Table of Contents Unilateral UMN (pyramidal weakness) Bilateral UMN (pyramidal weakness) Causes of unilateral LMN lesions – work down (nerve root to peripheral nerve)Bilateral LMN (distal weakness)Proximal weakness → Normal sensation Mononeuritis multiplex UMN + LMNCerebellar disease Cranial nerve palsiesMultiple cranial nerve palsiesComplex opthalmoplegiaPtosis Horner’s syndrome Visual field defectsChoreoathetosis Parkinsonism Now try some viva questionsNow for some stations! Unilateral UMN (pyramidal weakness) Work down (brain to cord): Intracranial – stroke, SOL → hemisensory lossBrainstem – stroke, SOL → may be crossed signsSpinal cord – MS, infarct/haemorrhage, SOL, disc prolapse, trauma, syringomyelia, congenital → sensory-level/segmental sensory loss Bilateral UMN (pyramidal weakness) 3M’s MS Motor neurone disease → normal sensationMyelopathy – cord compression (e.g. due to cervical myelopathy, SOL, disc prolapse, paraspinal infection), trauma, transverse myelitis, syringomyelia, congenital → sensory-level/segmental sensory lossOthers – brainstem stroke, hereditary spastic paraplegia, cerebral palsy, HTLV-1, syphilis Causes of unilateral LMN lesions – work down (nerve root to peripheral nerve) Bilateral LMN (distal weakness) Abnormal sensation distally i.e. sensorimotor polyneuropathy ABCDE: AlcoholB12/thiamine deficiencyCharcot-Marie-Tooth, Carcinomas (paraneoplastic)Diabetes, Drugs (e.g. TB drugs, metronidazole/nitrofurantoin, vincristine/cisplatin, amiodarone)Every vasculitis (e.g. SLE, RA, polyarteritis nodosa) and some infections (e.g. herpes zoster, HIV, leprosy, syphilis) Normal sensation i.e. distal motor neuropathy Chronic inflammatory demyelinating polyneuropathyMyotonic dystrophyInclusion body myositis (proximal in legs but distal in arms)Progressive muscular atrophyLead poisoningPorphyria Acute flaccid paralysis Guillain-Barré syndromeSome rare infections (e.g. rabies, polio, West Nile)Cauda equina syndromeSpinal cord shock Proximal weakness → Normal sensation DENIM: Dystrophies – Becker’s/Duchenne, limb girdle, facioscapulohumeral (shoulder, face and truncal weakness) Endocrinological – Cushing’s syndrome, hyper/hypothyroidism, diabetic amyotrophy (lower limbs)Neuromuscular – myasthenia gravis (fatigable), Lambert–Eaton myasthenic syndromeInflammatory – dermato-/polymyositis, inclusion body myositis (proximal in legs but distal in arms), viral myositisMetabolic/congenital/mitochondrial myopathies Mononeuritis multiplex Vasculitis – granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, polyarteritis nodosa, microscopic polyangiitis Autoimmune – RA, SLE, cryoglobulinaemia, Sjögren’s syndrome, paraneoplasticInfectious – Lyme disease, HIV, leprosyOthers – diabetes mellitus, amyloidosis, sarcoidosis UMN + LMN Motor neurone disease (no sensory deficit) Dual pathology (e.g. cervical myelopathy + polyneuropathy)MyeloradiculopathySubacute combined degeneration of the cord (symmetrical UMN signs with absent reflexes, impaired posterior column function, peripheral sensory neuropathy) NB: spinal cord lesions can cause LMN signs at the site of the lesion and UMN signs below the lesion Cerebellar disease MAVIS: MSAlcoholVascular – thromboembolic, haemorrhagicInherited – Friedreich’s ataxia, spinocerebellar ataxia, ataxia telangiectasia SOL Cranial nerve palsies LesionCausesOptic atrophyPost-optic neuritis/MS, arteritic ischaemia (giant cell arteritis), microvascular ischaemia, compression (SOL, raised intracranial pressure), glaucoma, toxins (methanol, ethambutol), neuromyelitis opticaThird nerve palsyMedical (classically pupil-sparing), M’s: Microvascular ischaemia (diabetes), Migraine, MS/autoimmune disordersSurgical (classically painful), C’s: posterior Communicating artery aneurysm (classic cause), Cavernous sinus lesion, Cancer (SOL)Sixth nerve palsy Raised intracranial pressure, microvascular ischaemia, SOL, traumaUnilateral facial nerve palsyBell’s palsy, Ramsay Hunt syndrome, SOL (e.g. acoustic neuroma, facial nerve tumour, meningioma), Lyme disease, nerve infiltration (TB, sarcoidosis, lymphoma), parotid tumour/surgeryBilateral facial nerve palsyLyme disease, sarcoidosis, Guillain-Barré syndrome, amyloidosisOther differentials for bilateral facial weakness: muscular dystrophies, myasthenia gravisBulbar palsy(LMN)Motor neurone disease, brainstem infarct/SOL, Guillain-Barré syndrome, polio, syringobulbia, neurosyphilis Pseudobulbar palsy(UMN)Motor neurone disease, high brainstem infarct/SOL, MS, bilateral internal capsule infarcts, traumatic brain injury, progressive supranuclear palsy Multiple cranial nerve palsies Any = mononeuritis multiplex, leptomeningeal process (e.g. tuberculosis, meningitis), SOLs, trauma, sarcoidosis, Lyme disease, neurosyphilis, vasculitis, botulismCN 3-6 = cavernous sinus lesion, Miller-Fisher syndromeCN 5-8 + cerebellar = cerebellopontine angle lesionCN 9-10+ 11 = jugular foramen syndrome+ 12 = pseudobulbar/bulbar palsy+ Horner’s syndrome + cerebellar + sensory disturbance (ipsilateral face, contralateral body) = lateral medullary (Wallenberg) syndrome Complex opthalmoplegia Work posteriorly (soft tissue to brainstem): Soft tissue – Graves’ diseaseMuscle – mitochondrial myopathyNeuromuscular junction – myasthenia gravis (test fatigability)Multiple CN’s – cavernous sinus lesion, mononeuritis multiplex, MSBrainstem – stroke, SOL, trauma Ptosis UnilateralThird nerve palsy (pupil ‘down and out’, dilated) – complete ptosisHorner’s syndrome (pupil constricted) – partial ptosisIdiopathicBilateralMyasthenia gravisMyotonic dystrophy (frontal balding, facial muscle wasting)CongenitalNeurosyphilis (Argyll Robertson pupils) Horner’s syndrome 1st order (central) – MS, spondylosis, SOL, syringomyelia, stroke/lateral medullary syndrome2nd order (pre-ganglionic) – Pancoast tumour, cervical rib, thyroid carcinoma/goitre3rd order (post-ganglionic) – carotid artery dissection, radical neck dissection Visual field defects Choreoathetosis Chorea – stroke, Huntington’s disease, Sydenham’s chorea, drugs (e.g. antipsychotics, levodopa), HIVHemiballismus – subthalamic stroke (most), SOL, traumatic brain injury, HIVAthetosis – asphyxia, neonatal jaundice, thalamic strokeDystonia – primary dystonia, brain trauma, drugs, Wilson’s disease, PD, Huntington’s disease, stroke, SOL, encephalitis, asphyxia Myoclonus – epilepsy, essential myoclonus, metabolic, psychological, toxins/drugs, SOL, MS, PD, Creutzfeldt-Jakob disease NB: Wilson’s disease can cause any. Parkinsonism Parkinson’s diseaseVascular parkinsonismParkinson-plus syndromes: multi-system atrophy, progressive supranuclear palsy, corticobulbar degeneration, Lewy body dementiaOther causes: antidopaminergic drugs, Wilson’s disease, communicating hydrocephalus, supratentorial tumours Now try some viva questions What is pyramidal weakness? Which type of lesions causes this pattern? Oops! This section is restricted to members. Create an account or log in to continue reading. Subscribe Log In Which conditions can present with both UMN and LMN signs? Oops! This section is restricted to members. Create an account or log in to continue reading. Subscribe Log In Please can you list some causes of unilateral and bilateral facial nerve palsy? Oops! This section is restricted to members. Create an account or log in to continue reading. Subscribe Log In What are the different causes of bitemporal hemianopia? Oops! This section is restricted to members. Create an account or log in to continue reading. Subscribe Log In – Pituitary tumours– Craniopharyngioma– Pregnancy, if there is pituitary hyperplasia– Sella turcica– Saccular aneurysm– Lymphoma– Chordoma [/restrict} Now for some stations! Charcot-Marie-ToothMotor neurone diseaseBells palsyHorner’s syndromeThere’s lots more!