Goals & Objectives
Objectives for Neurology Block
The material provided in this handout is not meant to limit your study and knowledge in the field of neurology. Throughout your training in medicine you will be exposed to a vast amount of material that will complement these learning objectives. Additional material and clinical skills obtained through coursework, PBL, PSD, clinical and research electives should be integrated in your conceptualization of clinical neurosciences and the doctor-patient interaction. Through this integration we believe that you will be able to have a clear understanding of the nervous system and the diseases affecting it.
Review
- Anatomy of the central nervous system (Development and Homeostasis)
- Anatomy of the ventricles (Development and Homeostasis)
- Anatomy of the spinal cord (Development and Homeostasis)
- Anatomy of the cranium (Development and Homeostasis)
- Aneurysm (Cardiovascular)
- Action Potential (Development and Homeostasis)
- Innervation of the bladder (Renal)
- Muscle innervation (Development and Homeostasis)
- Muscle microanatomy (Development and Homeostasis)
- Meningitis (Infection and Host Response)
- HIV (Infection and Host Response)
- Sleep and Sleep Apnea (Pulmonary)
- Autonomic Nervous System (D & H)
Specific Learning Objectives
Anatomy - General
- Distinguish between the central nervous system and the peripheral nervous system
- Define the terms used to describe the topography of central nervous organization (medial, lateral, coronal, saggital, rostral, caudal, dorsal, ventral, superior, inferior, ipsi- and contra-lateral)
- Describe the general anatomical organization of CNS white matter and grey matter (nuclei, cortex, spinal grey matter)
- Compare and contrast the autonomic nervous system and the somatic nervous system.
- Compare and contrast the parasympathetic and sympathetic nervous system.
- Name and identify the meninges: pia mater, arachnoid, dura (including the falx cerebri and tentorium cerebelli)
- Name and identify the main divisions of the central nervous system : cerebral hemispheres, diencephalon, brainstem, cerebellum, spinal cord.
- Name and identify the divisions of the peripheral nervous sytem: cranial nerves, spinal roots, rami, plexuses (cervical, brachial, lumbar, sacral), main peripheral nerves.
- Define dermatomes and myotomes.
Spinal cord
- Identify the gross morphological features of the spinal cord (dorsal roots, dorsal root ganglia, ventral roots, lumbar and cervical enlargements, conus medullaris), cauda equina.
- Name and identify the main tracts in the anterior, lateral and posterior funiculi of spinal white matter.
- Name and identify the main nuclear groups in spinal grey matter (dorsal horn, ventral horn, intermediolateral grey)
Brainstem and Cerebellum
- Identify the anatomical boundaries of the medulla, pons and midbrain
- Name and identify the 12 cranial nerves, including the location of their nuclei, point of exit from the brainstem, main peripheral connections and functions.
- Name and identify the following structures of the medulla oblongata: pyramids, olive, tegmentum, cranial nerve nuclei 5, 8-12, inferior cerebellar peduncles, gracile and cuneate nuclei, internal arcuate fibers.
- Name and identify the following structures of the pons: basis pontis, pontine nuclei, tegmentum, middle cerebellar peduncles, cranial nerve nuclei 5-7.
- Name and identify the following structures of the midbrain: cerebral peduncles, substantia nigra, red nuclei, tegmentum, cerebral aqueduct, periaqueductal grey, cranial nuclei 3-4, tectum.
- Describe the course of major ascending (spino and trigeminothalamic, medial lemniscus) and descending (corticobulbar and corticospinal) tracts in the brainstem
- List and describe major functional segments and nuclei of the brainstem reticular formation
- Identify the following cerebellar structures: folia, vermis, main fissures, hemispheres, tonsils, peduncles, deep nuclei.
Diencephalon
Identify the thalamus and hypothalamus. Name the main nuclei of the thalamus and their functions (subthalamic, VPL, VPM, VA, intralaminar, DM, medial and lateral geniculate)
Cerebral Hemispheres and Cortex
- Name and identify the major fissures and sulci of the cerebral cortex: interhemispheric, lateral (Sylvian) central (Rolandic).
- Name and identify the lobes of the brain and their main constituent gyri on the brain surface: frontal, temporal, parietal, occipital, limbic.
- Identify the major white matter connections within the forebrain (intrahemispheric fibers, interhemispheric fibers, ascending and descending projection fibers, tracts)
- Name and identify the following functional cortical regions: primary motor, premotor, supplementary motor, frontal oculomotor, Broca, olfactory, primary and secondary somatosensory, auditory, Wernicke, primary and secondary visual. Contrast with association cortex.
- Identify and describe the main functional components of the limbic system: parahippocampal and cingulate gyri, hippocampus, septal region, amygdala, fornix, mammilary bodies.
- Name and identify the components of the basal ganglia: caudate nucleus, putamen, globus pallidus, sub-thalamic nucleus. Define the terms lentiform nucleus, pallidum and striate nuclei.
- Describe and identify the main boundaries of the corona radiata and internal capsule.
Vascular anatomy
- Identify the origin and briefly describe the course of the vertebral and carotid arteries in the neck region.
- Identify the circle of Willis and name its major branches.
- Describe the main regions of supply of branches of the anterior circulation (ophthalmic, anterior and middle cerebral) and posterior circulation (basilar, PICA, AICA, superior cerebellar, posterior cerebral)
- Name and identify the main arterial branches supplying the spinal cord.
Histology
- Name and identify the different types of neurons: pyramidal, multipolar, pseudounipolar, bipolar.
- Name and identify the light microscopic features of a neuronal cell: dendrites, soma, perikaryon, Nissl bodies, nucleus, axon hillock, axon.
- Describe the structure and function of the neuronal cytoskeleton: microfilaments, intermediate filaments, microtubules.
- Name and identify non-neuronal cells of the central nervous system: astrocytes, oligodendrocytes, microglia, ependymal cells.
- Describe the light microscopic features of neocortex, deep nuclei, the hippocampal formation, cerebellar cortex, major levels of the brainstem, spinal cord and normal cerebral white matter.
- Name and identify the light microscopic features of a peripheral nerve: epineurium, perineurium, endoneurium, fascicles.
- List and identify cellular structures in a peripheral nerve: myelinated and unmyelinated axons, Schwann cells, perineurial cells, blood vessels, fibroblasts.
- Describe the process of myelination of an axon. Compare and contrast myelination in the CNS and PNS.
- List the different types of nerve fibers found in a mixed peripheral nerve.
- Classify these nerve fibers according to axonal diameter, presence of myelin and function.
- Name and identify the light microscopic and main ultrastructural features of muscle: endomysium, perimysium, sarcolemma, sarcoplasm, thin and thick filaments, nuclei.
- Describe the main histochemical features and function of the 3 major types of muscle fibers (1, 2A, 2B)
- Describe elements which constitute the blood-brain and blood-nerve barriers.
Neurophysiology
- Describe the electrochemical determinants of the resting membrane potential and the generation of an action potential.
- Describe the distribution of ion channels in relation to the fine structure of a myelinated nerve fiber.
- Briefly describe the electrophysiological properties of myelin.
- Compare and contrast the spread of action potentials in unmyelinated and myelinated axons.
- Use this knowledge to explain the phenomenon of conduction block and conduction slowing in demyelination
- List and describe different types of synapses (axo-dendritic, dendro-dendritic, neuromuscular) and basic elements of a synapse (pre and post synaptic elements, cleft)
- Describe the following events in synaptic transmission: action potential spread to the axon terminal, opening of voltage-gated calcium channels, vesicle docking and release, spread of transmitter in the synaptic cleft, receptor binding, neurotransmitter metabolism and catabolism/reuptake.
- Explain the importance of excitatory and inhibitory postsynaptic potentials in relation to the generation of action potentials
- Describe the major classes of neurotransmitters and list important examples in each category.
- Describe the mechanisms by which neurotransmitters may act on postsynaptic elements.
- List the main excitatory and inhibitory neurotransmitters of the CNS and PNS.
- Describe axonal transport (fast and slow anterograde, retrograde)
- Briefly describe the main principles, technique and clinical uses of neurophysiological investigations:
- Electroencephalography
- Evoked potentials (visual, auditory, somatosensory)
- Nerve conduction studies
- Electromyography
Functional anatomy of major neuronal systems
Sensory System
- List and briefly describe the function of peripheral sensory organs (cutaneous sensory receptors, tendon organs, muscle spindles)
- Describe the anatomy of the anterolateral (spinothalamic) pathway: peripheral axons, synapses and point of decussation, central projections. Review the main sensory functions of this system and how they can be tested clinically.
- Describe the anatomy of the lemniscal (dorsal column) pathway: peripheral axons, synapses, point of decussation, central projections. Review the main sensory functions of this systen and how they can be tested clinically.
- Describe the pain-sensitive structures of the head.
- Describe somatotopic organization in relation to sensory pathways.
- Apply anatomical knowledge of sensory pathways to explain patterns of sensory loss: cortical sensory loss, dissociated sensory loss in syringomyelia, sacral sparing of spinothalamic deficit in cases of intramedullary lesions, Brown-Sequard hemicord syndrome.
- Describe proposed descending brainstem-spinal projections which may play a role in modulating pain perception.
- Discuss the “gate-control” theory of pain.
- Define, give examples and explain the probable mechanism of referred pain.
- Review the visual pathways from retinal receptors to occipital cortex. Apply this knowledge to describe patterns of visual impairement (monocular visual loss, homonymous and bitemporal hemianopsia, quadrantanopsia, cortical blindness, visual agnosia).
- Describe afferents of the pupillary reflex and use this knowledge to explain the clinical sign of “relative afferent pupillary defect”.
Motor system
- Distinguish lower and upper motor neurons.
- Define the term motor unit.
- Describe the structure and function of the muscle spindle.
- Discuss the anatomical structures involved in the following reflexes: deep tendon (stretch), inverse myotactic, flexor withdrawal reflexes.
- Describe the anatomical features and function of the direct activation motor pathway (lateral and anterior corticospinal tracts)
- Describe the anatomical elements and main functions of indirect activation motor pathways (reticulospinal, vestibulospinal, rubrospinal, tectospinal)
- Describe the somatotopic organization of motor pathways
- List main inputs and outputs of the cerebellum. Describe clinical signs of cerebellar disease.
- Review the cortical areas involved in movement and their interaction with subcortical nuclei (basal ganglia, thalamus) and the cerebellum.
- Discuss the importance of the cortico-striato-pallido-thalamo-cortical loop in relation to hypokinetic and hyperkinetic movement disorders. Include a mention of the role of the the subthalamic nucleus and of the nigrostriatal pathway.
Specific Cortical functions
- List the cortical regions which underlie language comprehension, repetition and output.
- Describe the major types of aphasia and how they can be tested clinically.
- Identify the main anatomical substrates of memory (medial temporal cortex, diencephalic structures, basal forebrain, frontal lobes)
- Define the different types of memory (e.g.: primary/secondary/tertiary and declarative vs implicit)
- Give an overview of the concept of neural plasticity in relation to encoding of memory.
Neurological disorders
Peripheral Nervous System and its Disorders
- Explain the pathophysiological mechanisms of nerve dysfunction including: functional block, Wallerian degeneration and segmental demyelination.
- Define and describe the three types of nerve injury (neurapraxia, axonotmesis, neurotmesis).
- Describe a clinical approach to the classification of neuropathy (anatomical distribution, time course, type of nerve fiber involved, demyelinating or axonal)
- List and describe common examples of mononeuropathy (median, ulnar and lateral femoral entrapment, radial and peroneal acute compression) and radiculopathy (C5-7, L4-S1).
- Discuss Guillain-Barré syndrome under the following headings: epidemiology, pathophysiology, diagnostic criteria, laboratory features (including CSF findings), clinical course, treatment.
- State the usual mode of inheritance and main clinical manifestations of inherited neuropathy (Charcot-Marie-Tooth disease).
- List the common causes of acquired polyneuropathy.
- Briefly discuss the pathophysiology and clinical manifestations of diabetic neuropathy
Muscle Disease and Myasthenia Gravis
- List the clinical manifestations of muscle disease in the pediatric and adult population
- Give an overview of the classification of muscle disease
- Compare and contrast the clinical manifestations of neuropathy and myopathy
- List the main histologic differences between a myopathic disorder and a neuropathic process, as seen in sections of muscle.
- Give an overview of the electromyographic findings in myopathy and neuropathy.
- Discuss the clinical manifestations and laboratory features of inflammatory muscle diseases (dermatomyositis, polymyositis, inclusion body myositis)
- State the classification of muscular dystrophies, specifying the key clinical features and usual mode of inheritance of the main subtypes (Duchenne, Becker’s, myotonic, facioscapulohumeral, limb-girdle)
- Explain the role of dystrophin in muscle and genetic aspects of dystrophinopathies
- Define myotonia and give examples of myopathic disorders where it occurs.
- Discuss myasthenia gravis under the following headings: clinical features, diagnostic tests, pathophysiology and treatment principles.
Pain Mechanisms, Headache
- Define acute pain, chronic pain
- Distinguish physiological (nociceptive) and pathological (neuropathic) pain
- Describe sympathetically mediated pain
- List and describe the mechanism of action of classes of drugs used to treat pain
- Discuss the pathophysiology of migraine, including the aura and headache phase: spreading depression, vasogenic theory, trigeminovascular system, genetic aspects.
- List neurotransmitters most implicated in the pathogenesis of migraine.
- Describe the clinical features and classification of migraine
- List the classes of medications used in the acute treatment of migraine and in migraine prophylaxis. For each, state the probable mechanism of action.
- Describe the distinguishing clinical features of the following headache subtypes: tension and cluster cephalalgia, trigeminal neuralgia, temporal arteritis, subarachnoid hemorrhage (aneurysmal rupture).
- Describe the clinical features of a headache secondary to an intracranial tumor
- What clinical features may suggest a more ominous secondary cause of headache as opposed to a benign primary headache ?
- Discusss drug-induced headache and medication-related rebound headache.
Myelin, Multiple Sclerosis
- Review the main distinguishing features of CNS and PNS myelin in terms of cellular production and protein composition.
- Discuss the epidemiology of MS (regional differences in prevalence) and its genetic aspects.
- Discuss the pathophysiology of MS
- Describe possible clinical courses of MS.
- Describe and state the clinical relevance of L’Hermitte and Uhthoff phenomena.
- Compare and contrast: normal CNS white matter, acute MS plaque, chronic MS plaque.
- Discuss prognostic factors which may help predict the severity of disability in MS
- Discuss the main clinical criteria for the diagnosis of MS.
- List the main neuroradiological abnormalities in MS
- Describe common CSF and electrophysiological (evoked potential) abnormalities in MS
- List the disease modifying agents currently available for the treatment of MS
Autonomic nervous system
- Discuss the classification of neurogenic bladder disorders
- Discuss the clinical features and anatomical diagnosis of Horner syndrome
Parkinson’s disease and motor system disorders
- Define and describe the following abnormal movements: myoclonus, dystonia, fasciculation, , chorea, athetosis, hemiballism, dyskinesia.
- Compare and contrast clinical features of lesions of the lower and the upper motor neurons.
- Compare and contrast rigidity and spasticity.
- Define tremor and discuss its classification.
- Describe the clinical features of Parkinson’s disease.
- Discuss the late clinical manifestations of Parkinson’s disease.
- Describe the metabolic steps which lead to the conversion of oral L-dopa to dopamine in the nigrostriatal pathway, as well the use of peripheral dopa decarboxylase inhibitors.
- List common side-effects and long term effects of L-dopa therapy.
- List other medications used to treat Parkinson’s disease and state their probable mechanism of action.
- State the differential diagnosis for parkinsonism.
- Describe the mode of action of neuroleptic medications and their effects on the motor system.
- Describe salient clinical and diagnostic features of : Huntington’s disease, Wilson’s disease, tardive dyskinesia, essential tremor, Tourette syndrome, amyotrophic lateral sclerosis.
Infections of the Nervous System
- List common clinical manifestations of meningitis.
- Describe the pathogenesis of infectious meningitis.
- List the common causative organisms for bacterial meningitis in an infant, a child, an adult and an immunosuppressed adult.
- Describe CSF abnormalities in bacterial, viral, TB/Fungal meningitis.
- Discuss the principles of antibiotic management for the acute treatment and prophylaxis of bacterial meningitis.
- State the role of vaccination in the prevention of meningitis.
- List the agents responsible for viral encephalitis.
- Describe the clinical picture of herpes encephalitis and possible long-term complications.
- Discuss the herpes virus family and associated clinical syndromes.
- Describe the mechanism of action of acyclovir and its main toxicity.
- Describe the clinical picture, common pathogenic bacteria and treatment of brain abcess.
- Discuss slow virus infections of the CNS. State the cause SSPE, multifocal leukoencephalothy and Creuzfeld-Jacob disease (prion disorders). List main clinical manifestations of these conditions.
- List common CNS manifestations of HIV infection.
Epilepsy
- Compare and contrast the terms epilepsy, epileptic seizure, epilepsy syndrome
- State the prevalence and overall prognosis of epilepsy
- Describe the chemical nature, distribution, receptors and main functions of GABA and Glutamate
- Discuss the pathophysiology of partial epilepsy
- Discuss the pathophysiology of generalized epilepsy
- Discuss the role of EEG and neuroimaging in the diagnosis of epilepsy.
- Compare and contrast syncope and epileptic seizure
- Compare and contrast a psychogenic and an epileptic seizure.
- Discuss the international classification of epileptic seizures
- List and briefly discuss some examples of partial epilepsy syndrome (e.g benign Rolandic epilepsy) and generalized epilepsy syndrome (e.g. absence, myoclonic).
- Discuss the principles of rational epilepsy management: choice of medication, advantages of monotherapy, dose-related and idiosyncratic side-effects, value of blood level monitoring
- List proposed mechanisms of action of antiepileptic medications.
- Define status epilepticus
Coma and CNS Trauma
- Define consciouness and coma
- Discuss intracranial pressure and the clinical causes and consequences of intracranial hypertension
- Discuss the effects of systemic blood pressure, pO2 and pCO2 on intracranial pressure
- Describe herniation syndromes (subfalcine, uncal, tonsillar) and their clinical manifestations
- Discuss the following patterns of head injury: concussion, contusion, epidural hematoma, subdural hematoma, subarachnoid hemorrhage
- Outline the principles of management of head trauma and spinal cord injury
- Discuss the epidemiology of spinal cord injury and the most commonly affected spinal levels
- Briefly define and describe central cord contusion.
Sleep
- Describe the 4 stages of sleep.
- Discuss physiological and main EEG features of wakefulness, NREM and REM sleep.
- Discuss chronobiology and the need for sleep.
- Give a classification of hypersomnias and insomnias
- List cardinal manifestations of narcolepsy
- Describe the pathophysiology of sleep apnea.
- Define parasomnias.
Tumors
- Discuss the histopathological and topographical classification of CNS tumors.
- Discuss the etiology of brain neoplasms and genetic syndromes frequently associated with brain tumors
- Contrast the general and focal manifestations of cererebral tumors.
- Discuss neuroglial tumors: astrocytoma, glioblastoma multiforme, pilocytic astrocytoma, ependymoma, oligodendroglioma
- List and give salient features of undifferentiated small cell tumors
- Discuss tumors of meninges and nerve sheaths
- Discuss secondary tumors: incidence, usual sites and sources.
Stroke
- Describe the pathophysiological processes involved in electrical failure and cell death during cerebral ischemia. Include a brief review of chemical, ionic and neurotransmitter alterations.
- Explain the excitotoxicity theory and its role in cellular damage
- Describe the process of cerebral blood flow autoregulation and the concept of ischemic penumbra.
- Define TIA, stroke, lacune, ischemic and hemorrhagic infarct, intracerebral hematoma.
- Describe the mechanisms and risk factors for ischemic and hemorrhagic stroke.
- List frequent sites of hypertensive intracerebral hemorrhage.
- Outline a clinical approach to anatomical and etiological stroke diagnosis
- List the main clinical manifestations of stroke in the following arterial territories: MCA, ACA,
PCA, PICA, Basilar. - State the prognosis for stroke recovery.
- Describe the indications and possible benefits of laboratory investigations for stroke: CT, MRI, Doppler, echocardiography, blood coagulation profile, angiography.
- Discuss the mechanisms of action and indications of antiplatelet and anticoagulant medications for stroke
- List main indications and contraindications of tPA and other thrombolytic agents in stroke management.
- Outline the role of carotid endarterectomy for stroke prevention
Dementia and Alzheimer disease
- List the clinical criteria for the diagnosis of dementia
- Classify reversible and non-reversible dementias
- Describe the neuropathology of Alzheimer disease, Pick disease and multi-infarct dementia
- Discuss the main neurotransmitter alterations found in Alzheimer disease
- Discuss the epidemiology of Alzheimer disease
- What is the relationship of physiological senescence to Alzheimer disease ?
- Discuss the role of laboratory investigations in the differential diagnosis of dementia: blood tests, EEG, neuroimaging.
- Discuss the possible contribution of genetic factors in the pathogenesis of Alzheimer disease under the following headings:
- the significance of the AD link with Down's syndrome
- the chromosomal abnormalities that have been described in AD indicating that familial AD is most likely a heterogeneous disorder.
- the role of beta-amyloid precursor protein
- State the rationale for the use of central cholinergic medications and give examples available in current clinical practice
Developmental Brain Disorders
- Give an outline of the embryologic sequence the development of the nervous system
- Discuss disorders of dorsal and ventral induction
- Discuss disorders of cell proliferation and differentiation
- Discuss disorders of cell migration
- Discuss some hereditary metabolic disorders: tay-sachs, phenylketonuria.
- Define cerebral palsy and give a classification of clinical subtypes
- List main neuropathological alterations described in cerebral palsy.
Psychosocial
- Describe how the diagnosis of MS could affect a patient in the following 3 spheres: marital
relationship, work, insurance status (life, disability). - What is the role of emotional stress in producing headaches and how might it be managed?
- What are some of the restrictions on people's lifestyle and quality of life related to epilepsy and
its treatment? - What are some of the social attitudes toward epilepsy? How can the psychosocial stigma of
epilepsy be reduced for individual patients? - What are the ethical and legal aspects of determining mental competency?
- Discuss the resources available to families to enable them to cope with chronic neurological
disease.