Материал: Kaplan USMLE-1 (2013) - Anatomy

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Section IV • Neuroscience

Table IV-1-1

Disease

Symptoms

Multiple sclerosis (MS)

Symptoms separated in space and time

 

Vision loss (optic neuritis)

 

Internuclear ophthalmoplegia (MLF)

 

Motor and sensory deficits

 

Vertigo

 

Neuropsychiatric

Metachromatic

Four types; motor and cognitive issues

leukodystrophy (MLD)

with seizures

Progressive multifocal

Cortical myelin commonly affected;

leukoencephalopathy

causes limb weakness, speech

(PML)

problems

Central pontine

Pseudobulbar palsy

myelinolysis (CPM)

Spastic quadriparesis

 

 

Mental changes

 

May produce the "locked-in" syndrome

 

Often fatal

Guillain-Barre syndrome

Acute symmetric ascending

 

inflammatory neuropathy of PNS myelin

 

Weakness begins in lower limbs and

 

ascends; respiratory failure can occur in

 

severe cases

 

Autonomic dysfunction may be

 

prominent

 

Cranial nerve involvement is common

 

Sensory loss, pain, and paresthesias

 

rarely occur

 

Reflexes invariably decreased or absent

Abbreviations: CJD, Jacob-Creutzfeldt; MLF, medial longitudinal fasciculus

Notes

Occurs twice as often in women

Onset often in third or fourth decade

Higher prevalence in temperate zones

Relapsing-remitting course is most common

Well-circumscribed demyelinated plaques often in periventricular areas

Chronic inflammation; axons initially preserved

Increased lgG (oligoclonal bands) in CSF

Treatment: high-dose steroids, interferon-beta, glatiramer (Copaxone®)

Arylsulfatase A deficiency in lysosomes affects botn CNS and PNS myelin

Caused by CJD virus

Affects immunocompromised, especially AIDS

Demyelination, astrogliosis, lymphohistiocytosis

Focal demyelination of central area of basis pontis (affects corticospina corticobulbartracts)

Seen in severely malnourished, alcoholics, liver disease

Probably caused by overly aggressive correction of hyponatremia

Two-thirds of patients have history of respiratory or GI illness 1-3 weeks prior to onset

Elevated CSF protein with normal cell count (alburninocytologic dissociation)

334 MEDICAL

Development ofthe Nervous System

Neurulation

•Neurulation begins in the third week; both CNS and PNS derived from neuroectoderm.

•The notochord induces the overlying ectoderm to form the neural plate (neuroectoderm).

•By end of the third week, neural folds grow over midline and fuse to form neural tube.

•During closure, neural crest cells also form from neuroectoderm.

•Neural tube 3 primary vesicles --7 5 primary vesicles --7 brain and spinal cord

•Brain stem and spinal cord have an alar plate (sensory) and a basal plate (motor); plates are separated by the sulcus limitans.

•Neural crest --7 sensory and postganglionic autonomic neurons, and other non-neuronal cell types.

•Peripheral NS (PNS): cranial nerves ( 12 pairs) and spinal nerves (31 pairs)

Chapter 1 • Nervous System

Note

Alpha-fetoprotein (AFP) levels may also be elevated in gastroschisis and omphalocele. AFP levels are low in pregnancy of down syndrom fetus.

Neural plate

Neuroectoderm

Neural

 

 

groove

 

Ectoderm

 

Notochordal

process Neural groove

Day 18

Neural fold

Neural tube

Rostral neuropore (closes at day 25)

Failure to close results in anencephaly,

causing polyhydramios

and increased alpha­ Basal plate (motor) fetoprotein and AChE

Neural crest

Caudal neuropore (closes at 270)

Failure to close results in spina bifida

Day 22 and increased alpha­ fetoprotein and AChE

Figure IV-1-1 . Development of Nervous System

MEDICAL 335

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