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
