神經內科國考複習 (I) R4 唐奇峯

Outline

1. Seizure
2. Multiple Sclerosis
3. CNS Infection
4. Headache

5. Dementia
6. Movement Disorders
7. Peripheral Nervous System
8. Stroke
1.Seizure
Seizure

A seizure is a transient epileptic event, a symptom of

disturbed brain function.

Epilepsy is a chronic disorder, or group of chronic disorders,

in which the indispensable feature is recurrence of seizures
that are typically unprovoked and usually unpredictable.
Etiology of epilepsy
in all cases of newly
diagnosed seizures
Types of Seizure

Partial seizures begin focally in the cortex.

Such seizures may secondarily become
generalized, involving the whole of the cortex
Generalized seizures involve much of the cortex
bilaterally from the outset, and usually cause
immediate loss of consciousness.
Seizure
Partial Seizure
Simple partial seizures result when the ictal discharge occurs in a
limited area of cortex, the epileptogenic focus.

Complex partial seizures, are defined by impaired consciousness

and imply bilateral spread of the seizure discharge.

Patients with complex partial seizures usually exhibit

automatisms, such as lip smacking, repeated swallowing, or
complex motor activity that is undirected and inappropriate.

Postictally, patients are confused and disoriented for several

minutes.

Complex partial seizures, 70~80% arise from the temporal lobe.

Generalized Seizure
Generalized tonic-clonic (grand mal) seizures are
characterized by abrupt loss of consciousness with bilateral
tonic extension of the trunk and limbs (tonic phase)

Often accompanied by a loud vocalization as air is forcedly

expelled across contracted vocal cords (epileptic cry)

Followed by synchronous muscle jerking (clonic phase).

Postictally, patients are briefly unarousable and then lethargic

and confused, often preferring to sleep.
Generalized Seizure
Absence (petit mal) seizures are accompanied by motionless
staring and arrest of any ongoing activity.

Absence seizures begin and end abruptly; they occur without

warning or postictal period.
 Spike-and-Wave Complexes
型態 腦波特徵 相關癲 其他特徵
癇
3 Hz 1秒3個棘慢複合波， Childhood 兒童多，發育及
額葉區震幅最大 Absence 智力正常，上課
spike-and-wave 時常發呆
complexes Epilepsy
Slow 1秒1.5~2.5個，震 Lennox- 智障，合併多重
幅最大常見額葉區， Gastaut 癲癇型態
spike-and-wave 但亦可於其他多重
complexes 部位 syndrome

Fast 1秒4~6個，額葉區 Juvenile 青少年多，發育

震幅最大 Myoclonic 智力正常，早晨
spike-and-wave 睡醒偶有手腳抖
complexes Epilepsy 動，偶在夜間睡
眠時有大發作
Epilepsy Syndromes
Childhood Absence (Petit Mal) Epilepsy

Between the ages of 4 and 12 years

If untreated, can occur literally hundreds of times each day.

EEG : 3-Hz spike-wave discharges.

GTC also occur in 30% to 50% of cases.

Most children are normal, neurologically and intellectually.

Ethosuximide and valproate are equally effective in treating

absence seizures.
Epilepsy Syndromes
Lennox-Gastaut Syndrome

Heterogeneous group of childhood epileptic encephalopathies

that are characterized by mental retardation, uncontrolled
seizures, and a distinctive EEG pattern.

Seizures usually begin before age 4 years, and about 25% of

children have a history of infantile spasms.

No treatment is consistently effective, and 80% of children

continue to have seizures as adults.
Epilepsy Syndromes
Juvenile Myoclonic Epilepsy (JME)

Between the ages of 8 and 20 years.

The fully developed syndrome comprises morning

myoclonic jerks, generalized tonic-clonic seizures that occur
just after waking, normal intelligence, a family history of
similar seizures, and an EEG that shows generalized spikes,
4- to 6-Hz spike waves.

Valproate is the treatment of choice.

Lamotrigine, zonisamide, levetiracetam, and topiramate can
be equally effective in many patients.
AED
(tegretol)
Status Epilepticus: seizures last longer than 10 minutes or if two or
more seizures occur in close succession without recovery of
consciousness.
2.Multiple Sclerosis
 Affect central nervous system myelin
 The most common autoimmune (chronic)
inflammatory demyelinating disease of the central
nervous system (CNS)
 Recurrent
 Young onset: 20~40 y/o (rarely <10 and > 60 y/o)
 woman > man (2:1) (中年女性)

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Pathogenesis
 Inflammation, demyelination, and axon degeneration
 cause of MS remains unknown
 environment factor & susceptibility genes(HLA-DRB1*1501) 
aberrant immune response
 The most widely accepted theory
 begins as an inflammatory autoimmune disorder mediated
by autoreactive T lymphocytes
 EBV, HHV6

 Later, the disease is dominated by microglial activation and

chronic neurodegeneration

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Clinical manifestation
 Clinical isolated syndrome
 Optic neuritis
 EOM problemdiplopia
 usually due to CN6 or INO
 Internuclear ophthalmoplegia
 bilateral INO is most suggestive of MS

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 Sensory symptoms
 are a common initial feature of MS
 numbness, tingling, pins-and-needles, tightness,
coldness, or swelling of the limbs or trunk
 Trigeminal neuralgia in a young adult may be an early
sign of MS: 2%

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 Motor symptoms
 Spasticity: 70%  impairs mobility, disrupts sleep
 Movement disorder
 Tremor > tonic spasm
 Coordination
 Bowel/bladder/sexual function
 most common urinary complaint is urgency

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 Heat sensitivity
 Uhthoff phenomenon
 small increases in the body temperature can temporarily
worsen current or preexisting signs and symptoms

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 Brain MRI
 Dawson fingers
 Others: T1,T2, enhancement

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 Spinal cord lesion:
 more common in cervical
 white matter in lateral & posterior column
 typically < 2 vertebral segment, < ½ half cross-sectional
area
 CSF:
 Exclude other diagnosis
 normal protein and WBC
 35% not(lymphocytic pleocytosis)
 Oligoclonal band (OCBs): 95%
 IgG index > 0.7: 70~85%
 VEP
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 For neurological progression suggestive of primary
progressive MS
 the criteria require evidence of the one year of disease
progression plus two of the three following criteria
 Dissemination in space in the brain
 periventricular, juxtacortical, infratentorial, or spinal cord)

 Dissemination in space in the spinal cord

 based upon two or more T2 lesions in the cord

 Positive CSF findings with isoelectric focusing evidence of

oligoclonal bands and/or elevated IgG index

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Treatment
 Disease modifying drugs
 Inteferon beta: first line for RRMS
 side effect: flulike symptoms, injection site reactions, lab
abnormal(thrombocytopenia, anemia, leukopenia, increase liver
enzymes, thyroid dysfunction), worsen depression
 Glatiramer acetate: first line for RRMS
 side effect: injection site reaction, lipoatrophy, systemic reaction
 Tysabri (natalizumab): 2nd line for RRMS
 more effective than 1st line drugs, but with low risk of
PML(progressive multfocal leukoencephalopathy)
 anti-natalizumab Ab  loss of efficasy
 Nitoxantrone : for SPMS
 side effect: cardiotoxicity, leukemia, bone marrow suppression,
alopecia
 No proven therapy for PPMS 32
 Acute stage
 High dose IV corticosteroid +- oral prednisolone (not affect long-
term outcome)
 not affect degree of recovery

 Plasma exchange
 Symptomatic treatment
 spaticity: baclofen(SE: limb weakness, sedation, confusion, seizure
or hallucination when abrupt discontinuation), tizanidine,
gabapentin, botulinum toxin
 depression: lithium, depakin…
 fatigue: other medical condition?(thyroid disease, anemia…),
amantadine, SSRI, methyprednisolone
 overactive bladder: anticholinergic agents
 MS tremor: rivotril, tegretol, keppra, gabapentin…
 Paroxysmal symptoms: low dose AED
 pain
 constipation
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3.CNS Infection
 CNS Infection
CSF Study WBC Glucose Protein Pressure
(cell/mm )3 (mg/dl) (mg/dl) (cmH O)2

Normal <5 45 15~50 7~20

Bacteria ↑↑↑ < 45 ↑ ↑

. (PMN)

Virus ↑ = ↑ ↑
(lymphocyte)

TB ↑ <45 可能↑↑ 可能↑↑

(lymphocyte)

Fungus ↑ <45 可能↑↑ 可能↑↑

(lymphocyte)
 CNS Infection
Bacterial Meningitis

◎Classically, acute community acquired bacterial meningitis had been

most commonly caused by Streptococcus pneumoniae, Neisseria
meningitidis, Haemophilus influenzae type b, and Listeria
monocytogenes.

◎Most common complaint: headache(87%), neck stiffness(83%),

fever(77%), and an altered mental status(69%). 95% of patients have two
of the four symptoms.

◎Empiric antibiotics for bacterial meningitis: ceftriaxone +- vancomycin

Age >50 and/or risk factors for Listeria: add ampicillin

 CNS Infection

Bacterial Meningitis

◎Dexamethasone, started just before or during initial antibiotics and

given for 4 days, reduces the risk of a poor neurologic outcome in
patients with meningitis caused by streptococcus pneumoniae.
 CNS Infection
Viral encephalitis and Meningitis

◎Herpes Simplex encephalitis (HSE): HSV-1 encephalitis is the most

common identified cause of sporadic fatal encephalitis.

◎Symptoms and signs: fever(90%); headache(80%);

disorientation(70%); personality change(70-85%); focal or generalized
seizures(40-67%); memory disturbance(25-45%); motor deficit(30-40%);
and aphasia(33%)

◎MRI: involving the temporal lobes and inferior frontal lobe.

◎EEG may demonstrate periodic lateralizing epileptiform discharges.

◎Empirical therapy with acyclovir should be started immediately.

Standard adult dose is 10 mg/kg, given IV every 8 hours for 14~21 days.
CNS Infection
HSE is often homorrhagic.
CNS Infection
Fungal Infection
• The clinical presentation is similar to bacterial meningitis except
that the time course is usually subacute to chronic, and fever tends
to be less common.
• CSF glucose concentration in fungal meningitis is moderately
decreased, while in tuberculous meningitis, the CSF glucose
concentration is only mildly decreased.
• Imaging studies often show basilar meningitis with contrast
enhancement or unexplained hydrocephalus.
• For cryptococcus spp., a serum and/or CSF cryptococcal antigen
test is recommended.
4.Headache
 Migraine
1. At least 5 attacks fulfilling criteria 2-4

2. Headache attacks lasting 4-72 hours

3. Headache has at least two of the following characteristics:

a. unilateral location b. pulsating quality c. moderate or severe pain

intensity d. aggravation by or causing avoidance of routine physical
activity (eg, walking or climbing stairs)

4. During headache at least one of the following:

a. nausea and/or vomiting b. photophobia and phonophobia

5. Not attributed to another disorder

 Tension Type Headache
1. Tension-type headache(TTH) is almost always very mild and
therefore self-treated..

2. Muscle contraction is NOT the cause of the pain.

4. The IHS criteria: the headache lasts from 30 minutes to a week

and has two or more of the followings:

(1) Bilateral (2) Pressure and nonpulsatile (3) Mild-to-moderate

severity (4) Not aggravated by routine physical activity

5. For frequent or chronic TTH (>15 headaches days monthly),

preventive therapies are employed. Mirtazapine was effective in
the treatment of CTTH.
Cluster Headache
1. Men: Women= 8:1; Usually start between age 20 and 50.

2. One to three short-lived attacks of periorbital pain each day

for 4 to 8 weeks, followed by a pain-free interval for a mean of
1 year.

3. Periorbital pain begins without warning and reaches a

crescendo within 5 minutes. Pain is strictly unilateral and
attacks last from 30 minutes to 2 hours

4. Associated with homolateral lacrimation. Reddening of the

eye, nasal stuffiness and lid ptosis.

5. Treatment: oxygen inhalation. Prophylactic drugs are

prednisone, lithium, topiramate, divalproex, and verapamil.
Idiopathic Intracranial Hypertension
1. Pseudotumor cerebri.
2. Risk factors: obesity, hypervitaminosis A, streroid withdrawal
and female gender. (Drugs: tetracycline, amiodarone and lithium
carbonate, insecticide chlordecone)
3. Typically the first symptoms are headache and impaired vision.
The headache may be worse on awakening and aggravated by
coughing.

4. CSF pressure is usually 250~600 mm H2O but the fluid is

otherwise normal.

5. The patient with headache and papilledema without other

neurologic signs suggest the diagnosis of IIH.
 Giant Cell Arteritis
1. Granulomatous arteritis affecting large-size and medium size
arteries. The pain is usually unilateral, and often localized to the site
of the affected arteries in the scalp.

2. Patients generally present after age 70. Women:65%

3. Jaw claudication and ischemic nodules on the scalp, with

ulceration of the overlying skin.

4. The threat of blindness from thrombosis of the opththalmic or

posteriror ciliary arteries.

5. The earliest suspicion of cranial arteritis should lead to the

immediate administration of corticosteroids and then to biopsy of
the appropriate scalp artery. Usually ESR is greatly elevated.