flap arm **Completion:** Assess FVC / respiratory function

Answer 55

**Invx** Anti-AChR, Anti-MuSK EMG: decreased response to titanic train of impulses Tensolin test: imrpovement with edrophonium chloride (no longed used) TFTs: Graves in 5% \<50 female: check for autoimmune disease: DM, RA, GRaves, SLE \>50 male: check for thymoma **Mx** **Acute** Plasmapheresis or IVIG Monitor FVC - ventilation may be required **Chronic** **Pyridostigmine** **Immunosupression:** corticostreoids and azathioprine Surgical: **thymectomy** (removes molecular mimicry)

Answer 56

Antibodies against voltage-gate calcium channels --\> reduces acetyl choline release from nerve terminal Paraneoplastic e.g. SCLC Lower limb girdle weakness - difficulty climbing stairs and rising from chair Areflexia **Weakness improves on repetitive testing**

Answer 57

**Myasthenia gravis** Myotinic dystrophy Congenital Senile Bilateral horner' (rare)

Answer 58

**Classification** **AIDP:** Acute inflammatory demyelinating polyneuropathy **Miller-Fisher:** opthalmoplegia + ataxia + areflexia Molecular mimicry: antibodies cross-react with ganglioside Bacteria: Campylobacter jejuni, Mycoplasma Viruses: CMV, EBV **Presentation** Symmetrical ascending flaccid paralysis Sensory disturbance: paraesthesia (tingling) Autoimmune neuropathy: labile BP **Invx** Stool MC+S Anti-gangliosde antibodies LP: Increased CSF protein Nerve conduction studies: demyelination (reduced velocity) **Mx** **Supportive: 4 As** Airway: ventilation if FVC \<1.5L Analgesia: NSAIDs, Gabapentin Autonomic: vasopressors, catheter Antithrombotis: TEDs, LMWH **Immunosupression:** IVIG, plasma exchange **Physiotherapy**: prevent flexion contractures Prognosis: 85% complete recovery, 10% unable to walk at 1 year, 5% mortality

Answer 59

**Inspection** Unliateral facial droop Absent nasolabial fold +/- absent forehead creases ?scar on parotid mass Rash around external meatus **Weakness** Raising eyebrows: frontalis Screwing up eyes: orbicularis oculi Bell's sign: papebral oculogyric reflex, eyeball rolls back on closure of eyelid (this is normal response 75% but you can't normally see this as eye lids closes --\> becomes noticeable only when the orbicularis oculi muscle becomes weak) Smiling: orbicularis oris **UMN:** sparing of frontalis and orbicularis oculi Due ot bilateral cortical representation

Answer 60

Also kown as ventral pontine syndorme One of teh crossed paralysis syndromes **Pons** CN VI (abducens) nucleus ---\> ipsilateral lateral rectus palsy CN VII (facial) ncuelus ---\> ipsilateral LMN facial palsy Corticospinal tracts ---\> contralteral hemiparesis Causes vary by age Young * tumour * demyelination (e.g. MS) * viral infection (e.g. rhombencephalitis) Older * vascular (basillar artery occlusion /stroke)

Answer 61

Distinct neurological syndrome of deficits that can arise due to the closeness of the cerebellopontine angle to specific cranial nerves **CN V**: Trigeminal nerve **CN VI:** Abducens nerve **CN VII:** Facial nerve **CN VII**I: Vestibulocochlear nerve AND Cerebellar Signs **Signs** Facial anaesthesia + absent corneal reflex Lateral rectus palsy LMN facial nerve palsy Sensorineural hearing loss DANISH **5, 6, 7, 8 and cerebellum**

Answer 62

75% idiopathic Bell's palsy **UMN: likely stroke** **LMN: likely to be Bell's palsy** **Causes:** **Bell's Palsy** **Supranuclear:** Vascular, Multiple sclerosis, SOL **Pontine:** Vascular, Multiple sclerosis, SOL **CPA:** Vestibular Schwannoma, Meningioma, secondary mets **Intra-temporal:** Ramsay Hunt, Cholesteatoma, Trauma **Infra-temporal:** Parotid tumour, trauma Systemic Neuropathy: Diabetes melitus, Lyme disease, Sarcoidosis Psuedopalsy: Myasthenia gravis

Answer 63

Bilateral Bell's Sarcoidosis Guillain Barre Syndrome Lyme Disease Pseudopalsy: myasthenia gravis, myotonic dystrophy

Answer 64

**Hx** Onset: sudden --\> Bell's / Ramsay Hunt Rash around external ear SOL: headache, nause Vertigo, tinnitus, diplopia --\> Other CN involvement **Hyperacusis and Aguesia** Chorda tympani and nerve to stapedius arise just distal to geniculate ganglion within temproal lobe Loss of function indicates prosimal lesion - common in Ramsay Hunt **Invx** Urine dip: glucose Glucose HbA1c Antibodies: Anti-ACh **Imaging** MRI posterior cranial fossa Pure tone audiometry Lumbar puncture: exclude infection Nerve conduction studies: predict delayed recovery **Mx** Prednisolone within 72 hours Valaciclovir if VZV suspected Protect eye: Dark glasses, artifical tears, tape closed ay night

Answer 65

**Incomplete = complete recovery within weeks** **Complete palsy: 80%** get full recovery Remainder have delyaed recovery or permanent neurological / cosmetic abnormalities **Complications** Synkinesis e.g. blinking causes upturning of mouht Crocodile tears: eating stimulated crying

Answer 66

Sudden onset Complete LMN facial palsy Ageusia: cord tympani Hyperacusis: stapedius Assoc with other CNs in 8%

Answer 67

Reactivation of VZV in geniculate ganglion of CN VII Preceding ear pain or stiff neck Vesicular rash in auditory canal +/- TM, pinna, tongue, hard palate No rash = zoster sine herpete Ipsilateral facial weakness, hyperacusis and ageusia May affect CN VIII --\> vertigo, tinnitus and SNHL

Answer 68

**Locally destructive expansion of stratified squamous epithelium** within the **middle ear** **Usually secondary to attic performation of TM in chronic suppurative otitis media** **Presentation** Foul smelling **white** discharge Vertigo, deafness, headache, pain Facial paralysis **Complications** Deafness: ossicle destruction Meningitis Cerebral abscess **Mx** Surgery

Answer 69

**Borellia burgdoferi** **Early: local erythema migrans** Systemic malaise **Late Disseminated:** CN palsy e.g. Facial nerve palsy Polyneuropathy Meningoencephalitis Arthritis Myocarditis Heart block

Answer 70

Absent/decreased sensation in **trigeminal** distribution May be **one or more branches of trigeminal nerve** **Weak masseter and temporalis** Jaw Jerk reflex Brisk = UMN Absent = LMN **Loss of corneal reflex** **Causes** Supranuclear: * Demyelination * Infarct * SOL Nuclear: * CPA lesion (other CN involved) * Lateral Medullary Syndrome (loss of pain and temp ipsilaterally in face and CONTRALATERALLY in trunk) Peripheral mononeuropathy * DM * Sarcoidosis * Vasculitis * Cavernous sinus (opthalmic and maxillary divisions - bilateral)

Answer 71

**The reflex is mediated by:** Nasociliary branch of the **ophthalmic branch (V1) of the 5th cranial nerve** (trigeminal nerve) sensing the stimulus on the cornea only (afferent fiber). **Temporal and zygomatic branches** of the **7th cranial nerve** (Facial nerve) initiating the motor response (efferent fiber).

Answer 72

**Face: PEAS** **P**tosis: partial (superior tarsal muscle) **E**nopthalmos **A**nhydrosis **S**mall pupil No opthalmoplegia Ptosis, Myosis, Anhydrosis **Neck scars:** central lines, carotid endarterectomy **Hands:** complete claw hand and intrinsic hand muscle weakness Decrease or absent sensation in T1 dermatome

Answer 73

**Central** Multiple sclerosis Wallenberg's syndrome / Lateral Medullary Syndrome **Pre-ganglionic (neck)** Pancoast tumour: T1 nerve root lesion Trauma: carotid endarterectomy or central line **Post-ganglionic** Cavernous sinus thrombosis: Usually secondary to spreading facial infection through opthalmic veins --\> CN 2, 4, 5, 6, palsies

Answer 74

**1st-order neuron** Arnold-Chiari malformation CVA/intrapontine haemorrhage Basal meningitis (syphilis) Intrinsic tumour - glioma Syringobulbia/ syringomyelia **2nd-order neuron** Pancoast tumour Occlusion/dissection Tumour Trauma – surgical,birth Thyroid malignancy **3rd-order neuron** Tumour Granuloma Herpes zoster NPC

Answer 75

**Features** **Complete ptosis:** Levator Palpebrae Superioris **Eye points down and out:** unopposed supeior oblique and lateral rectus **+/- Dilated pupil** (does not react to light) Medical CN III palsy: Pupil NOT dilated (normal and reactive) Surgical CN III palsy: Pupul DILATATED * Parasympathetic fibres originate in the Edinger Westphal nuceal and run on periphery of oculomotor nerve * Recieve rich blood supply from external pial vessels: affected late in medical causes

Answer 76

**Medical causes** (pupil sparing) **MMMM** **M**ononeuritis: Diabetes Melitus **M**utlipel sclerosis **M**idbrain infarction: Weber's (CN III palsy + contralateral hemiplegia) **M**igraine **Surgical causes** (Dilated unreactive pupil) **ICP** Raised **I**ntracranial pressure **C**avernous sinus thrombosis **Posterior communicatin artery aneurysm = painful**

Answer 77

Superior cerebellar artery aneurysm

Answer 78

Weber's Syndrome Ipsilateral CN III palsy Contralateral hemiaplegia

Answer 79

Holmes-Adie (Myotonic) Pupil **Dilated pupil that has no response to light** **Sluggish** response to **accomodation** Therefore light-near dissociation Decreased or absent ankle and knee jerks **Benign condition,** more common in young females **Triad:** Dilated pupil (mydriasis) which does not constrict in response to light Loss of deep tendon reflexes Abnormalities of sweating

Answer 80

Previously known as "prostitutes pupil" **Features** Small, irregular pupils Accomodate but doesn't react to light Atrophied and depigmented iris May have sensory ataxiaa -\> Tabes dorsalis Do fasting glucose / random glucose **Causes** Quaternary syphilis Diabetes melitus Accomodate but do not react Light-near dissociation

Answer 81

More commonly known as a **relative afferent pupillary defect (RAPD)** **Suggests optic nerve pathology** Optic nerve is afferent fibre of light-reflex Affected eye constricts from consenual response but dilates as light is shinning directly into eye - swinging light reflex

Answer 82

Decreased visual acuity Decreased colour vision esp. red desaturation Central scrotoma **Pale optic disc** **RAFP** (Marcus Gunn pupil)

Answer 83

**CAC VISION** _Commonest: Multiple sclerosis and Glaucoma_ **C**ongenital Leber's hereditary optic neuropathy HMSN / CMT Friedrich's ataxia Wolfram syndrome, also called DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness) **A**lcohol and other toxins: ethambutol, lead, B12 deficiency **C**ompression Optic glioma, pituitary adenoma Glaucoma Paget's **V**ascular: DM, Giant cell arteritis, thromboembolic **I**nflammatory: optic neuritis - MS, Devic's, DM **S**arcoidosis **I**nfection: herpes zoster, TB, syphilis **O**edema: papilloedema **N**eoplastic infiltration: leukaemia, lymphoma

Answer 84

Retina --\> Optic nerve --\> Optic chiasm (decussation of nasal fibres /temporal fields) --\> Optic tract --\> Lateral geniculate nucleus of thalamus --\> Optic radiation Superior field: temporal Inferior field: parietal --\> Visual cortex

Answer 85

**Retrochiasmic** Greater defect = closer to chiasm OR larger lesion Contralateral Examine for ipsilateral hemiparesis Examine for cerebellar signs **Right:** test for neglect **Left:** test for aphasia

Answer 86

Middle cerebral artery supplies the optic radiation in the temporal and parietal lobes Causes homonymous hemianopia Hemiparesis Higher cortical dysfunction: aphasia, neglect

Answer 87

Posterior cerebral artery supplies occipital lobe and visual cortex Homonymous hemianopia with macular sparing Middle cerebral artery has a branch that supplies part of visual cortex No hemiparesis May have cerebellar signs

Answer 88

**Chiasmatic lesion** Pituitary tumour: compresses from below = descending visual loss Prolactinoma Acromegaly Cushing's Craniopharyngioma: compresses from above = ascending visual loss Supraseller tumour (calcified)

Answer 89

No vision in one eye Check counting fingers Hand movement Light perception Lesion in front of optic chiasm (in between retina and optic chiasm) **Causes** Eye: cornea, vitroeus, retina Optic nerve: neuropathy

Answer 90

Diplopia is worse as the movement proceeds in direction of failed contraction of offending muscle **Cover test** If the outer image dissapears = affected eye

Answer 91

**Failure of ipsilateral adductio**n, eye looking in towards nose **Eye can adduct on convergence** Nystagmus in the ocntralateral **ab**ducting eye, eye looking out Convergent gaze MLF connects nuclei of CN III and CN VI Pontine centre for lateral gaze initiates movement and output to CN III and CN VI via the MLF **Failure of adduction is iplsiateral to MLF lesion** **Causes** **Multiple sclerosis** Infarct: ischaemic or haemorrhagic Syringomyelia Phenytoin toxicity

Answer 92

Diagnosis of exclusion Does not fit a single regular pattern **Causes** Diabetes meltius: mononeuritis multiplex Multiple sclerosis Myasthenia gravis Thyrotoxicosis

Answer 93

Impaired conduction anywhere beyween auricle and round window Canal obstruction: wax, foreign body Tympanic membrane perforation: trauma, infection Ossicle defects: otosclerosis, infection Fluid in the middle ear

Answer 94

Defect in cochlear, cochlear nerve (CN VIII) or brain **Congenital** Alports: SNHL + haematuria Jewell-Lange-Nielsen: SNHL + long Qt syndrome **Acquired** Presbycusis Drugs: gentamicin, vancomycin Infection: meningitis, measles Tumour: vestibular schwannoma

Answer 95

**Impaired speech production** Voice quiet or hoarse Cough: bovine Say Ahhhh: vocal cord tension **Cause** Local cord pathology: laryngitis, tumour, nodule Recurrent laryngeal nerve palsy

Answer 96

**Impaired articulation of sound** Repetition Yellow lorry: test lingual sounds Baby hippopotamus: labial sounds Count to 30: Myasthenia gravis fatigue **Cause** Bulbar palsy: palatal weakness (nasal, donald duck) Pseudobulbar palsy: Difficult with lingual sounds Cerebellar: slurred, drunken speech

Answer 97

Impairment of language production or registration Name three objects: nominal dysphasia Three staged command: receptive dysphasia Repeat sentence: Today is Thursday: tests for conductive dysphasia Expressive dysphasia: Broca's area in frontal lobe ---\> dmaage causes non-fluent speech Receptive dysphasia: Wernicke's area in temporal lobe ---\> fluent but meaningless speach, comprehension imapired Conductive dysphasia: Damage to arcuate fasciculus connecting Broca's and Wernicke's area, comprehension intact, unable to repeat words or phrases

Neurology Flashcards

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