Localising the lesion: “where in the CNS” Learning objectives Definition of CNS and PNS Definition of UMN and LMN Function of each of the cerebral lobes The homunculus Circle of willis and blood supply to the cerebral hemispheres Motor tracts – lateral corticospinal Sensory tracts – lateral spinothalamic and dorsal columns Stroke syndromes Clinical case scenarios Definitions CNS = Brain and spinal cord PNS = anything outside brain and spinal cord Also include autonomic nervous system and cranial nerves Motor control systems Corticospinal (pyradmial) Skilled, intricate, strong and organised movements Defectiveness loss of skilled voluntary movement, spasticity and reflex changes Such as hemiparesis, hemiplegia or paraparesis Extrapyradimal system Fast, fluid movements that the corticospinal system has generated Defectiveness bradykinesia, rigidity, tremor, chorea Such as huntingtons The cerebellum Co-ordinating smooth and learned movement initiated by the pyradimal system and in posture and balance control Defectiveness ataxia, past pointing, action tremor and incoordination The motor system Pyradimal motor system are the tracts of the motor cortex that reach their targets by traveling through the "pyramids" of the medulla. The pyramidal pathways the lateral and anterior corticospinal tracts directly innervate motor neurons of the spinal cord or brainstem of the anterior horn cells. whereas the extrapyramidal system centers around the modulation and regulation of the pyradimal tracts via indirect control of anterior horn cells. Extrapyramidal tracts modulate motor activity without directly innervating motor neurons. The corticospinal system The homunculus UMN vs LMN UMN LMN Wasting no yes Fasciculation no yes Tone increased decreased Power decreased decreased Reflexes increased decreased Plantars up going down going Sensory pathways Peripheral nerves carry sensation from dorsal roots to the cord Posterior columns (dorsal columns) Vibration, joint position, light touch and point discrimination Cross in the brainstem passing to the thalamus Spinothalamic tracts Pain and temperature Cross within the cord and pass in the spinothalamic tracts to the thalamus and reticular formation Sensory cortex Fibres from the thalamus pass to the parietal region sensory cortex and motor cortex Cortical functions Frontal lobe Reasoning, planning, parts of speech, movement, emotions and problem solving Left frontal = broccas area (aphasia) Parietal lobe Movement, orientation, recognition, perception of stimuli Occipital lobe Visual processing Temporal lobe Perception and recognition of auditory stimuli, memory and speech Left temporal = wernicke’s area Cerebellum Balance and co-ordination Basal ganglia Initiation and inhibition of movement Wernickes area – like broccas area is it the understanding of written and spoken speech Circle of Willis Internal carotid artery supplies brain External carotid artery supplies face Middle cerebral artery supplies 1/3rd of brain Vertebral arteries join to form the basillar artery which join at the base of the brain Stroke TACS – All three of Hemiplegia or hemi sensory loss Visual field defect Disturbance of higher function Dysphasia Dysphagia PACS – 2 out of 3 LACS – blockage of small branch of big artery No visual field defect Pure motor stroke Pure sensory Sensory motor Ataxia POCS – brain stem, cerebellum, cranial nerves Bilateral motor or sensory Conjugate eye movement disorder Cerebeller dysfunction Hemiplegia or cortical blindness Acute occlusion of blood vessel leading to hypoxia and infarction Risk factors DM, hypertension, smoking, hypercholesterolemia, FHx, AF Investigations bloods, CT, MRI, carotid dopplers, Echo, ECG, 24 hour tape Treatment in ischaemic stroke Aspirin Clopidogrel Supportive management In ischaemic stroke you have in ischaemic penumbra which is the area of the brain which is damaged during ischaemia in order to reduce the effects from this you need to optimise conditions – temp, BP, glucose Cerebellar syndrome Causes Vascular lesion Alcohol Demyelination Tumours Hypothyroidism Metabolic disorders Signs “DANISH” Dysdiadochokinesis Ataxia Nystagmus Intention tremor Slurred speech, dysarthria Hpyotonia, hyporeflexia Multiple Sclerosis Areas of demyelination and perivascular inflammation (white plaques) Disseminated in time and occurring anywhere within CNS Aetiology - ?autoimmune ?vitamin D deficiency Classification Benign -little disease activity for many years, minimal disability Relapse remitting - most common, repeated attacks with periods of recovery Secondary chronic progressive - continuous progression of symptoms following an initial relapsing and remitting disease course Primary progressive - accumulation of pernament disability over time with superimposed relapses Investigations LP – increased protein, increased immunoglobulin, oligoclonal bands Visual evoked potentials MRI On examination Unsteady gait Reduced proprioception Brisk reflexes Brown-sequard syndrome Loss of movement on same side as damage Loss of pain and temp and sensation on opposite side spinal cord lesion where there is an incomplete lesion characterized by loss of motor function loss of vibration sense and fine touch, loss of proprioception and signs of weakness on the same side of the spinal injury. This is a result of a lesion affecting the dorsal column and the corticospinal tract. On the contralateral side of the lesion, there will be a loss of pain and temperature sensation and crude touch 1 or 2 segments below the level of the lesion Management Symptoms control (tremors, pain, muscle spasms) Steroids - severe relapses to speed up any recovery with will occur naturally. A severe relapse is usually classed as one that has significantly affected activities of daily living Beta-inferons and Glatiramer - reduce rates of relapses by 30% and is only used in relapsing and remitting or relapsing progressive disease IV natalizumab - is a newer monoclocal antibody treatment used in patients with very severe active disease that can reduce relapses by 80%, cost, practical consideration and complications limits its use. Motor neurone disease Degeneration of upper and lower motor neurones of unknown cause 5-10% autosomal dominant Types o Spinal muscular atrophy – limb weakness due to involvement of spinal cord anterior horn cells o Primary later sclerosis – spastic limb weakness due to UMN involvement of the spinal cord o Progressive bulbar palsy – involvement of bulbar motor neurones, progressive disease o Amyotrophic lateral sclerosis – mixture of all the above Investigations o Diagnosed clinically after other causes excluded o EMG confirms fasciculation's and fibrillations Management – symptom control Fatal within 3-5 years Cardiac and smooth muscle aren’t involved and ocular muscle very rarely Autonomic dysfunction occurs late Signs o Dysarthria, brisk jaw reflex o Fasciculation/wasting in deltoids, biceps, quadriceps and in tongue o Weakness in all4 limbs, brisk reflexes in arms, absent in legs Combination of UMN and LMN Clinical case 1 23, female presents to her GP with a 2 week history of bilateral leg weakness having started with pins and needles and numbness in her hands and feet. She has had a few days of urinary incontinence which has resolved. 2 years ago she had an episode of blurred vision and pain in the right eye which lasted a month and fully resolved Diagnosis – MS Visual – optic neuritis, diplopia, nystagmus, internuclear opthalmoplegia, dysarthria, dysphagia, weakness, muscle spasms, ataxia, pain, paraesthesis, fatigue, cognitive impairment, depression, unstable mood Uhthoff’s phenomenum – the worsening of neurological symptoms after periods of exercise and increased body heat Lhermittes sign – an electrical sensation that spreads from the back into the limbs on neck flexion and or extension Bowel problems – incontinence, diarrhoea, constipation Urinary – incontinence, frequency, urinary retention Plaques of demyelination within the CNS caused by an inflammatory process. Different areas of the CNS are involved over time LP – cell count, protein, glucose and oligoclonal bands. WCC less than 50/mm3 MRI Visual evoked potentions – show delayed conduction between the retina and the occipital cortex There is no curative treatment Multidisciplinary team Symptomatic – spasticity, pain, fatigue, depression, continence Steroids, beta interferon, glatiramer, natalizumab Clinical case 2 61 female Becoming increasingly weak on her right side over a one week period. She is unable to walk and has slurred speech and right side of her face is drooping Past history of breast cancer o/e – right facial weakness, grade 4/5 weakness of the right arm and leg, right homonymous hemianopia and some difficulty naming objects and reflexes are brisk on the right side and her right plantar response is upgoing diagnosis = Cerebral maetastases from carcinoma of the breast CT head shows extensive oedema surrounding the subtle impression of a ring enhanced lesion in the left frontal lobe, extending into the left parietal lobe. There is associated mass effect displacing the lateral ventricle Features of raised intracranial pressure it is likely the oedema around the tumour has increased or bleedin has occurred within the tumour Features of raised ICP – visual loss, seizures and focal neurological deficit such as third and 6th cranial nerve palsies Multidisciplary team, neurosurgery, corticosteroids, radiotherapy, chemotherapy Case 3 76 male Background of AF (on warfarin) has 2 hour history of severe global right sided weakness. He is eye-opening to painful stimuli and is moving his left side spontaneously. When questioned he seems confused 12/15 E2, V4, M6 Left hemisphere primary intracerebral haemorrhage causing right sided hemiparesis Bloods tests, CXR, head CT head CT should have been performed within 24 hours or immediately in patients presenting with acute stroke if any of the following apply to them. – on anticoagulation treatment, known bleeding tendancy, decreased consciousness, papliodemea, neckstiffness or fever, severe headache with sudden onset, Ultrasound doppler, cerebral angiography, echocardiography Risk factors – hypertension, smoking, DM, FH, increasing age, previous strokes, vascular disease, hyperlipidaemia, hypercoagulable state, alcohol abuse, malignancy In ishcamic stroke – thrombolysis three hours from obsets are elegible Aspirin, lipid lowering drugs, anticoagulation if patient has AF or other source of embolus Haemorrhaging stroke – supprotive care. neurosurgery Case 4 56 male 6 month history of progressive weakness of his right hand. Also had problems with swallowing and has choked whilst eating on several occasions o/e he has wasting of his upper and lower limbs and some fasciculation's were noted his right plantar was up going and his reflexes were generally brisk Motor neurone disease MRI – to exclude local brainstem pathology EMG – acute denervation of the lower motor neurones Cases were the diagnosis isn’t clear – LP to exclude MS, muscle biopsy to exclude muscle disease. Blood tests for other conditions