Neurology Final Flashcards
Classifications of strokes (4)
1) Extend: Focal or global
2) Time:
transient, reversible, completed, progressing
3) Location: Anterior (carotid), vertebrobasillar
4) Course:
- Macroangiopathy: 50%; arterosclerosis
- Microangiopathy: 20% esp. HTN
- Cardioembolic stroke 20% atrial fib or paradox
- Others: Coagulation diosorderss
Clinical symptoms stroke depending on location
Carotis=Anterior und media (80%)
Basilovertrebal=Post
1) A. cerebri anterior:
- Supplies medial parts
- Lower limb contralateral hemiparesis
- Changed cognition
2) A. cerebri media
- Supplies lateral parts
- Upper limb contralateral hemiparesis/
hemihypesthesia
- Aphasia/Dysarthria (Motor or sensor)
- Apraxia
- Hemineglect
- contralateral homonymous hemianopsia
3) A. Cerebri posterior
- Contralater homonymous hemianopsia
4) A. vertrebralis
- Brainstem or cerebellar infarct
- Cerebellar symptoms and brainstem symptoms including cranial nerves and worse
5) A. basilaris
- Brainstem: Alternating hemiparesis
- Ipsilalateral cranila nerve paresis
- Contralateral peripheral paresis
- Cranial nerve specific symptoms
6) Multiinfarct dementia
- Affects behaviour/emotions (emotional incontinence)
- Parkinson like
- Pseudobulbar syndrome
Causes of ischemic stroke (5)
1) Cardioembolic in atrial fib or paradoxical
2) Atherosclerosis
3) Dissection of Carotid or vertebral
4) Others (fat/air emboli, vasculitis)
5) Cryptogenic
DD ischemic stroke
1) Hypoglycemia
2) Migraine with aura
3) Epileptic seizure with Todd paresis (often patients dont remember and looks like wake up stroke)
4) Infection with paresis
5) Peripheral nerve damage
6) Vestibular neuritis
7) Intoxication
8) Tumor (edema esp oin morning, seizures)
Diagnosis of ischemic stroke
1) CT:
- to exclude hemorrhagic stroke
- the less is visible on CT the better
- perfusion CT to assess age of lesion
2) MRI:
- takes too long but useful to determine extent
of lesion
- st useful in brainstem
3) Digital subtraction angiography
- to assess for thrombectomy
4) US
- to asses vessels
5) Echocardiography
- in endocarditis bc. thats KI for thrombolysis
Therapy of ischemic stroke
ASAP: Time = Brain
Aim of therapy: Save penumbral area
1) Total intensive therapy on stroke unit:
- rehabilitation on first day (speech, physio)
- prevent further complications
2) Recanalization:
- tPA/Alteplase 4,5h after onset (SE and KI)
- Mech. thrombectomy: best but slow, might
be helpful even 24h after stroke (less SE/KI)
3) Treatment and prevention of secondary injury
- Antiedematic: Elevated head, hyperventilation,
osmotherapy, sedation
Primary/Secondary preventio nof ischemic stroke
Primary: HTN, DM, others
Secondary: Treat atrial fib. and anticoagulation (mostly warfarine, st. NOAGs,) and antiplatelets (ASA) if it wasnt cardioembolic
Hemorrhagic stroke classification
1) Typical:
- 80%
- Basal ganglia
- worse prognosis
2) Atypical:
- 20%
- cerebellar or cerebral cortex
- better prognosis
Therapy of hemorrhagic stroke:
1) Total intensive therapy
2) Suppression of bleeding
- Decreasing BP
- Antagonizing Warfarine with Vit K.
- Antagonozing NOAG with ABs
- By Prothromblex
Main causes and secondary prevention of hemorrhagic stroke
1) HTN
2) Amyloid angiopathy
3) AV malformations
4) Tumors
5) Alcohol
6) Stimulants
7) Vasculitides
Prognosis of different kinds of cerebral insults
1) Ischemic stroke: Relatively good
2) Hemorrhagic stroke: 70% ded
3) Subarachnoid bleeding: U ded except if traumatic
Differentiation of ischemic vs hemorrhagic stroke
CT
Hemorrhagic stroke symptoms
1) Basal ganglia
- Contralateral hemiparesis
- Eye deviation twoards lesion
- Aphasia if dominant hemisphere
- Homonymous hemianopsia
2) Thalamus
- Thalamic pain
- Decreased conciousness
- Contralatera sensomotor problems
3) Cerebellum
- Dysarthria
- Nystagmus
- Ataxia
- Vertigo
4) Pons
- Cranial nerve defects
- ARAS: Coma
- Contralateral hemiparesis
Subarachnoid bleeding causes
1) Non traumatic
- Aneurysma (usually in anterior circulation)
- AV Malformations
- Dural malformations
- Endothelial dysfunctions
2) Traumatic
Cushing reflex?
Followign subarachnoid bleeding increased ICP leads to increased sympaticus; increased pressure in baroreceptors leads to increased vagus; leads to cardiac complications
Symptoms of subarachnoid bleeding
1) Disturbances of conc.
2) Vegetative symptoms (vomiting)
3) Obliteration pain
4) Cranial nerve palsies esp occulomotor
5) Mengingeal symptoms
Therapy of subarachnoid bleeding
1) Chill in bed
2) Prevent vasospasm by Ca2+ blockers
- spasms max after 2w, decrease after 4 week
3) Treat hydrocephalus
- usually obstructive or hyporesorptive
4) Clip or coil aneurysms
DD of subarachnoid bleeding
Block of C-Spine Post coital headache Migraine Acute psychosis Meningitis Intoxication
Diagnosis of subarachnoid
CT: Sensitive in first 24 hours
Lumbar puncture: Bilirubin, also good for meningitis
Parkinson symptoms motor and non motor
Motor: - Rigidity - Resting tremor - Hypokinesia - Postural (flexion) - Hypomimia - Dysarthria - Freezing and initiation Non-motor: - Depression - Dementia - Sleep disorders - Psychoses - Sexual disturbances
Therapy of parkinson disease
Mild: MAO inhibitors
Moderate:
Young: Dopamine agonists
Old: L-Dopa + Carbidopa
Severe:
MAO inhibs
COMT inhibs
In late motor symptoms:
Young: Deep brain stimulation
Old: Intrajejunal duodopaDiagnosis of parkinson disease
2/3 of main symptoms (Tremor, Rigidity, Hypokinesis)
L-Dopa test
SPECT rarely
Pathogenesis of parkinson disease
Neurodegen. of substantia nigra with lewy bodies
Alpha-Synnuclein (possibly from gut bacteria via N. Vagus) are misfolded proteins that infect others
Late motor complications of PDs
The later you start with therapy the quicker they appear, depend on state of S. Nigra
1) Fluctuation (On-off, wear off)
2) Dyskinesia (Involuntary movements)
Fixable by continous intrajejunal dopamine or maybe deep brain stimulation
Sideffects of PDs pharmacotherapy
Dopamine:
Peripheral: Nausea and postural hypotension
Central: Anxiety and hallucinations
Late motor symptoms
DD of PDs
1) Vascular dementia
2) Depression
3) NPH
4) Other hypokinetic syndromes
Kinds of parkinson syndrome and epidem.
80% Parkinsons disease 10% Other neurodegenerative - Prog. supranuclear palsy - Multiple system atrophy - Corticocerebellar degeneration - Lewy Body Disease 10% Secondary - Neuroleptics, Antiemetics, Wilsons, NPH
Forms of Parkinson Syndrome according to pathophys
1) Präsynaptic: Can be treated by Dopa, basically only PDs
2) Postsynaptic: Receptors dead, Dopa doesnt help
Normal pressure hydrocephalus symptoms and therapy
Symptoms: Wet, wack, wobbly (urinary incontinence, dementia, gait disturbance)
Therapy: VP shunt, lumbar puncture
Wilsons disease pathophys, symptoms and therapy
Symptoms: Hepatitis, parkinson syndrome but also other neurological symptoms, Kaiser Fleischer Ring
Pathophys: Loss of ceruloplasmin; less copper in bile; accumulation everywhere
Therapy: Penicilamine (chelator)
Prog. supranuclear palsy Pathogenese, symptoms
Atrophy of mesencephalon due to tauopathy
Symptoms:
Eye movement disorder
Dementia
Gait disturbanceMultisystem atrophy
Atrophy of pons, striatum, cerebellum and more
Symptoms:
- Autonomous: Incontinence, sexual,
hypotension
- Parkinsonoid
- Cerebellar
Gait examination what do zou look for
Balance (wide gait, deviation to one side( Symmetry Speed Rythm Regularity Correct movement Arm movements
Normal gait?
Initiation by leaning forward
Flex in hip knee and ankle
Heel support => Footpad support
Gait disorders
1) Ataxic Gait:
- Signs: Broad legs, uncertain, short step,
staggering,
-Types:
Cerebellar - infarct etc.
Proprioceptive - worse when eyes closed
- dorsal column or neuropathy (DM)
Intoxication - alcohol (paleocerebellar)
Vestibular - towards one side
2) Antalgic gait:
- Signs: Main weight on healthy side, shorten
time on affected side (Footballer gait)
- DD: Radicular vs Pseudoradicular pain
(Lassegue + in radicular; Pseudoradicular
usually osteoarthritis of hip or SI Joint
problem)
3) Foot drop gait:
- Peroneal paresis, polyneuropathy, motor
lesion
- One foot hangs, abnormal lifting in hip and
knee, dragging foot behind, stomping
4) Waddling gait:.
- in myopathy causing weakness of hip
extensor
- hyperlordosis with retraction of shoulders an
waddle (like pregnancy
5) Paretic gait:
- Hemiparesis: Circumduction of leg or
whole body rotation, arm in wernicke
- Paraparesis: Shifting weight on walker
- Paraplegia: tense adductors, scissor gate
6) Flaccid paraparesis
- extremly lifted hips
7) Parkinsonian gait
- Gaze to floor
- Shuffling
- Initiation problems
- Small steps
- Flexion everywhere
- Propulsion movement of thorax while feet are
stuck
- Sensory trick.
8) Choreatic
Hyperkinetic syndromes
1) Tremor
2) Chorea
3) Dystonia
4) Tics
5) Myoclonus
Tremor def. and class.
Def: Rythmic oscillating movement bc. of alternating flexions
Class:
1)Position
- Resting tremor (Parkinson)
- Postural tremor (Essential, Hyperthyroidism,
Physiological, accentuated physiological)
- Intention tremor (Cerebellum)
2) Location
3) Frequency
4) Amplitude
Essential tremor stuff (Epidem, Symptoms, Therapy, PRognosis)
Most common cause of tremor, prevalence 5%
Low frequency postural symmetric
Typically hands 90% > head 30% > voice > axial
Alleviated with alcohol
WOrse in stress
Very very slow progression
Therapy: Propranolol, maybe neurosurgery
Accentuated physiological tremor causes
Lithium, TCA, Valproate, Corticoids, Caffeeine
Metabolic causes of tremor
Hyperthyroidism
Cerebellar tremor
Intention tremor Low frequency Asymmetrical depending on side of lesion Together with ataxia, dyskinesia, titubations Therapy very hard
Tremor subtypes (5)
1) Essential tremor
2) Accentuated physiological tremor
3) Endocrine in hyperthyroidism
4) Cerebellar
5) Parkinsonian syndrome
Chorea definition
Random, involuntary, irregular, nonstereotypic, fast (dance like) movements
chorea causes (6)
1) Vascular - sudden onset, one location
2) Drug-induced - (Tardive dyskinesia in long term neuroleptics or on-off phenomena in long term parkinson L-DOPA)
3) Autoimmune chorea: Sydenham chorea after streptococcus or in pregnancy (very rare)
4) Hypo-/Hyperglycemia: reversible “ischemic” lesions, can be focal
5) Thyreotoxicosis
6) Neurodegenerative (Chorea huntington)
Chorea huntington pathogenesis, symptome, diagnose, therapy
Pathophysiology: Too many CAG repetition (>27) in Huntingtin gene leading to atrophy of Ncl. caudatus; AD
Symptoms:
Huntington triad -
Choreatic movement, Cog. impairment, Personality
changes
Emotional, Psychosis, “Motor impersistence”=Cant keep their tongue sticked out
Therapy: Neuroleptica
Tardive dyskinesia
Caused by longterm treatment with esp. typicel neuroleptics
Blepharospasms and facial spasm
RFs: High age, female gender
Young patient with movement disorder, what do you check?
M. Wilson
Neuroleptics can cause possibly which kinetic disorders
Acute: Acute dystonia (in susceptible); also metoclopramid
Chronic: Tardive dyskinesia
Pathogenesis of choreatic disorders examples
Tardive dyskinesia: Long term use of neuroleptica fucks up D-Receptor sensitivity
On-Off Phenomena in Parkinson: L-Dopa after several years leads to fucked up receptors leading to choreatic movement right after taking medication and freezing when concentration goes down
Dystonia def., Classification, Causes, Therapy
Sustained muscle contraction of longer duration, twisted body parts, abnormal posturw
Classification according to: Location: -Focal (Blepharospasm esp in elderly, cervical in younger, laryngeal dysphonia, task specific (writing or musicians)); -Segmental -Hemidystonia -Generalized
Etiology: Idiopathic (Sporadic, hereditary), secondary (drugs, metabolic)
Therapy: Botox, BZs, Anticholinergics (Triphenidyl) for 2 weeks
Myoclonus def, class, therapy
Isolated repetetive muscle jerk, very quicky, one muscle or group of muscles
Classification according to:
Location of spasms
Type: Epileptic vs non-epileptic
Etiology: Often postischemic (after resuscitation)
Location of generator:
- Cortical
- Subcortical
- Spinal
- Peripheral ONLY IN FACIIAL HEMISPASM after bells
paresis
Therapy: Levetiracetam, Valproate
Tics def. symptoms, types, therapy,
irregular movements/vocalizations with urge, can be suppressed temporarily
SImple vs complex
Motor vs Vocal
Worsened by stress
Alleviated by concentrating on st
Simple tics: 25% of children
Tourette: Comb. of vocal/motor changing over time
Very often with comorbidities (ADHD, Depression, OCD)
Therapy: Antipsychotics, BZs, Botox
Difference non-ergot dopamineagonists and ergot-dopamineagonists and one example of non-ergot
Ergot rarely lead to fibrosis, non-ergot almost never
Apomorphin
Symptoms MS
1) Charcots triad (Dysarthria, Intention tremor, Nystagmus)
2) Paresis, spasticity
3) Incontinence, Constipation
4) Visual defects
5) Sensory disturbances (Lhermitte sign: Electric/Coldlike sensation following down spine)
6) Incoordination
7) Mental changes
8) Internuclear Opthalmoplegia ALMOST PATHOGNOMONIC
DD MS
MS is multifocal so almost anything can be DD (Inflammatory, Infections, Vascular disorders, Metabolic, Tumors, Myelopathy)
Diagnosis of MS
1) Plaques in MRT
2) CSF examination with oligocloncal IgG bands
3) Evoked potentials
Treatment
Aimed at slowing progression and reducing symptoms
1) Acute relapse: Corticoids and plasmapheresis
2) Reduce frequency of relapses: IFN beta or some MABs (Natalizumab) (immunosupressive)
3) Immunosupressiva (Methotrexate, Cyclophosphamide)
4) Symptomatic treatment and rehabilitation
Types of MS
1) Primary progressive: Continous, no remissions
2) Relapsing remitting: Attacks that may or may not leave lasting disability
3) Secondary progressive: FIrst relapsing remitting, then progressive
4) Progressive relapsing: Steady decline with superimposed attacks
Def Epilepsy and Epileptic seizure
Epilepsy: Recurrent, unprovoked seizures with high propability of recurrence
Epileptic seizure: transient motor/sensory/autonomic/psychic/behavioural symptoms due to abnormal excessive synchronized neuronal firing
Epidemiology of epilepsy
1% of population
U shaped prevalence
- Young people due to birth injuries or
developmental defects
- Old age due to neurodegen, traumas, strokes
Diagnostic tools for epilepsy
1) History preferably by eye witness of seizure
2) EEG (50% sensitivity)
3) MRI to find focus
4) Video-EEG
EEG in epilepsy
- search for epileptiform discharges (spike and slow wave complexes)
- Location: If over one focus only then focal, if generalized then generalized
- Interictal 50% of EEGs are normal
- Ictal development of seizure can be seen (spikey then wavy then postictal flattening)
Classification of seizures
1) Focal onset - one hemisphere
- Simple vs complex (Awareness)
- Motor vs non-motor (sensory, cognitive,
emotional, autonomic) onset
- Progression to generalized
2) Generalized onset - both hemispheres
- Motor onset (Tonic-clonic, myoclonic, Atonic,
Tonic, Clonic)
- Non-motor (absence)
3) Unknown
- often because focal to generalization or
primary generalized are hard to differentiate
Focal onset seizure
Etiology: Focal lesions (Cortical dysplasia, tumors, stroke)
Might look like generalized in case of
- Very quick generalization of focal
- Frontal lesion leading to complex motor
symptoms
Symptoms: Depend on lesion location
- Occipital: Visual hallucinations
- Frontal: Complex movement
- Temporal: Oral automatisms
- etc.
Generalized seizure
Etiology: Genetic, Metabolic, Large lesions
EEG: Spike/Wave complexes
Absence seizures around 3Hz
Tonic-clonic generalized seizure
Symptoms:
First: Maybe aura
Tonic phase: Sudden loss of conciousness with tonic contraction starting at same time,; leads to falls and epileptic cry and cyanosis, eyes open
Clonic phase: 10-20s after tonic phase starts, tongue biting, loss of bladder control, whole body including face with eyes open and symmetrical bilateral movements in phase; slow recovery of conciousness
Important DD:
- Syncope: Slower onset of unconciousness
Eyes are closed
Keep breathing
Unconciousness at same time as
motor symptoms
Quick recovery of conciousness
Myoclonic seizure
irregular, sporadic jerks usually bilateral, very short lasting
awareness not impaired
may lead to injuries
Absence seizure
Symptoms: Short loss of awareness with abrupt onset and termination
Progression: Usually start in childhood or adolescence and then either become less or progress to tonic-clonic with age
Only seizure where EEG is always positive when patient hyperventilates and is untreated
Good response to treatment
Epileptic syndromes (2)
1) Juvenile myoclonic epilepsy
- Onset and progression:
5-16y: Absence seizures
14-15y: Myoclonic seizures often after
awakening and unnoticed/not taken
seriously
Few months after that: Tonic-clonic
- Seizures precipitated by sleep deprivation/
alcohol leading to typical patient:
- Typical: First party with alcohol and sleep
deprivation leads to first tonic clonic
- Therapy: Well treatable but lifelong
2) Mesotemporal epilepsy with mesotemporal sclerosis
- Onset of focal seizures at 4-16y
- Often pharmacoresistant
- Typical history: Prolonged febrile convulsions as kids or other brain insults
- Temporal symptoms: Automatism and confusion
- Imaging: Unilateral hippocampus atrophy/sclerosis detectable on PET-Scan/MRI
- Therapy: Usually neurosurgery
-
Psychogenic seizures (3) and typical freatures
Can be motor or flaccid
1) Dissociative disorders
- most frequently misdiagnosed as epilepsy
- Patient isnt aware of the seizure and of the
reason of the seizure
2) Factitious disorder
- Patient is intentionally producing symptoms
but dont know why
3) Malingerer
- Simulates to get benefits
Typical features
- Preparation before seizure happens
- Pharmacoresistant
- Very high frequency and length of seizures
- No seizures when alone
- Psychiatric history
- Rotational movement of head (not in clon-tonic)
Diagnosis by Vid-EEG
DD of epilepsy
1) Syncope
2) Psychogenic
3) Metabolic causes
4) Feverish in children
5) Narcolepsy
6) Sleep disorders
Therapy of epilepsy
Focal: Lamotrigin, Levetiracetam and others
Generalized: Valproate, Lamotrigin/Topiromat
Absence: Etosuximid/Valproate
Monotherapy prefered
Stop only after years
Neurosurgical:
- Resection of focus
- Callosotomy
- Vagus stimulation
Status epilepticus
1) Tonic-Clonic > 5min
2) Others > 20min
3) Several attacks without regaining concioussness
Time points:
t1 - 5min after onset = Status epilepticus
t2 - 30min after onset = Irreversible damage
Classification:
motor (tonic-clonic mortality 10-20%)
non motor
Therapy:
1) Maintain vital functions
2) Treat cause (e.g. O2, Hypoglycemia, Cooling)
3) Treat convulsuion
- <15min Diazepam iv./rectal
- >15min Phenytoin,Valproate,Levetiracetam
- >60min Propofol/Thiopental
First aid of epileptic seizure
1) Protect head
2) Remove surrounding danger
Give BZs if possible
COmplications of Status Epilepticus
1) Brain edema
2) Cardiopulmonary dysregulation
3) Consequences of myotonus (Acidosis, Myoglobinemia, Hyperthermia)
Muscular dystrophies (4)
XR
1) Duchenne
- caused by loss of dystrophin
- Symptoms:
Symmetrical muscle weakness leading to Gowers sign (climbing stand up), hyperlordosis, scoliosis, winged scapulae
Pseudohyperthropy of calfs
Trendelenburg sign
Dilatative Cardiomyopathy
Respiratory insufficiency
Wheelchair from around 13y
2) Becker
- same but later and slower progressig
- results from defective dystrophine
AR
1) Limb-girdle
- dominant or recessive
- weakness mainly of shoulder and pelvic
girdle leading to atrophy
2) Facioscapulohumeral MD
- asymmetric weakness of facial and shoulder
muscles (closed eyes, opened mouth)
- winged scapula
- deltoid typically spared
Diagnosis of Muscular Dystrophie
Therapy
Gene analysis
Muscle biopsy
Therapy: Gluccocorticoids maybe Supportive therapy Ventilation at night Surgery against contractures
Myotonia definition, types, warm up effect, paramyotonia
Myotonia: Delayed relaxation of skeletal muscle perceived as stiffness after voluntary contraction (action myotonia) or percussion (percussion myotonia)
Warm up effect: Repeated contractions diminish stiffness
Paramyotonia: Exercise leads to more stiffness
1) Non dystrophic myotonic myopathies
2) Dystrophic myotonic myopathies
Non-dystrophic myotonic myopathies
Channelopathies affecting Na and Cl channels
1) Dominant mytonia congenita (ThoMsen)
- in children: clumsy
- in adults: hypertrophic legs
2) Recessive myotonia congenita (BeckeR)
- same
3) Paramyotonia congenita (Eulenburg)
- Paradoxical myotonia
- Worse in cold
- typical: Delayed eye opening
Therapy: Carbamazepine
Prognosis: Normal life expectancy
Dystrophic myotonic myopathies (2)
Both AD
1) Adult onset/Type 1
Symptoms:
- Atrophy of distal extremities, anterior neck
and face
- Myotonias slight
- no myalgias
2) Prox. myotonic myopathy (PROMM)
- 30-50y
- proximal extremities
- severe myalgias
Extramuscular symptoms:
Catarract
Cognitive decline
Cardiomyopathy
Myasthenia syndromes types
1) Myasthenia gravis
2) Lambert Eaton Myasthenia Syndrome
Myasthenia gravis Epidem/Etiology/Pathogen/Diagnosis/Therapy/symptoms
Epidem:
Women 30-40
Men 60-80
More in women
Etiology:
Autoimmunity vs Acetylcholine receptors or muscle specific tyorosine kinase
Thymic hyperplasia, Thymoma, Paraneoplastic
Pathogenesis:
Blockade and after some time destruction of AchR
Symptoms and Signs:
Occular: Ptosis, Diplopia
Dysarthria, Dysphagia, Swallowing problems
Asymmetric weakness increasing in fatigue
Increased symptoms in stress
Dropping head/Held on hands
Diagnosis: Simpson test: Let them look up to provoke Edrophonium test (AchE inhibit) Electromyography Serum antibodies (Anti AchR/MSTyrKinase
Therapy: Cholinesterase inhibitors (Pyridostigmine), many side effects Corticosteroids (CAVE: Osteoporose) Azathioprin/Cyclophosphamide etc Rituximab Intravenous IGs Plasmaphoresis Thymectomy
SOME DRUGS MIGHT INDUCE MYASTHENIC CRYSIS (Some antibiotics etc.)
Lambort Eaton Myasthenia
Etiology:
Paraneoplastic in SCC
Pathogenesis:
AB vs presynaptic Ca-Channels so no release of ACh
Symptoms/Signs:
Proximal weakness
Autonomic symptoms
Ptosis rarer than in MG
Diagnosis:
Antibodies
Find tumor
Therapy:
Treat tumor
Symptomatic treatment same as for MG
Neonatal myasthenia
Caused by maternal antibodies crossing placental barrier causing symptoms in newborn
Symptoms:
Flaccid, suckling problems
Good prognosis
Myositides (eher 2 als 3)
1) Polymyositis
Etiopathogenesis: Autoimmunity T-Cell mediated, paraneoplastic sometimes
Symptoms:
Muscle weakness: Proximal and cervical
weakness and atrophy
2) Dermatomyositis
Etiopathogenesis: Autoimmunity antibody mediated often paraneoplastic (ovary, colon, breast), more frequent in women
Symptoms: Facial erythematous rash (Purple) Muscle weakness as in (1) Organ involvement (esp. thoracal) Atelectasias on nailbed
Diagnosis: Increased muscle enzymes Autoantibodies MRI/Biopsy LOOK FOR TUMOR
DD: Myasthenia gravis etc.
Therapy:
Gluccocorticoids
Azathiprin/Methotrexate, IVIgs
Amyotrohpic lateral sclerosis
- Progressive degeneration of first and second order neurons with unknown cause
- usually starts 50-60y
- sporadic 90%, familial 10%
Symptoms:
- Atrophy and paresis of distal small muscles, then moves proximally
- Mix of spastic and flaccid problems
- Bulbar and Pseudobulbar symptoms
- Fasciculations/Atrophy of tongue (bulbar)
- Life expectancy 2-4 years, 10% live much longer
Therapy:
- Supportive palliative
- Help against cramps
What does peripheral nerve lesion lead to
Denervation of muscle with fasciculations and atrophy like in bulbar syndrome
Lumbar puncture process
1) Patient either lying or sitting bent over with straight shoulders so spine is not twisted
2) Atraumatic needle at level of Spina iliaca anterior super (
KI of Lumbar Puncture
- Increased ICP (Fundoscopy or MRI)
- Coagulation (Warfarine or oncological)
- Local infection at site of puncture (Varicella)
CSF analysis
1) Normal
- Appearance: Clear
- Cells: <5/microlitre
- Proteins: 0,5g/L
- Glucose: 50% of blood
2) Bacterial
- Appearenace: Cloudy
- Cells: <1000/microlitre (Neutrophils)
- Proteins: >1g/L
- Glucose: Decreased
3) Viral
- Appearance: Clear
- Cells: 100s/microlitre at most
- Proteins and Glucose normal
4) Tbc
- Mono/Lympho/Neutrophils up to 500
- More proteins, less glucose
5) MS
- at most 50 cells
- Oligoclonal bands
6) Guillan-Barée
- increased proteins
7) Subarachnoid hemorrhage
- Erythrocytes either free or in macrophages
- Yellow bc. bilirubin
- Increased glucose and proteins
Bacterial brain inflammations
Etiology:
Neonates: Listeria, S. agalactiae, E. coli
Children: N. meningitides and Pneumococcus
Adults: Listeria und pneumococcus
Others:
- Viral. HSV (Hemorrhagic temporal), Coxsackie, EBV, CMV, Arboviridae, Mumps, Measles, HIV with JC Polyoma (PML)
- Treponema und Borellia
- Fungus: Cryptococcus, Candida, Aspergillus
- Parasitic: Echinococcus, Toxoplasma, Plasmodium, Neurocysticercosis (Taenia Soleum)
- Non-infectious: Lupus, Sarcoidosis, Drugs
- Prions: CJD, vCJD, Kuru
Routes:
- Per continuitatem (Empyema)
- Hematogenic (Abscesse)
Symptoms:
- Nuchal rigidity
- Fever-
- Headache
- Photophobia, Phonophobia
- Nausea/Vomiting
- Seizures, Changed conciousness
Complications:
- Neurologic: Lasting damage, abscess, empyema, edema
- Internal: ARDS, DIC
- Neisseria: Waterhouse-Friedrichsen
Treatment:
Bacterial: Ceftriaxone + Antiedema+ Acyclovir
Viral: Acyclovir
Borellia Stage 1: Doxycycline
CNS Abscess and Epyempa
Symptoms: Focal and Increased ICP
Treatment: Evacuation and antibiotics
CAVE: KI for Lumbar Puncture
Diagnosis of brain inflammation
1) Physical exam (Brudzinsky, Kernig, Lassegue, Meningeal sign)
2) CSF
3) MRI
Diagnosis of brain inflammation
1) Physical exam (Brudzinsky, Kernig, Lassegue, Meningeal sign)
2) CSF
3) MRI
Bulbar syndrome
Reason:
- Lower motor neuron lesion of 9-12
- Due to infarct, tumor, syringobulbia
Symptoms:
- Atrophic and fasciculating tongue
- Nasal speech/Dysarthria (more severe in pseudo)
- Dysphagia (more severe than in pseudo)
- Flaccid paralysis
Pseudobulbar syndrome
Upper motor lesion of 9-12
Symptoms:
- Emotional incontinence/incongruity
- Jaw jerk and gag reflex present
- Tongue not atrophic and no fasciculations
- Dysphagia and dysarthria less severe than in bulbar
Mesencephalon lesions locatgion and szmptoms
1) Weber/Anterior: Corticospinal/bulbar, occulomotor
nerve
- Symptoms
Ipsilateral occulomotor paresis/unreactive
pupi and dilated
Contralateral body and facial paresis
Dysarthria
- Cause: Usually infarct
2) Benedikt/Medial midbrain: Occulomotor, Medial lemniscus, Superior cerebellar peduncle
- Symptoms
Ipsilateral oculomotor fucked
Contralateral dorsal column fuck (Vibration,
Fine touch, Proprioception)
Contralateral ataxia
- Cause: Usually infarct
3) Lesion of fasciculus longitduinals medialis
- Posteromedial location directly near Ncl. oculomotorius leads to
- Lack of coordination of eye and neck movement
Pontine syndromes
1) Anterior: Abducens, Trigeminus, Facialis, Pyramidal tract
- Symptoms:
KL central paresis
Locked in syndrome: Quadriplegia, only motor working is III und IV so eyes except abduction and eyelids still work, sensaation is intact bc. lemniscus medialis is more posteriorly, awareness is intact bc. ARAS is intact
2) Dorsal: Ncl of trigeminus, abducens, facialis, Medial lemniscus, spinothalamic tract
Symptoms:
- Peripheral paresis of cranial nerves
- KL loss of vibration/proprioception/fine touch
- kL loss of pain and temeprature
Medullary syndromes
1) Medial lesion: Medial lemniscues, Fasciculus longitudinalis medialis, Ncl. hypoglossus, Pyramidal tract
- Cause: Occlusion of spinal or vertrebal artery
- Symptoms:
KL loss of proprio/fine/vibration
FLM - Upbeat nystagmus
Ipsilateral hypoglossal paresis
KL spastic paralysis
2) Lateral Ncl of trigeminus, vestibulocochlearis, solitarius, ambiguus, Reticular formation, Lateral spiinothalamic
- Cause: Occlusion of PICA post. inf.
cerebellar. art. or vertebral artery
- Symptoms:
Ipsilateral loss of sensation and corneal reflex
Fall to ipsilateral side, Nystagmus to KL side
Hypogeusia
Ipsilateral paresis of larynx/pharnyx (dysphagia, dysarthria, hoarseness, TONGUE INTACT)
RF - Hiccups
KL loss of thermoception and pain
Def of Dysarthria aphasia apraxia agnosia alexia acalculia agraphia Hemi-neglect
Dysarthria: Impairment of speech caused by motor problems like bulbar syndrome; affects phonation
Aphasia: Impairment of speech bc of central lesions including motor(nonfluent)/sensory(fluent) aphasia; affects language
Apraxia: Difficulty carrying out purposeful or learned movements
Agnosia: Impairment of recognition of sensory stimuli although intelligence, perception etc. are unimpaired
Alexia: Inability to read although visual is fine
Acalculia: Inability to calculate due to parietal lobe
Agraphia: Inability to write
Hemi-neglect: Neglect of on side of the patient
Aphasias (5) + examination
1) Broca/Non-fluent/Motor/Expressive
Lesion: Broca area (Frontal lateral), dominant hemisphere mostly
Symptoms: Mostly intact understanding, usually patient is aware, gramatically non-fluent, cant find words
2) Wernicke/Fluent/Sensory/Receptive
Lesion: Wernicke area (Temporal lobe), dominant hemisphere mostly
Symptoms: Fluent speech without meaning, word salad, neologisms, understanding impaired, patient tbypically unaware
3) Global aphasia
Lesion: Involves broca and wernicke (MCA stroke), arcuate fasciculus
Symptoms: Cant understand, cant speak, utter sounds
4) Transcortical motor aphasiaLesion: Supplementary motor area, might occure during recovery from Broca
Symptoms: Difficulty initiating speech, difficulty expression, echolalia intact
5) Transcortical sensory aphasia
Lesion: Temporal lobe and wernicke area
Symptoms: Impaired speech comprehension and intact echolalia
Examination: Check spontaneous speech, naming objects, let patient repeat
Apraxia (3)
Apraxia: Inability to perform targetted, voluntary movements despite intact motor functions
1) Ideomotor apraxia:
Lesion: Association pathways
Symptoms: Faulty execution of planned action e.g. combing your hair with a spoon (parapraxia), inability to imitate
2) Ideational apraxia:
Lesion: Temporoparietal
Symptoms: Difficulty completing multistep action, usually in wrong sequence
3) Visomotor apraxia:
Symptoms: Difficulty pucking up objects in the contralesional visual field
Agnosia (5)
Agnosia: Impairment of recognition of sensory stimulus (Sensory systems are intact), usually visual
1) Tactile agnosia:
Cant recognize objects by touching
2) Visospatial agnosia:
Inability to orient in space
3) Prosopagnosia:
Inability to recognize faces
4) Autotopagnosia:
Inability to perceive own body parts or recognize them
5) Anosognosia:
Unawareness of neurological impairment
Dysarthrias(5)
- Disorder of mechanical production of speech
- Symptoms: Slurring, mumbling, stakkaton, changes in speed and pitch
Classification according to lesion site:
1) Peripheral lesion:
- affecting articulations muscles: mumbling
- affecting N. laryngeus recurrents: whispering
- Causes: Palsy, Myasthenia gravis, AML,
- Bulbar syndrome (nasal)
2) Cerebellar:
- Ataxic speech (speek like they move)
3) Parkinsonian:
- Hypophonia, montonous
4) Choreatic:
- Explosive, uncoordinated
5) Global:
- Indistinct, slurred due to intoxication
Normal ICP?
<20mmHg
Etiology of increased ICP
1) Idiopathic
2) CNS Inflamma (Inflammation, Abscess)
3) Space occupying (Tumor, Aneurysm, Bleeding)
4) Increased blood (Acidosis in hypervent)
5) Metabolic (Hyponatremia, Hepatic encephalo)
6) Status epilepticus
7) Hydrocephalus (Obstructive, Resorptive); (Normal pressure hydrocephalus)
Consequence of ICP increase
1) Decreased perfusion
2) Herniation (Subfalcine, Transtentorial, Tonsilar, Ascending, Transcalvarine)
3) Cushing triad (Decreased perfusion leading to increased BP leading to Parasympatikus and decreased diastolic pressure == Irregular breathing, bradykardia, increased pulse pressure)
4) Reduced concioussness
5) Headache, Nausea, Vomitting
6) Papilledema
7) Psychiatric changes
Herniations of brain
1) Subfalcine: Cingulate gyrus to other side 2) Transtentorial: Temporal passes tentorium (Ipsilateral occulomotor palsy, infarction of PCA) 3) Foramen magnum herniation Potentially deadly
Therapy of increased ICP
1) 30° elevation
2) Fluid management
3) Hyperventilation
4) Drugs: Mannitol, Corticosteroids
5) VP shunt,
6) Decompressive cranektomie
Meningeal syndrome
Def: Condition marked by fever, headache, stiffness when mengines are irritated
Causes: Bleeding Cancer cells (Breakdown of the cells) Inflammation (Infectious or Lupus) CSF drugs
Ventral/Dorsal spinocerebellar lesion leads to
Dorsal: Ipsilateral ataxia
Ventral: Contralteral ataxia
Spinothalamic tract crosses how?
Anterior: Crude touch
Comes in, goes 3-4 segments up then crosses
Lesion leads to: Ipsilateral loss in 3 segments, contralateral below that
Lateral: Temperature and pain
Comes in, goes up 1 segment, then crosses
Dorsal root syndrome
Initially: Irritative lesion with hyperesthesia and pain ipsilaterally
Destructive leads to anesthesia
Syringomyelia
Abnormal formation of central canal cavity usualyl in cervical area
Etiology:
Primary: Chiari-Malformation, Spina bifida
Secondary: Inflammation, trauma, tumor
Symptoms:
Leads to bilateral loss of pain temperature and crude touch initially,
Then affects anterior horn leading to Lower motor neuron paresis
T1 leads to bilateral horner syndrome
Kyphosis
Syringobulbia in brainstem leading to cranial nerve dysfunction
Diagnosis: MRT
DD: Prolapsed disk, MS, ALS
Therapy:
Shunting
Physiotherapy
Peripheral nerve lesions
1) N. axillaris C5-C6
Causes: Anterior dislocation or surgical neck fracture
Symptoms: Deltoid atrophy and loss of sensation above it
2) N. musculocutaenous C5-C7
Causes: Trauma and Erb’s Palsy
Symptoms: Impaired elbow flex/suppination
3) N. Medianus C5-T1
Causes: Carpal tunnel or supracondylar fracture
Symptoms: Preachers hand, sensation loss of palmar first three fingers, thenar atrophy
4) N. radialis C5-T1
Causes: Humeral shaft fracture, radial fracture
Symptoms: Wrist drop, sensation loss dorsal first two fingers
5) N. ulnaris C7-T1
Causes: Fracture of medial epicondyle or ulnar tunnel
Symptoms: Claw hand, Warthenberg sign (Persistent abduction of 5th finger), 3-5 finger sensation loss
6) N. femoralis (L2-L4)
Causes: Psoas hematoma, Aortic aneurysma
Symptoms: Paralysed quadriceps and iliopsoas (impaired extension in knee and hip)
Sensation loss in anteromedial leg
7) N. ischiadicus (L4-S3)
Causes: Iatrogenic, Trauma, Hip dislocation
Symptoms: Paralysis of hamstrings and of fibularis/tibialis nerves leading to no flexion in knee and drop foot
8) N. tibialis (L4-S3)
Causes: Tibial fracture, Tarsal tunnel syndrome
Symptoms: Paralysis of foot flexors (triceps surae), sensory loss on sole of foot
9) N. fibularis(L4-S2)
Causes: Fracture of fibular head, Compartment syndrome
Symptoms:
Profundus: Loss of foot extensors, flip flop anesthesia
Superficialis: Supination loss
Peripheral neuropathies classification
1) Polyneuropathy (DM, Toxic, Autoimmune)
2) Mononeuropathy - Carpal tunnel etc
3) Mononeuropathy complex - some neurons are affected others not at all
Polyneuropathy
Etiology:
- Metabolic: DM, renal, hypothyroidism, pregnancy
- Toxic: Drugs/Alcohol, Heavy metals
- Inflammatory: , Autoimmune, GB Syndrome, Postradiation, Vasculitides
- Infections: Borrellia, Lepra
- Paraneoplastic esp Lung ca.
- Hereditary: Amyloidosis, Porphyria
- 20% unknown
Symptoms: Hypesthesia/Paresthesia/Dysesthesia peripherally usually symmetrical Burning feet syndrome DM: Cranial nerves, Autonomic stuff Alcohol: Mostly lower limbs
Pathophys types:
- Axonal damage in alcohol/toxic/DM
- Myelin damage in AIDP
Course:
Acute in AIDP (Autoimmune demyelinating polyneuropathy)
Chronic in others usually
Therapy:
Treat cause
Amitryptiline
Chronic inflammatory demyelinating polyradiculopathy (CIDP)
Etiology: Autoimmune
Symptoms:
- Similar to GBS but slower onset and responsive to GCs
- Pure motor and pure sensory subtypes exist
Therapy:
GCs
Plasmapbharesis, IVIG
Guillain-Barré-Syndrome /AIDP)
Etiology: Autoimmune after infection esp after C. jejuni, certain CMV, EBV, Mycoplasma pneumoniae
Pathophysiology: Segmental demyelinisation
Symptoms:
- Symmetrical pain in back and loewr limbs often
- Symmetrical weakness, areflexia, paresthesia
- Can affect respiratory muscles, needs ventilation -(Landry Paralysis)
- Cranial nerve loss esp. peripheral facial bilateral
- Autonomic symptoms
Diagnosis:
- CSF increased protein
- EMS for demyelinisation
- Maybe blood antibodies
Therapy:
- Control respiratory functions
- High dose IGs with plasmapharesis
- GCs ARE NOT WORKING
Complications:
- Autonomic dysfunctions (Arrythmias)
- Pandry paralysis with resp arrest
- Pulmonary embolism
Dementia definition
Progressive decrease of multiple cognitive functions from a previously higher point
Cognitive domains (6)
Memory Language Executive Attention Viso spatial Social cognition
Cortical vs subcortical dementia
Cortical: Memory, Language, Apraxia, Agnosia
Subcortical: Behaviour, executive dysfunctions, psychomotor slowing
Pseudodementia
Looks like dementia (Hypomimia, PM slow etc.)
caused by mainly depression
Epidemiology of dementias
50% Alzheimer 20% Vascular 20% Lewy-Body Dementia 5% Frontotemporal 5% Rest
Atrophy locations of different kinds of dementia
Alzheimer: Parietotemporal
Frontotemporal: Frontotemporal
Lewy-Body: Alzheimer like and subcortical
Vascular: Depends
Alzheimer stuff
Epidemiology: 50% of all dementias
Onset:
- Early onset: <60y: Quick progression, worse prognosis, focal deficits
- Late onset: >60y: Much more common
Pathogenesis:
Intracellular tau protein microfibrillary tangles
Extracellular beta amyloid plaques
Aseptic inflammation and atrophy
Symptoms:
Early: Short term memory problems, depression, amnestic aphasia
Middle: Behavioural disorders, Inability to learn new
Late: Cant take care of themselves, loss of spatial orientation
Diagnose: Anamnesis of caregivers MRI of brain (Atrophy of hippocampus and cortex) CSF (Tau increased, Amyloid normal) MMSE, Clocktest
DD: Mild cognitive impairment -two types (Amnestic, non-amnestic) -amnestic can progress to alzheimer ==> should be treated as early as possible
Therapy:
1) Cognitive therapy:
- ACh-E inhibitors (Donepezile, Rivastigmine)
- NMDA Antagonists (Memantadine)
- Gingko/Vit E
2) Supportive therapy:
- Antidepressants
- etc.
3) Alzheimer groups
Vascular dementia
Either cortical or subcortical or mixed Subcortical is bimswanger Symptoms: - variable depending on location - Emotional and others Treatment: Underlyign cause
Poststroke dementia
30-40% of stroke patients develop depression in first three months
10-20% develop dementia in 1st year
Lewy-Body-Dementia
Epidemiology: 20% of all dementias
Symptoms:
- Parkinsonian symptoms +
- Psychotic problems (visual/accoustic hallucinations) +
- Paranoid delusion/Anxiety +
- Dementia
- Symptoms fluctuate very strong in a matter of hours
CAVE: Agitiation treated by neuroleptics leads to malignant neuroleptic syndrome
Therapy:
Hard, maybe low dose L-Dopa
Neuroleptica can rapidly be lethal
Frontotemporal/Pick’s
Tauopathy Epidemiology: 5% of all dementias, 40-60y Symptoms: - Early behavioural changes - Normal intelligence in beginning - Thus often misdiagnosed as psychiatric illness - Stereotypical movements, compulsivity, - Social disinhibiton - Hyperorality
Normal pressure hydrocephalus
Wack, Wet, Wobbly (Dementia, Incontinence, Frontal gait)
Therapy by VP-Shunt
Slow progression
Diag: MRI with ventricular enlargement
Dementia types
Alzheimer Vascular Lewy-Body (Parkinson and psychotic) Frontotemporal (Behavioural) NPH Others
Atypical parkinsonism syndromes
All dont respond to L-Dopa
All can be with dementia
1) Multiple system atrophy (MSA)
Types:
MSA-P: Parkisonian
MSA-C: Cerebellar
Symptoms:
- Generally: Autonomic (Orthostatic hypotension,
incontinence, erectile dysfunction)
- MSA-P: Parkinsonian symptoms
- MSA-C: Ataxia, cerebellar dysarthria, cerebellar
oculomotor dysfunction
- Others: Respiratory disturbances, contractures, cold hands
- alpha-synnuclein
2) Progressive supranuclear palsy (PSA)
Symptoms:
- Postural instability with backwards falls
- Parkinsonian symptoms
- Pseudobulbar palsy
- Loss of voluntary downward gaze with retained vestibulo-ocular reflex
- Tauopathy
- Micky-Mouse-Sign (Mesencephalic atrophy)
3) Corticobasal degeneration Symptoms: - Asymteric parkinsonian symptoms without tremor - Asym. Muscular symptoms (Myoclonus, Dystonia) - Alien-Limb-Phenomenon - Unstable gait, dysarthria, dysphagia - Blepharospasm - Tauopathy
Friedreich ataxie
Etiopathogenesis:
AR, most common hereditary neurodegenerative ataxia
Gen-defect in Frataxin leads to mitochondrial dysfunction affecting cells with high demand
- Pancreatic beta cells (DM)
- Neurons (Purkyne cells, Spinal tracts)
- Cardiac cells
Symptoms usually start 8-15y:
- Ataxia (Spinocerebellar and dorsal column)
- Cardiomyopathy (Affects cardiomyocytes)
- DM (Beta cells)
- Weakness/Spasticity esp lower limbs
- Blindness (Optic nerve)
- Hearing (Cochlear nerve)
- Kyphoscoliosis
- Hammer toe
- Areflexia
Diagnostic:
Genetic
MRI
Echocardiography
Therapy: Physiotherapy
Disorders of conciousness and structural correlate
Definition: Awareness of self and surrounding, vigilance, alertness, arousability
Quantitative changes: Somnolence, Stupor, Coma
Qualitative: Delirium and Obnubilation
GCS: 15 Points max. 3min Eyes: - 1: No reaction - 2: Reaction to pain - 3: Reaction to command - 4: Spontaneous
Verbal:
- 1: None
- 2: Sounds
- 3: Words
- 4: Disoriented but can talk
- 5: Oriented
Motor:
- 1: None
- 2: Extensors on pain
- 3: Flexors on pain
- 4: Non targetted reaction to pain
- 5: Targetted raction to pain
- 6: On command
ARAS: Ascending reticular activating system
Group of nuclei in pons and mesencephalon projecting to thalamus and cortex, get activated by sensory input like pain, activate cortex
Therapy of MS
1) Acute attack: Glucocorticoid CAVE: Young women with contraception and smoking and GCs ==> Thrombosis so give LMWH
2) Chronic: Escalation principle
- Immunomods (IFN-beta: flu like, skin; Dimethyl fumarate, Glatiramere)
- IVIG (Natalizumab: Anti-Adhesion, stops BBB crossing; Ocrelizumab: CD20)
- Immunosuppressants
Spingosin-1-Phospahte S1P Fingolimode - Lymphocytes are trapped in LNs ==> Lymphocytopenia)
Alemtuzumab - Anti-CD52 which basically kills all lymphocytes (=BM transplant), done in pulses and can lead to several years remission without therapy
Cladribine Adenosine agonist
Methotrexate/Cyclophosphamide
- Autologous stem cell transplant
3) Symptomatic
Spasticity - Baclofen
Sphincters - Catheter
Types of MS
Remittent-Relapsing
Primary progressive
Secondary progressive
Progressive relapsing
MS Symptoms
1) Visual - 30% optic neuritis
2) Somatic - paresthesia/hypesthesias
3) Sphincter
4) Motor - central paresis
5) Cerebellar - Ataxia, balance
6) Depression bc of TFN a increase
7) Cognitive
Uhtoff symptom: Worsening of symptoms if heart
Charcot triad: Nystagmus, Dysarthria, Intention tremor
Lhermitte sign: electrical sensation down spine when bending neck
MS Diagnosis
Must be disseminated in space and time
1) MRI - lesions in typical locations (periventricular, brainstem, cerebellum)
2) CSF - oligoclonal bands and inflammatory markers, lymphocytes
3) Evoked potentials - mainly visual
Dissemination in time: New lesions in new MRI or on same MRI active and resolved lesions (seen with Gd)
Dissemination in space: At least one leasion in at least 2 of the typical areas
Other demyelinating disease except MS
Neuromyelitis optica (NMO) (Devica syndrome)
- Epidemiology: Women, associated with other autoimmune diseases
- Pathogenesis: Autoimmunity against Aquaporine 4 which is espcially expressed on N. opticus and spinal cord (Astrocyted) also other antibodies can be found (against Oligodendrocytes)
- DD: MS
- Symptoms: Optic neuritis, symptoms of spinal cord affection, stronger affection of N. opticus than in MS
- Diagnose:
MRI esp. of spine and N. opticus
CSF
Antibody test
- Therapy:
Acute: GCs, Plasmaphoresis
Chronic: Rituximab, Azathioprin, Methotrexate
MUST BE DIFFERENTIATED FROM MS BECAUSE MS THERAPY MIGHT WORSEN NMO
Contusion vs Concussion
1) Contusion
- Focal bruising of brain with possible
hematoma
caused by strong impact
- Symptoms include loss of concioussness (ICP),
focal neurological deficits depending on
location
- e.g. coup-contre-coup
2) Concussion
- Diffuse injury of the brain without bruising/
evidence
- Symptoms are rather amnesia and confusion
but may be very variable,
- often very mild and may persist for weeks
- Contact sports or car accidents
Focal TBIs
Epidural
Subdural acute
Subdural chronic
Contusion
Diffuse TBIs
Concussion
Diffuse axonal injury
Mechanisms of TBIs
1) Blunt
a) Contact - hit or fall - epidural hematoma, fracture, contusion
b) Acceleration
Linear - Contusion, hemorrhages
Rotational - Concussion, Axonal injury
2) Piercing
a) Low velocity - Only damage in canal
b) High velocity - Damage by shockwave in
whole brain
3) Blast injury
- Blast pressure causes damage
- Can cause all kinds of injuries (Rotational, linear, blunt etc.)
Diffuse axonal injury
Predominant white matter damage caused by shearing forces on axons mainly caued by rotational acceleration as in vehicular accidents or shaken baby syndrome.
Pathomechanism: Stretching of axons, disruption of cytoskeleton, Calcium influx
Symptoms:
Coma very frequent in ARAS damage
Decerebration (vegetative state)
Diffuse damage ==> Diffuse symptoms
Diagnose:
MRI: Microhemorrhages
Therapy: Treat ICP but really bad anyway
Skull fractures
1) Linear: no bone displacement
2) Displaced:
3) Skull base fracture
- Cranial nerve injuries
- Signs: Racoon’s eye, Battle sign, CSF leak through nose
Risk factors that have to be considered if TBIs
Long lasting symptoms
Old age
Coagulopathy or therapy
Therapy of TBIs
1) Mild: Observation
2) Moderate/Severe: ICU and decrease ICP
- Head elevation
- Diuretics
- Osmotherapy
- Surgical hematemectoy
- CSF removal
- Hyperventilation
- Craniectomy
Therapy of TBIs
1) Mild: Observation
2) Moderate/Severe: ICU and decrease ICP
- Head elevation
- Diuretics
- Osmotherapy
- Surgical hematemectoy
- CSF removal
- Hyperventilation
- Craniectomy
Late complications of TBIs
- Postconcussion syndrome (10-30%, headache for several months)
- Seizures
- Lasting neurological deficits
- Depression
- Neurodegeneration
Chronic traumatic encephalopathy (CTE)
After repetetetive mild TBIs in boxers and footballers
Symptoms: Personality changes, cog problems, parkinson, depression
Epidural hematoma
- Usually meningea media
- Lens shaped hyperdense
- Quick development of symptoms: Hemiparesis, unconcioussness, ipsilateral pupillary dilation
- Epidemology: Young people bc. get in fights and dura not so tight with skull
- Therapy: Craniotomy
Subdural acute hematoma
- Crescent shaped hyperdense
- Caused by rotational damages so often together with parenchymal damage => bad prognosis
- Symptoms: more variable than epidural, slower developing
- Therapy: Craniotomy
Subdural chronic hematoma
- Hypodense crescent shaped (bc basically water)
- Clinical presentation is delayed and slowly progressing
- RFs: Coagulopathies, Age (Atrophy increases tension on bridging veins), often not connected to falls, shaken baby
- Symptoms: headache, confusion, hemiparesis, seizures
Therapy: Trepanation
Spinal cord injuries
1) A. spinalis anterior infarct
- Loss of anterior two thirs of spinal cord thus only preservation of dorsal column
- Causes: Thrombosis or spinodural AV fistula
2) Brown-Sequard
- Hemisection of spinal cord leading to
- Ipsilateral loss of dorsal column sensation
- Spinothalamicus anterior (Crude touch) ipsilateral loss for 4 segments, contralteral below that
- Spinothalamicus lateralis (Pain, Temp) ispilateral loss for 1 segment, contraletral below that
- Corticospinalis ipsilateral spastic below, flaccid on same level
3) Syringomyelia
- Spinothalamicus loss below
- Corticospinalis later lost
Drop attack
Sudden fall without loss of conciousness Causes: - CV: Transient ischemic attacks - Neuro: Seizures, Guillan Barré, Ataxia - Myopathies: MG, GBS, - Vestibulopathies: Menieres disease
Brachial plexopathies
Brachial plexus C5-T1
- Complete loss: Atrophy and anesthesia of arm, flaccid paralysis
- Upper lesion (C5-C6): Erb-Palsy
No adduction, ext. rotation and supination
Waiter’s tip position
Sensory loss C5-C6
Etiology: Excessive lateral flexion of neck in
trauma or birth injury
Therapy: Abductionbrace
- Lower lesion (C8-T1): Klumpke
Claw hand
Loss of sensation C8-T1
Horner syndrome ipsilateral
Etiology: Hyperabduction of arm in trauma or
birth injury like braking a fall by holding onto
something; Pancoast tumor; Presence of
cervical rib
Therapy: Splinting hand to correct hand,
physiotherapy
Lumbosacral plexopathies
1) Lumbar plesxus (L1-L4)
- Paresis of Hip flexors, knee extensors, adductors, external rotation
- Loss of sensation in L1-L4
2) Sacral plexus
- Paresis of hip extensors, knee flexors, plantar flexors and extensors
- Loss of sensation
- Trendelenburg sign
3) Lumbar sympathetic trunk
- Anhidrosis of soles and warm feet
Myofascial pain syndrome
Local painful nodule in muscle (Triggerpoint) caused by muscle tension, repetetive motion or spasm leading to local hypoxia
Symptoms: Pain, weakness, Jump-Sign
Therapy: Physiotherapy, massage, ice bag, stretching
Diskopathy
Protrusion - Ncl. pulposus doesnt leave annulus fibrosus but annulus protrudes into spinal canal
Herniation - Ncl. pulposus leaves annulus fibrosus, more extensive protrusion into spinal canal
Sequestration - Piece of Ncl. pulposus breaks off
Epidemiology.
30-50y
Fat, inactive, male, bad posture, hyperlordosis
Location:
60% Lumbar (mainly L5 and S1)
35% Cervical
Rarely thoracal
Pathophysiology:
1) Degenerative: Ischemia and microtrauma leading to tear which can easily be broken
2) Traumatic: Sudden onet due to bad movement
Symptoms:
Pain (Acute, Subacute, Chronic, <6-12< weeks)
Stabbing pain
Pain in back and dermatome (Lumbalgia, Ischialgia, Femoralgia)
Paresis and decreased reflexes
Direction of herniations:
1) Mediolateral/Paracentral:
- 90%
- Bypasses Lig. longitudinalis posterius
- Symptoms of same segment
2) Lateral
- 10%
- Affects one segment above bc these nerves are leaving and going down a bite
3) Central
- rare
Diagnosis:
- Anamnesis (Location, type of pain, event)
- Antalgic gait
- Hard lower back, painful
- Neurological exam
- Lassegue for radicular pain, hip rotation and vertical pressure on femur for Arthritis/SI joint
- MRI
DD: Cauda equina!!! Tumor (Metastases, Hematoma, Abscess, Myelopathys) Vertebral fracture Peripheral nerve lesions
Therapy: Acute: Rest, NSAIDs, maybe myorelaxants/alcohol Conservative: Pharmacotherapy -GCs and Mesocain into perinerve space -Physiotherapy Surgery: -Absolute indication in Conus, Cauda, serious disease -Nucleotomy -Vertebral synthesis
Red flags to distinguish serious pathologies accompanying back pain from “just” back pain
1) Sphincter function
2) Oncological history
3) Immunosuppression/IV drugs/ surgery bc. of abscess
Bladder function normal?
Strong urge at 400ml
Residue >200ml is pathological but consider other causes like pain or unfitting environment
Radicular syndromes
Cervical syndromes: C5 - Deltoid + Dermatome C6 - Biceps + Dermatome C7 - Triceps + Dermatome C8 -
Spondylogenic myelpathies
Caused by degenerative changes leading to osteophytes (usually in overuse)
Symptoms according to affected levels (peripheral + possibly lower levels also affected (centrally)
1) Cervical myelopathy
- Paresis, paresthesia, atrophy of arm
- If sphincter dysfunctions then emergency
- DD: Syringomyelia, ALS
- Therapy: Conservative or fixation
2) Lumbar myelopathy
- Neurological cloudication
- Increased weakness after walking
- Specific position to decrease pain (bend over)
- Provocation by increased lordosis or walking downstairs
- Shopping cart sign
- Peripheral pulse is normal
Babinski fun facts
1) Kids cant walk because they go full babinski when they touch the floor
2) Hallux extends and rest flexes because babinski is a grip reflex (like in hand with babies) and hallux extensor used to be a flexor so now when its activated the flexors activate leading to flexion of 2-5 and extension of 1
Cerebral palsy
Caused by brain damage before or at birth
Non progressive motor/postural disorder
Causes:
Prenatal: Genetic, infection, hypoxia
Perinatal: Hypoxia, hemorrhage, birth injury
Postnatal: Encephalitis, stroke, tumor, metabolic
RFs:
TORCH
Kernicterus
Preterm birth
Classification:
Spastic - 75% - Scissor gate
Nonspastic - choreatic movements, ataxia
Symptoms:
- Ataxia
- Spastic paraparesis/hemiparesis
- Intellectual disability
- Seizures
- Contractures
- ADHD
- Positive babinski
Diagnosis:
US
MRI
Therapy:
Myorelaxants
Surgery for scoliosis
Special therapies
Psychomotor development milestones
1 month: Lift head when on stomach
2 month: Responds to smile, holds head up, follows objects with eyes
3 month: Holds head steady, laughs, recognizes face
4-7 month: Bears weight on legs, sitting, talking
7-10 month: Crawling
12-18 months: walking, very short sentences
Autism and asperger
1) Autism - Severe impairment of development which presents before age of 3 years manifesting in social areas especially.
Typical features:
- Inability to relate, gaze avoidance
- Delayed development of speech
- Cognitive abnormalities esp lack of
creativity
- Stereotyped behaviour: prefer same
environment, plays and movements, small
changes can lead to aggression/depression
75% mental retardation
Ca. 0,3% prevalence, 4:1 boys:girls
Therapy: Mainly special schooling, speech etc.
Can be very demanding for family
2) Asperger’s syndrome - similar to autism but no delay of speech and cognitive function
Boys to Girls 8:1
Spectrum
Headaches classifications
Primary:
- Migraine
- Neuralgia (Trigeminal, Cluster)
- Tension headache
Secondary:
- Trauma
- Substance abuse/withdrawl
- Vasculitides
- Hypertension
- Intracranial disease (tumor, ICP increase)
- Infections (Meningitis, Sinusitis)
- Somatization disorders
1) Migraine
2) Cluster
3) Trigeminus
4) Tension
5) Cervicogenic
6) Drug induced
7) Medication overuse
8) Vascular
Headache epidemiology etc.
Tension: 60-80% of all
Migraine: 15%
Rest: rest
Red flags for headaches
- Obliteration pain
- Signs of increased ICP
- Meningism
- Orbital pain
- Focal neurological deficits
- Fever
Red flags for headaches
- Obliteration pain
- Signs of increased ICP
- Meningism
- Orbital pain
- Focal neurological deficits
- Fever
Tension headache
Duration: Episodic or chronic
Location: Whole head or frontal
Paincharacter: Pressing, not pulsating (schraubstockphenomen)
Intensity: Low to medium
No vegetative symptoms
Triggers: Alcohol, stress, sleep deprivation, cold
Therapy: NSAIDs and massage, Amitryptiline
Migraine
Duration: 4-72 hr, few attacks per month usually
Localization: Usually unilateral
Character: Pulsating, 15% with aura
Intensity: Middle to strong
Other symptoms: Photo/Phonophobia, nausea
Triggers: Hormonal (menstruation), exercise
Pathophysiology: Vasoconstriction with spreading oligemia (aura) leading to aseptic inflammation with vasodilation and pain caused by trigeminus)
Genetic components!
Therapy: Triptane/Paracetamol for acute; amitryptiline/Ca-blockers for chronic
Metoclopramid
Cluster headache
Duration: 30-180min in periods lasting 1-3 months with intermittent months of remission, often dependent on season (spring/autumn); often at night waking up the patient
Intensity: Strong
Localization: Periorbital unilateral or unilateral face
Character: Stabbing
Other symptoms: Ipsilateral horner, lacrimation, rhinorrhea, prominent temporal artery, usually restlessness
Triggers: Alcohol, smoking
Alleviation by heat on eye or temporal pressure
Therapy: Triptane and O2; Chronic; Ca blockers (verapamil)
Trigeminal neuralgia
Types:
Classic: Caused by vascular compression of trigeminal nerve root supply
Symptomatic: Caused by MS or cerebellopontine tumor
Duration: short attacks (2min) in quick succession in periods lasting weeks than can be followed by months of remission
Location: Typically V3, rarely V1, st V2
Characteristic: Extreme sharp pain
Triggers: Chewing, Speaking, toothbrushing, cold air
Other symptoms: Might lead to somatic or motor problems
Therapy: Carbamazepine, Irradiation, Surgery for decompression of vasculature
Cervicogenic headache
Pain originating in cervical or occipital region extending in a band from nuchal region to frontal region, worsened with exercise
Vascular headaches
Intracranial:
- Sinus thrombosis - dull increasing pain
- Epidural/Subarachnoid
- Arteritis temporalis: Pulsating, visible temporal artery
- Stroke
Pain coming from arteries might precede a vascular event like dissection or be simultanoeous with it
Tumorous headache
Progressively worse over weeks/months
Dull pain
With focal symptoms
Signs of ICP
Medication overuse headache (MOH)
In patients suffering from chronic headaches leading to overuse of esp. triptanes/opioids leading to tolerance and eventual chronic headache similar to migraine
Also leads to side effects of drugs like ulcers, renal failure, dependence etc.
Frontal lobe dysfunction
Lateralized syndromes: Left: Depending on location can cause - Hemiparesis - Transcortical motor aphasia - Depression/Anxiety Right: - Hemiparesis - Hemineglect - Mania
Nonlateralized syndromes:
- Fronto-orbital lesion: Disinhibition, confabulations, increased PM activity, sexual impulsiveness, emotional lability
Cingulate gyrus:
- Abulia, apathy
Cortical lobe syndromes
Frontal lobe syndromes Broca Wernicke Temporal epilepsy Occipital dysfunction
Peripheral vestibular syndrome
Causes:
- Vestibular schwanoma, Pontocerebellar meningeoma, other lesions e.g. infarct
Signs: Nystagmus to healthy side Fall to weak side Tonic deviation to weak side Symptoms: Rotational vertigo, nausea
Cerebellum physiology
Functions: Tonus, balance, coordination
Structure:
- Archicerebellum (Flocculonodularis) - Balance (Vestibulocochlear afferents)
- Paleocerebellum (vermis, tonsils) - Muscle tone (Spinal afferents)
- Neocerbellum (Hemispheres) - Movement coordination (Cortical afferents)
Cerebellar syndromes
Usually occur together
1) Paleocerebellar syndrome:
- Axial ataxia (Astasia (bad stance), Abasia (bad gait), asynergy (loss of coordination), titubations, ataxic dysarthria
- DD: Sensory ataxia differentiated by Romberg sign (sensory gets worse with eyes closed)
2) Neocerebellar syndromes:
- Limb ataxia (ipsilateral bc. pathways cross twice)
- Dysdiadochokinesis
- Hypermetria
- Asynergy
- Intention tremor
- Hypotonia
Cerebellar syndromes
Usually occur together
1) Paleocerebellar syndrome:
- Axial ataxia (Astasia (bad stance), Abasia (bad gait), asynergy (loss of coordination), titubations, ataxic dysarthria
- DD: Sensory ataxia differentiated by Romberg sign (sensory gets worse with eyes closed)
2) Neocerebellar syndromes:
- Limb ataxia (ipsilateral bc. pathways cross twice)
- Dysdiadochokinesis
- Hypermetria
- Asynergy
- Intention tremor
- Hypotonia
Cerebellar syndromes
Usually occur together
1) Paleocerebellar syndrome:
- Axial ataxia (Astasia (bad stance), Abasia (bad gait), asynergy (loss of coordination), titubations, ataxic dysarthria
- DD: Sensory ataxia differentiated by Romberg sign (sensory gets worse with eyes closed)
2) Neocerebellar syndromes:
- Limb ataxia (ipsilateral bc. pathways cross twice)
- Dysdiadochokinesis
- Hypermetria
- Asynergy
- Intention tremor
- Hypotonia
Cerebellar syndromes
Usually occur together
1) Paleocerebellar syndrome:
- Axial ataxia (Astasia (bad stance), Abasia (bad gait), asynergy (loss of coordination), titubations, ataxic dysarthria
- DD: Sensory ataxia differentiated by Romberg sign (sensory gets worse with eyes closed)
2) Neocerebellar syndromes:
- Limb ataxia (ipsilateral bc. pathways cross twice)
- Dysdiadochokinesis
- Hypermetria
- Asynergy
- Intention tremor
- Hypotonia
Cerebellar syndromes
Usually occur together
1) Paleocerebellar syndrome:
- Axial ataxia (Astasia (bad stance), Abasia (bad gait), asynergy (loss of coordination), titubations, ataxic dysarthria
- DD: Sensory ataxia differentiated by Romberg sign (sensory gets worse with eyes closed)
2) Neocerebellar syndromes:
- Limb ataxia (ipsilateral bc. pathways cross twice)
- Dysdiadochokinesis
- Hypermetria
- Asynergy
- Intention tremor
- Hypotonia
Cerebellar syndromes
Usually occur together
1) Paleocerebellar syndrome:
- Axial ataxia (Astasia (bad stance), Abasia (bad gait), asynergy (loss of coordination), titubations, ataxic dysarthria
- DD: Sensory ataxia differentiated by Romberg sign (sensory gets worse with eyes closed)
2) Neocerebellar syndromes:
- Limb ataxia (ipsilateral bc. pathways cross twice)
- Dysdiadochokinesis
- Hypermetria
- Asynergy
- Intention tremor
- Hypotonia
Cerebellar syndromes
Usually occur together
1) Paleocerebellar syndrome:
- Axial ataxia (Astasia (bad stance), Abasia (bad gait), asynergy (loss of coordination), titubations, ataxic dysarthria
- DD: Sensory ataxia differentiated by Romberg sign (sensory gets worse with eyes closed)
2) Neocerebellar syndromes:
- Limb ataxia (ipsilateral bc. pathways cross twice)
- Dysdiadochokinesis
- Hypermetria
- Asynergy
- Intention tremor
- Hypotonia
Cerebellar syndromes
Usually occur together
1) Paleocerebellar syndrome:
- Axial ataxia (Astasia (bad stance), Abasia (bad gait), asynergy (loss of coordination), titubations, ataxic dysarthria
- DD: Sensory ataxia differentiated by Romberg sign (sensory gets worse with eyes closed)
2) Neocerebellar syndromes:
- Limb ataxia (ipsilateral bc. pathways cross twice)
- Dysdiadochokinesis
- Hypermetria
- Asynergy
- Intention tremor
- Hypotonia
CNS Tumors classification
WHO 1: Pilcyotic astrocytoma Meningeoma Neurinoma Hypophyseal adenoma Craniopharyngiom Plexus papilloma
WHO 2:
Diffuse astrocytoma
Ependymoma
Oligodendroglioma
WHO 3: Anaplastic astrocytoma Anaplastic meningeoma Anaplastic oligodendroglioma Anaplastic ependymoma
WHO 4: Glioblastom Primary CNS lymphoma Medulloblastoma Germinoma Metastases
Medulloblastoma
- Highly malignant from neuroectoderm
- Most frequent CNS tumor in children
- 4th ventricle/cerebellum
- Increased ICP, Ataxia
- Metastasizes into spinal cord via drop-down
- Resection, Radiation, possibly AV shunt
- Prognosis: 10y 40-60%
Ependymoma
- Ependymoma cells
- children and adolescents
- usually 4th ventricle
- Hydrocephalus with headache
- Drop down metastases
- Resection/Radiation
- Prognosis depending on Grade (1 or 3)
Craniopharyngioma
- from Rathke-Pouch
- Children or >50y
- Supra- or intrasellar
- Headaches, Hypophyseal insufficiency (anterior: Growth disturbance, posterior Diab. insipidus)
- Resection, hormonal replacement
- often rezidive
Metastases to brain
1) Lung (SCC)
2) Breast
3) Melanoma
- Headaches and pareses
- Resection and radiation, GCs
Astrocytomas
Pilocytic:
- Children
- Infratentorial esp. cerebellar and optic nerve
- Often with ataxia
- Resection curative
Diffuse:
- 35y
- Usually frontal lobe
- Radiation + Resection
Anaplastic:
- 35y
- Supratentorial
Often with seizures
Glioblastoma
- 60y
- 10-15m survival
- Hypodense center, hyperdense surrounding
- Butterfly sign from corpus callosum
Oligodendrogliomas
- 40-50y
- frontal lobe
- standard signs + seizures
- resection/radiation/chemotherapy
- 5-10y survival
Meningeomas
Grade I or III
- Elderly
- Often involve adjacent cones
- Usually falx cerebri, tentorial, N. opticus or anywhere
- Can occlude vessels
- Can be resected often or maybe just watchful waiting
Pituitary adenomas
- usually if <1cm then hormonally active, if larger then not
- Symptoms:
Intrasellar - hormonal (Gigantism, Cushing, Hyperthyroidism, Amenorrhea/Prolactin); can cause too much or too little hormones
Suprasellar - bitemporal hemianopsia
Other symptoms - ICP, pain
Neurinomas esp. accoustic
Schwanomma of vestibular part of VIII Symptoms: Loss of 5/7/9/10 cranial nerves Tinnitus Cerevellar ataxia Often with neurofibromatosis 2
Primary CNS lymphoma
- NHL B type usually DLBCL
- Often focal neurological signs
- Often in immunosuppression
- Corticosteroids for edema, radiation
General symptoms of CNS tumors(5)
Behavioural changes Headache Nausea vomiting/Increased ICP Seizures Focal neurological deficits
Therapy of CNS tumors generally
Symptomatic: Antiedema (GCs, Mannitol) Antiepileptic Antithrombotic Pain
Grade I:
Resection usually cure, st radiation after
Grade II:
Rseection can be curative but usually with adjuvant radiation; CHT for Oligodendroglioma
Grade III:
Resection and Radiation;
Grade IV:
EEG Waves and application
Delta: <4Hz, deep sleep; P: Absence seizure
Theta: 4-8 Hz, drowsiness
Alpha: 8-13 Hz, relaxed closed eyes
Beta: 13-30 Hz, Active concentration
Gamma: >30 Hz, strong concentration, meditation
Use: Epilepsy diagnosis Brain death Depth of coma Sleep medicine Localization of lesions
Evoked potentials
1) Visual evoked potentials: MS
2) Brain steam auditory evoked potentials
- Hearing assesment, cerebellopontine angle lesions, perioperative monitoring during surgery
3) Somatosensory EPs: Peripheral nerve lesions
4) Motor EPs: Pyramidal tract lesions, intraoperative monitoring
EMG
Localization (proximal, distal, conduction block)
Classification (axonal, demyelination)
Evaluation of demyelinisation and NMJ disorders
E.g. in MS, Guillan Baré, MG, Entrapment
Relative afferent papillary defect
Optic neuritis
Sleep disorders examination methods
- Anamnesis esp of third party
- Sleepy anamnesis
Time of goign to sleep and sleep delay
Night: Continuity, snoring, abnormal behaviour
Morning: Awakening, feeling rested
Daytime: Wakefulness, planned naps, unintentional sleep - Epworth-Sleepiness-Scale: How likely are you susceptible to fall asleep in following situations 0-4
- Sleep diary
- Polysomnography
Has at least EEG, eye movements, muscle
activity
Can also have O2, EKG, Video
Result is hypnogram giving phases of sleep
Sleep disorders classificagtion
1) Insomnia
- Primary/Psychosocial
- Secondary (Drugs, Addiciton etc.)
2) Sleep-Breathing
- Sleep-Apnoe-Syndrome (central/peripheral)
- Sleep hypoventilation in NM disorders
3) Central hypersomnias
- Narcolepsy with cataplexia
- Narcolepsy without cataplexia
- Idiopathic hypersomnia
4) Parasomnias
- NREM (confusional arousal, sleep walking, night terror)
- REM (Rem sleepy behaviour disorders RBD, sleep paralysis, nightmare disorders)
5) Movement disorders
- Restless Leg Syndrome
Insomnia
Primary/Secondary
Primary has vicious cycle (Insomnia -> fear of insomnia -> insomnia)
Therapy: CBT
Obstructive sleep apnea
Peripheral: Due to obstruction of airways
Central: Brainstem doesnt activate breathing
Differentiation by thorax movement analysis
Peripheral much more common
Diagnosis:
- Polysomnography and history
- more than 5 arousals/hour
Treatment:
Weight loss
Orthodontic or surgical
CPAP (continous positive airway pressure)
Sleepy hypoventilation in neuromuscular disorder
Weak respiratory muscles (typical obese)
Symptoms are present day and night but worse at night
Therapy: Ventilation (different than CPAP)
Narcolepsy (with or without cataplexy)
Narcolepsy
- Excessive daytime sleepiness without ability to resist falling asleep
- Naps are refreshing (usually very short)
- Automatisms when falling asleep
- Hypnogogic hallucinations + sleep paralysis
- Naps have REM phases which is pathological
- Therapy:
Plan naps
Ritaline/Methylphenidate
Cataplexy: Antidepressants
Cataplexy
- Loss of muscle tonus in response to emotional triggers (mostly suprise, laughter, joy)
- They dont loose conciousness
- Tonus is regained after <1min
- Usually symetrical
- Typically focal (face, hands, knees)
- Eye movements, breathing
- Pathogenesis: Loss of hypothalamus neurons containing hypocretine
- Diagnose: Decreased hypocretine in CSF
Idiopathic hypersomnia
- Increased daytime sleepiness
- Naps are NOT refreshing
- Problems with awakening mainly, sleep 18 hrs or so
- Therapy: Methylphenidate
Parasomnias NREM
- Confusional arousal (Sleep drunkness, confusion, automatic behaviour)
- Sleep walking (Eyes open!)
- Night terrors
Pathophys: Incomplete awakening from NREM
Symptoms: Complex seemingly aimed but absurd behaviour
Therapy: Sleep hygiene, Clonazepam
Parasomnias REM
REM sleep behaviour disorders
- Realization of movements perceived in the dream in reality
- Problem is no inhibition of lower neurons
- Typically dream of attacks or st.
- Injury common
- Can later tell what they dreamt about
- Eyes closed! Non oriented in space!
- Maybe prestage of a-synnuclein neurodegenerative disorders (PD;MSA;Lewy-Body-Disease) ==> might allow early therapy
Sleep paralysis
Night mare disorders
Therapy: Clonazepam, SSRIs
Night movement disorders
Restless-leg-syndrome
- Unpleasant feeling in limb, mainly leg
- Moving alleviates feeling
- Gets worse at night, interferes with sleep
- Therapy: Low dose L-DOPA or anticonvulsants
- Might be secondary to iron deficiency so check ferritin
Nocturnal epilepsy
- Happens at night surprisingly
- Symptoms: Headache, Wetting bed, fracture, concussion, bite on tongue, might lead to serious injuries or suffocation
Spinal cord lesions
Autonomic dysfunctions:
Acute spinal shock leads to loss of autonomic functions below the level of the lesion, C4 and above leads to respiratory arrest, first loss then recovery of reflexes
Cord transection:
Flaccid paralysis, anesthesia and areflexia below the lesion, spinal shock
Motor and sensory impairment might improve 6 weeks if incomplete transection leading to spastic paresis
Incomplete lesions: Brown-Sequard: - Ipsilateral spastic paresis, vibration and proprioception loss - contralateral pain, temperature loss two segments below the lesion (dissociated sensory deficit) Anterior Horn Syndrome Posterior horn syndrome Anterior spinal artery syndrome: - Loss of everything but dorsal column Posterior column syndrome
Posterior cord syndrome
- Paresthesia, vibration and proprioception loss
- Lhermitte sign
- Rhomberg sign
Cervical cord lesion:
- Upper: First loss of arms innervation, later also of lower limbs,
- Lower: Might spare arms, might have Horner
Thoracic cord lesions
Lumbar/Sacral lesions
Myelitis
Viruses causing parestehsias and pareses Tables dorsalis Polio anterior motor neuron VZV in dorsal roots Often from vertebral osteomyelitis
Guillan-Barré-Syndrome/AIDP
AIDP=Acute inflammatory demyelinating polyneuropathy, a subtype of GBS
Etiology: Autoimmune, often following infections e.g. C. jejuni, M. pneumoniae, Vaccines
Pathogenesis: Demyelinisation of PNS
S&S
- Ascending, symmetrical, rapidly progressive polyradiculoneuropathy with weakness, areflexia, sensory abnormalities and sometimes pain
- Cranial nerve pareses; Facialis often bilateral
- Other sinclude: 3,4,6,9,10
- Respiratory muscle paresis
- Autonomic dysfunction e.g. arrythmias, hypotension, etc.
Diagnosis:
- EMG: Dispersed and prolonged signals
- CSF: Increased proteins
Therapy:
- Treat symptoms esp. respiration and autonomic
- IVIG/Plasmapharesis
- NOOOO GLUUUCCOOOCORTICOIIIIIDS!!!!!
CIDP
Chronic inflammatory demyelinating polyneuropathy
Similar to AIDP but slower progression, responsive to GCs
Course:
- Progressive in elderly
- Relapsing in younger
- some subtypes affect only motor or only sensory
- Treatment:
GCs, IVIG, P,asmapharesis, Immunosuppression
Types of polyneuropathy
1) Axonal damage: DM, Alcohol, Drugs
2) Demyelinating: AIDP
Causes of polyneuropathy
1) Metabolic: DM, Hypothyroidism, renal
2) Toxic: Alcohol, Drugs (Platin CHT)
3) Inflammatory: AIDP
4) Paraneoplastic in DM
5) 20% unknown
6) Genetic: Hereditary sensorimotor neuropathy (Charcoat-Marie-Tooth disease
Symptoms of polyneuropathy
Motor - Paresen
Sensory - Paresthesias, Balance problems,
Hypesthesias
Autonomic - Arrythmias, Hypotension
Mixed - most commonCharcot-Marie-Tooth disease
- Several subtypes inherited in different fashions
- Two most common types:
Hypertrophic type leading to myelin hypertrophy and resulting neuronal death
Atrophic type with no myeline
Spinal cord and cerebellar neurodegenerative diseases1
1) Friedreich ataxia
- AR
- early onset
- mitrochondrial defect
- Symptoms
Scoliosis
Cardiomyopathy
DM
Loss of dorsal column thus ataxia, gait disturbances, hyporeflexia
2) Spinocerebellar ataxia
- AD
- loss of Purkyne cells
- middle age onset
- Gait disorders, nystagmus,
Nerve entrapments
Carpal tunnel syndrone
Guyon canal syndrome
Ulnar canal
Ischadicus
Development of nervous system
First year: Maturation of CNS
Myelinisation completes by 6th year
Cerebellar functions complete by 6th year
Development leads to
Motor functions from automatisms to voluntary
Disappearance of primitive reflexes
Mental development
Medical history for psychomotor development
Prenatal: TORCH, Gestoses, smoking, alcohol
Perinatal: Prematurity, Asphyxia, Apgar score,
hypogylcemia
Postnatal: Febrile, traumas, convulsions
Apgar score: Skin, Pulse, Face, Muscles,
Respiration
Examination of neonates
Spontaneos, passive and provoked movements
Positions: Suppine, Prone, Pulled to sit, horizontal and vertical suspension
Head circumference
Fontanelle size
Motor development
6m - rolling on belly 6-8m - sitting 9m - crawling 10m - standing with support 12-15m - walking
Psychological development
3rd month: Vocalization, social smile
6th month: Syllables, vivid facial expressions
9th month: Double syllables, react to own name
1st year: Speech development, comprehension
Primitive developmental reflexes
Generated by brainstem or spinal cord, not yet inhibited by higher systems Abnormal - if asymmetric, - absent early or - present too long
1) Feeding reflex up to 6th month
2) Palmar grasp up to 6th months
3) Moro reflex - Extension and abduction of arm and fingers in response to being laid on back (Also in West Syndrome)
4) Reflex stepping
Pathologies of infant age
1) Hypotonia: Myasthenia, Botulinum
2) Hypertonic: Extrapyramidal syndromes
3) Cerebral palsy/Perinatal encephalopathy:
- Chronic non-progressive
- due to perinatal damage (Prematurity, Asphyxia, TORCH etc.)
- Damage is non progressive but symptoms change according to level of development
- Delayed psychomotor, epilepsies
- Types: Spastic, dyskinetic, hypotonic, mixed
4) Hydrocephalus
- obstructive due to malformations, bleedings, infections
ADHD
Symptoms:
- Hyperactivity
- Inattention
- Impulsivity
Subtypes:
- Inattentive 30%
- Hyperactive/Impulsivity 20%
- 50% Mixed
Etiology:
- 50% genetic
- 30% perinatal
- 20% environmental
Pathophys:
pathophys:
- Abnormal prefrontal cortex and basal ganglia
- Dopamine/Noradrenaline dysbalance
Therapy: 1) Nom pharm Educate, Pachyotherapy, Special school 2) Phama Methylphenidate, Atomoxetin
Comorbidities:
Depression Anxiety OCD
Autism
Epidemiology:
0,1%, Boys»_space;Girls
Diagnoisis:
Impairment of social behaviour
Impairment of communication
Restricted repetetive/stereotypic behaviour
Clinial: First three years Typical caus efor first examination is - speech delay - Autistic regression (esp communicating)
Comorbidities
40% Epilepsy
Aspergers:
mild symptoms, no mental retardation
Peripheral motor Neuron problems
1) Muscle: Myopathien
2) NMJ: Myasthenic
3) Nerve: Mono/Polyneuropathien
4) Anterior root: Polio, ALS,
