●General Neurology
| ☉Dermatome: |
Forearm: C 6-7-8 (Thumb-...)夾 T1在內側
Foreleg: L 4-5 S1 (Toe....)夾 S2在後內側(popliteal fossa)
C5: △ delta m.
C6: 2 Biceps m. BSR
C7: 3 Triceps m. TSR
C8: 4 Interosseous m. Horner syndrome |
| ☉Brainstem reflex: |
1.light reflex: 2,3
2.corneal reflex: 5,6,7
3.oculocephalic reflex(doll eye sign): 3,6,8
4.vestibuloocular reflex: 3,6,8
5.pharyngeal and tracheal stimulation: 9,10 |
| ☉Frontal lobe syndrome: |
1.Defective attention and object recognition.
2.Reduction in memory
3.Difficulty in solving problem
4.Insociality
5.Apathy and/or distractibility
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| ☉Angular gyrus syndrome |
(Gerstmann's syndrome):
(left dominant parietal lobe)
1. finger agnosia
2. agraphia
3. right-left disorientation
4. acalculia
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| ☉Right parietal lobe injury |
1.Anosognosia(Hemineglect)
2.Dressing apraxia
3.Geographical agnosia
4.Constructional apraxia
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| ☉CTG repetition: |
Myotonic muscular dystrophy
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| ☉antiParkinsonism: |
1.A: Amantadine
2.B: MAO-B inhibitor
3.C: anti-Cholinergic
4.D: levo-Dopa |
| ☉GCS: M: |
local(5五的台語)ize,
with(4)draw,
decorticate(flexion)（只有皮質）:3
decerebrate(extension)（整個大腦）:2 |
| ☉Wilson's dz |
→ATP7B gene mutation,
→DXL: 1.ceruloplasmin < 20mg/dl 2.Copper on liver biopsy↑
→KF ring: Descemet's membrane |
| ☉Migraine |
→Triad: premonitory visual sensory/motor symptoms, unilateral throbbing
headache, nausea and vomiting
→Trigger: wine, cheese, chocolate, contraceptives, stress
→Onset: early
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| ☉Tourette Syndrome (TS) |
→most debilitating tic disorder, and is characterized by multiform,
frequently changing motor and phonic tics.
→The prevailing diagnostic criteria include onset before the age of 21;
recurrent, involuntary, rapid, purposeless motor movements affecting
multiple muscle groups; one or more vocal tics; variations in the
intensity of the symptoms over weeks to months (waxing and waning); and a
duration of more than one year.
→Since the 1960's, haloperidol (Haldol) has been the mainstay of treatment
for TS
→Pimozide (Orap) was approved in this country for treatment of TS in 1984,
and is now in fairly common use. Pimozide is a diphenylbutylpiperidine,
chemically distinctive from haloperidol or phenothiazines, with potent
dopamine blocking properties.
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| ☉Antiepileptics |
Valproate(Depakine): 對所有seizure有效（V代表勝利），Carbamazepine, Phenytoin對Absence,
myoclonic 無效！！
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| ☉Pregnacy with seizure: |
folic acid supplement |
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●Neuromuscular disease
| ☉Acute Weakness (<1 week) |
1.Postinfectious Guillain-Barre: lower to upper
→Albumincytological dissociation: CSF protein ↑↑ in 2wk
→AIDP: acute inflammatory demyelating polyneuropathy
2.Diphtheria: cranial
3.Porphyria: from arm
→heme biosynthesis disturbance
→CNS dysfunction, skin sensitive to sunlight
→colic abd. apin, portwine colored urine, neurologic/psychiatric disorder
→Dx:urinary porphobilinogen (PBG↑), r-aminolevulinic acid(ALA↑)
★Fresh urine may darken on standing because PBGs polymerize to
uroporphyrin and porphobilin |
| ☉progressive supranuclear palsy |
(Steele-Richardson-Olszewski syndome)
→about 8% of patients with clinical diagnosis of Parkinson disease
→median age of onset = 64 yrs (range 50-77)
→core clinical syndrome
1.supranuclear ophthalmoplegia: "dirty-tie" sign (can't see that they are
dropping food when they eat....)
2.akinesia (presents as frequent falls)
3.rigidity
4.nuchal dystonia
5.pseudobulbar palsy (dysarthria, dysphagia)
6.cognitive abnormality leading to dementia
→imaging:
atrophy of midbrain (substantia nigra) and pontine tegmentum with
abnormally enlarged prepontine cistern
variable atrophy of globus pallidus
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| ☉Lateral Medullary Syndrome |
→Wallenberg's Syndrome ,PICA Syndrome ,Occlusion of the Posterior Inferior
Cerebellar Artery (PICA)
→Onset is usually acute with severe vertigo due to involvement of the
region of Deiter's nucleus, which may result in falling.
→Nausea, vomiting, ipsilateral ataxia, mucular hypertonicity, pastpointing,
lateropulsion, and other cerebellar signs are often present.
→Pain and facial paresthaesia, a coarse spontaneous homolateral nystagmus,
hiccough, dysphagia, dysphonia, dysarthria, and diplopia may be evident.
→Horner's syndrome is usually present.
→Sensory disturbances include ipsilateral loss of pain and temperature
perception of the face and contralateral hypoesthaesia for pain and
temperature of the trunk and extremities. The affected persons have
difficulty in swallowing. →Persons well over 40 years of age are most
often affected. |
| ☉Brown Sequard syndrome: |
布朗西瓜
1.Interruption of the lateral corticospinal tracts
→Ipsilateral spastic paralysis below the level of the lesion, Babinski
sign ipsilateral to lesion
2.Interruption of posterior white column
→Ipsilateral loss of tactile discrimination, vibratory, and position
sensation below the level of the lesion
3.Interruption of lateral spinothalamic tracts（只有這個最怪）
→Contralateral loss of pain and temperature sensation. This usually occurs
2-3 segments below the level of the lesion.
|
| ☉Central cord syndrome |
→Impair: upper extremities> lower extremities (lateral corticospinal
tracts:arm→trunk→leg)
→Sacral sparing
→Dissociated sensory loss(only bil. pain & temp defect) |
| ☉Cauda Equina Syndrome |
→Low back pain
→Acute or chronic radiating pain
→Unilateral or bilateral lower extremity motor and/or sensory abnormality
→Bowel and/or bladder dysfunction
Usually with associated saddle (perineal) anesthesia (examiner can inquire
if toilet paper feels different when wiping)
Bladder dysfunction may present as incontinence, but often presents
earlier as difficulty starting or stopping a stream of urine. |
| ☉Eaton-Lambert syndrome |
→immune-mediated, myasthenia-like syndrome with weakness usually affecting
the limbs and sparing ocular and bulbar muscles.
→presynaptic, resulting from impaired release of acetylcholine from nerve
terminals.
→IgG antibody is involved.
→precede, occur with, or develop after the diagnosis of cancer.
→most commonly in men with intrathoracic tumors (70% have small or oat
cell lung carcinoma).
→Symptoms and signs include fatigability, weakness, sometimes pain in
proximal limb muscles, peripheral paresthesias, dry mouth, impotence, and
ptosis. Deep tendon reflexes are reduced or lost.
→The diagnosis is confirmed by finding an incremental response to
repetitive nerve stimulation: Amplitude of the compound muscle action
potential increases > 200% at rates > 10 Hz. |
| ☉Tensilon TEST |
→an injection of edrophonium chloride used to diagnosis myasthenia gravis
(MG).
→Tensilon blocks the action of an enzyme, acetylcholinesterase. By
blocking the action of acetylcholinesterase, Tensilon prolongs the muscle
stimulation, and temporarily improves strength. Increased strength
following an injection of Tensilon strongly suggests a dignosis of MG.
→Only useful in patients with objective, preferably measurable, findings
on physical examination , Rarely helpful in the diagnostic evaluation of
equivocal cases of MG
→Sensitivity for MG is relatively low (60%) compared to other diagnostic
tests
→False positive results: Can occur in patients with LES, ALS or even
localized, intracranial mass lesions
→Positive testing does not necessarily predict respose to a longer-acting
anti-AChE drug
|
| ☉Gower's sign |
→Duchenne's muscular dystrophy.
→On attempting to stand from the ground the child:
begins with both hands and feet on the floor
then works his way up the legs with his hands until in an upright posture
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| ☉Autonomic dysreflexia (AD) |
→syndrome of massive imbalanced reflex sympathetic discharge occurring in
patients with spinal cord injury (SCI) above the splanchnic sympathetic
outflow (T5-T6).
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●Cerebrovascular disease
| ☉Imaging |
CT: 如T2
MRI: 一水黑，二水白，油骨血（肉骨茶？）反之
DWI: for recent infarct
|
| ☉MRI |
Acute hematoma: T1 low, T2 low
Chronic hematoma: T1 high, T2 high
Subacute hematoma: T1 high, T2 low
Infarction: T1 low, T2 high |
| ☉CT Contrast enhancement |
infarction: 6-13 days(14-21 days: fogging effect)
hemorrhage: "Rim enhancement" : 11 days- 2 months
|
| ☉CSF xanthochromia: |
bleeding & hemolysis eg. spinal AVM |
| ☉possible causes of accelerated vascular disease: |
(1) endothelial cell damage,
(2) smooth muscle cell proliferation,
(3)
lipid peroxidation,
(4) up-regulation of prothrombotic factors (XII and
V), and
(5) down-regulation of antithrombotic factors or
endothelial-derived nitric oxide.
|
| ☉Golden time for thrombolytic TX |
3 hr
(for AMI: 12 hr)
|
| ☉Moyamoya dz: |
→intimal thickening in the walls of the terminal portions of the internal
carotid vessels bilaterally. The proliferating intima may contain lipid
deposits. The anterior, middle, and posterior cerebral arteries that
emanate from the circle of Willis may show varying degrees of stenosis or
occlusion.
→associated with fibrocellular thickening of the intima, waving of the
internal elastic lamina, and thinning of the media. Numerous small
vascular channels can be seen around the circle of Willis. These are
perforators and anastomotic branches. The pia mater also may have
reticular conglomerates of small vessels.
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●Neurosurgery
| ☉Brain herniation |
a) Subfalcial (cingulate) herniation ;
b) uncal herniation ;
c) downward
(central, transtentorial) herniation ;
d) external herniation ;
e) tonsillar herniation.
Types a, b, & e are usually caused by focal, ipsilateral space occupying
lesions, ie., tumor or axial or extra-axial hemorrhage. |
| ☉Foster-Kennedy Syndrome: |
→papilloedema in one eye and optic atrophy in the other.
→simultaneous raised intracranial pressure and optic nerve compression
secondary to tumour - classically, a meningioma of the olfactory groove,
or more commonly, due to a meningioma of the sphenoid wing. |
| ☉交感神經節燒灼術 |
T1: Horner's syndrome: unilateral anhidrosis, miosis, ptosis
T2: treat hyperhidrosis palmaris
T3: treat hyperhidrosis 腋下 |
| ☉dural hemorrhage |
SDH:Crescent
EDH:Spindle（每人公平分一個S）
|
| ☉Triad |
→IICP: headache, projectile vomiting, papilledema
→Cushing: Bradycardia, HTN, irregular breath
→Vasospasm TX: HTN, hypervolemia, hemodilution |
| ☉Spinal shock |
→characterized by flaccid paralysis (loss of bladder tone) below the level
of the injury, and loss of spinal cord
reflexes including bladder and bowel sphincter control.
→coincides with neurogenic shock, which is evidenced by loss of autonomic
control (bradycardia, vasodilation,
hypotension). |
| ☉CPP |
CPP=MAP-ICP
90 10-15 |
| ☉Brain tumor: |
most metastatic! most lung ca. breast ca.... |
| ☉Lagophthalmos |
→describes difficulty in complete closure of the eyelid over the eyeball.
→Causes of lagophthalmos:
exophthalmos
an impairment of mechanical closure of the lids e.g. burns of the eyelids
paralysis of orbicularis oculi(CN VII)
leprosy
→may result in corneal ulceration and keratitis |
| ☉AVM |
→Hemorrhage is more likely to be caused by small lesions, while seizures
are more likely to be caused by large lesions.
→The annual risk of intracranial hemorrhages associated with AVMs is 2-3%.
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| ☉Sturge-Weber syndrome (SWS) |
→also called ★encephalotrigeminal angiomatosis
→a neurocutaneous disorder with angiomas involving the leptomeninges (leptomeningeal
angiomas [LAs]) and skin of the face, typically in the ophthalmic (V1) and
maxillary (V2) distributions of the trigeminal nerve.
→The cutaneous angioma is called a port-wine stain (PWS).
→seizures, which may be intractable; focal deficits, such as hemiparesis
and hemianopia, both of which may be transient, called "strokelike
episodes"
→A "vascular steal phenomenon" may develop around the angioma, resulting
in cortical ischemia.
→Ocular manifestations (ie, buphthalmos, glaucoma)
→skull x-ray may show the classical "tram-line," or "tram-track" or
"trolley-track," calcifications considered pathognomonic for SWS
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| ☉Neurofibromatosis |
→a neurocutaneous condition that can involve almost any organ system.
→neurofibromatosis 1 (NF-1), the most common subtype, peripheral NF, also
central
→neurofibromatosis 2 (NF-2), central NF.
→A third variant is known as segmental NF, limited to a single body
region.
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| ☉NF-1 |
→→Diagnostic criteria for NF-1 (2 or more of the features listed are
present.)
1. Six or more café au lait macules larger than 5 mm in greatest diameter
in prepubertal individuals and those larger than 15 mm in greatest
diameter in postpubertal individuals
2. wo or more neurofibromas of any type or 1 plexiform neurofibroma
3. Freckling in the axillary or inguinal regions
4. ★Optic glioma (X-ray shows: The tumor has enlargedoptic foramen)
5. Two or more Lisch nodules (iris hamartomas)
6. A distinctive osseous lesion, such as sphenoid dysplasia or thinning of
the long bone cortex, with or without pseudoarthrosis
7. A first-degree relative with NF-1 according to the above criteria
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| ☉NF-2 |
→→Diagnosis criteria for NF-2 (1 or 2 is present.)
1. Bilateral masses of the eighth cranial nerve seen with appropriate
imaging techniques (eg, CT, MRI)
2. A first-degree relative with NF-2 and either (a) a unilateral mass of
the eighth cranial nerve or (b) 2 of the following: neurofibroma,
meningioma, glioma, schwannoma, or juvenile posterior subcapsular opacity
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| ☉Tuberous sclerosis complex (TSC) |
→abnormalities of the skin (hypomelanotic macules, facial angiofibromas,
shagreen patches, fibrous facial plaques, ungual fibromas), brain
(cortical tubers, subependymal nodules, seizures, mental
retardation/developmental delay), kidney (angiomyolipomas, cysts), and
heart (rhabdomyomas, arrhythmias).
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●Pediatric Neurology
| ☉Spinal cord |
birth: L3
3 y/o: L1-2 |
| ☉neurologic syndrome of vitamin E deficiency. |
spinocerebellar ataxia
1.loss of deep tendon reflexes,
2.truncal and limb ataxia,
3.loss of vibration and position sense,
4.ophthalmoplegia, muscle weakness, ptosis, and dysarthria.
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| ☉Craniosynostosis |
→Scaphocephaly(Sagital) > Brachycephaly(Coronal) > Oxycephaly(all)
→Cruzon's dz: craniofacial dysotosis, hypertelorism, proptosis, choanal
atresia
→Apert syndrome: Acrocephalosyndactyly
|
| ☉Anencephaly |
absence of the fetal cranial vault. Exposure of cerebral tissue to
amniotic fluid precludes brain development. a form of neural tube defect
that is typically an isolated birth defect that is not related to
chromosomal abnormalities. |
| ☉Spina bifida |
1. Spina Bifida Occulta:
2. Meningocele:
3. Myelomeningocele:
→There are two indirect signs that may lead to the suspicion of spina
bifida during an ultrasound examination, generally before the actual
spinal lesion can be observed.
→The fist is called the ★"lemon sign", consisting of a more or less
pronounced depression at the level of the metopic suture, giving to the
calvarium the shape of a lemon.
→The second sign is known as the★ banana sign and is observed at the level
of the cerebellum and cistern magna. It is caused by herniation of the
cerebellar vermis through the foramen magnum, giving the cerebellum the
aspect of a banana.
→Both signs are the consequence of the Arnold-Chiari Malformation type II,
commonly associated with spina bifida |
| ☉Meningomyelocele |
→Chiari II malformation which include: small posterior fossa, hindbrain
herniation into the upper cervical spinal canal, dysgenesis or agenesis of
the corpus callosum, neuronal migration disorders of varying degree, and
hydrocephalus
→Leitsymptome
1.Querschnittsyndrom: Schlaffe motorische und sensible Lähmung in
Abhängigkeit von der Läsionshöhe, neurogene Blasen- und
Darmentleerungsstörung
2.Hydrozephalus: Makrozephalie, Erbrechen, Kopfschmerz,
Sonnenuntergangsphänomen
3.Chiari-Syndrom: Hirnstammfunktionsstörungen (schlafbezogene
Atemstörungen, Schluckstö-rungen, Stridor), nuchale Schmerzen,
Hals-Schulter-Arm-Paresen
4.Wirbelsäule und untere Extremitäten: Skoliose, Kyphose und
Fehlstellungen der Hüft-, Knie- und Sprunggelenke
5.Assoziierte spinale Fehlbildungen: (Lipom, Diastematomyelie, Syringo-/Hydromyelie-Komplex,
Tethered cord): progrediente motorische und sensible Funktionsverluste,
Spastik, Kontrakturbildung, Schmerzen |
| ☉Dandy-Walker malformation |
→congenital malformation and involves the cerebellum and fourth ventricle.
→characterized by agenesis or hypoplasia of the cerebellar vermis, cystic
dilatation of the ★fourth ventricle, and enlargement of the posterior
fossa.
→Approximately 70-90% of patients have hydrocephalus, which often develops
postnatally. Dandy-Walker malformation may be associated with atresia of
the foramen of Magendie and, possibly, the foramen of Luschka. |
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