339 Functional Neuroanatomy

Text and Atlas

Paired openings in the lateral recesses of the arachnoid roof of the fourth ventricle through
which CSF from the fourth ventricle reaches the cisterna magna. Named after Hubert von
Luschka, the German anatomist, in 1863.
Foramen of Magendie.
Median aperture in the roof of the fourth ventricle connecting it with the cisterna magna. Named
after Franéois Magendie, the French physiologist, who described the foramen in 1842.
Foramen of Monro.
Site of communication between the lateral and third ventricles. First described by Alexander
Monro, the Scottish anatomist, in 1753. Before that time, it was assumed that the lateral and third
ventricles communicated by a hole or passage at the upper end of the third ventricle called the
vulva or by a place under the fornix called the anus. There had been no demonstration of these
apertures. They were presumed to occur by necessity.
Lumbar puncture.
A method of accessing CSF in the lumbar subarachnoid space by introducing a needle between
the lumbar vertebrae. The procedure was introduced in 1891 by Heinrich Quinke, a German
physician who obtained CSF for the first time from a living patient. William Gowers disapproved
of the procedure and discouraged its use at the National Hospital in London until after his
retirement.
SUGGESTED READINGS
Alami SY, Afifi AK: Cerebrospinal fluid examination. In Race GJ (ed): Laboratory Medicine, vol 4, chap 2. Hagerstown, MD,
Harper & Row, 1973:1.
Bradbury M: The structure and function of the blood-brain barrier. Fed Proc 1984; 43:186–190.
Egnor M et al: A model of intracranial pulsations. Pediatr Neurosurgery 2001; 35:284–298.
Fox RJ et al: Anatomic details of intradural channels in the parasagittal dura: a possible pathway for flow of cerebrospinal fluid.
Neurosurgery 1996; 39:84–90.
Friede RL: Hydrocephalus-special pathology. In: Developmental Neuropathology. New York, Springer-Verlag, 1989:240–241.
Goldstein G, Betz A: The blood-brain barrier. Sci Am 1986; 255:74–83.
Gomez DG et al: The spinal cerebrospinal fluid absorptive pathways. Neuroradiology 1974; 8:61–66.
Hughes RA et al: Caves and cysts of the septum pellucidum. Arch Neurol Psychiatry 1955; 74:259–266.
Johnston I, Teo C: Disorders of CSF hydrodynamics. Child Nerv Syst 2000; 16:776–799.
Kempe LG, Busch E: Clinical significance of cisterna veli interpositi. Acta Neurochir 1967; 16:241–248.
Leech RW: Normal anatomy of ventricles, meninges, subarachnoid space, and venous system. In Leech RW, Brumback RA
(eds): Hydrocephalus: Current Clinical Concepts. St Louis, Mosby–Year Book, 1991:18.
Leech RW: Normal physiology of cerebrospinal fluid. In Leech RW, Brumback RA (eds): Hydrocephalus: Current Clinical Concepts.
St Louis, Mosby–Year Book, 1991:30.
Leslie W: Cyst of the cavum vergae. Can Med Assoc J 1940; 43:433–435.
Mori K: Subcallosal midline cysts in anomalies of the central nervous system. In Nadjmi M (ed): Neuro-radiologic Atlases. New
York, Thieme-Stratton, 1985:69.
Pryse-Phillips W: Companion to Clinical Neurology. Boston: Little, Brown, 1995.
Rubin LL, Staddon JM: The cell biology of the blood-brain barrier. Annu Rev Neurosci 1999; 22:11–28.
Sage MR, Wilson AJ: The blood-brain barrier: An important concept in neuroimaging. AJNR 1994; 15:601–622.
Saunders NR et al: Barrier mechanisms in the brain: I. Adult brain. Clin Exp Pharmacol Physiol 1999; 26:11–19
Schiffer E et al: Influence of sex on cerebrospinal fluid density in adults. Br J Anaesth 1999; 83:943–944.
Schwidde JT: Incidence of cavum septi pellucidi and cavum vergae in 1032 human brains. Arch Neurol Psychiatry 1952; 67:625–
632.
Segal MB: The choroid plexuses and the barriers between the blood and the cerebrospinal fluid. Cell Molec Neurobiol 2000;
20:183–196.
Selmaj K: Pathophysiology of the blood-brain barrier. Semin Immunopathol 1996; 18:57–73.
Tyler HR, Tyler KL: Communication between lateral and third ventricle: First description. Neurology 1985; 35:1298.
Vastola EF: CSF formation and absorption estimates by constant flow infusion method. Arch Neurol 1980; 37:150–154.
Whitelaw A: A new view on the CSF-circulation with the potential for pharmacological treatment of childhood hydrocephalus.
Acta Paediatr 1997; 86:125–132.
Zellweger H, Van Epps EF: The cavus veli interpositi and its differentiation from cavum vergae. AJR 1959; 82:793–805.
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3"     
   
     
      
Cerebrospinal Fluid in Disease
Ventriculomegaly
Hydrocephalus
Normal-Pressure Hydrocephalus (Hakim-Adams Syndrome)
Benign External Hydrocephalus
Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) and Benign Intracranial
Hypertension
Intraventricular Neuroepithelial Cysts
The Bobble-Head Doll Syndrome
Dandy-Walker Syndrome (Malformation)
KEY CONCEPTS
1) Ventriculomegaly is associated with overproduction of CSF, brain atrophy, developmental
failure of growth of the cerebral mantle, and obstruction of CSF flow or absorption.
2) Hydrocephalus refers to an increased amount of CSF in the ventricle, with or without a
concomitant increase in CSF pressure. Hydrocephalus is classified into communicating and
noncommunicating varieties.
 340

3) Normal-pressure hydrocephalus refers to uniform enlargement of the ventricular system

without a concomitant increase in CSF or intracranial pressure.
4) Benign external hydrocephalus refers to the accumulation of CSF in the subarachnoid
spaces around the brain without significant enlargement of the ventricular cavities.
5) Idiopathic intracranial hypertension is characterized by increased intracranial pressure
without hydrocephalus or a brain tumor, small ventricular cavities, and a favorable
response to acetazolamide or corticosteroids.
6) Intraventricular cysts may occur in any of the ventricular cavities but are most common in
the third ventricle (colloid cysts of the third ventricle).
7) Bobble-head doll syndrome is associated with third ventricular cysts and less commonly is
associated with aqueductal stenosis and shunt obstruction.
8) Dandy-Walker syndrome is characterized by the triad of large cystic dilation of the fourth
ventricle, agenesis of the cerebellar vermis, and enlargement of the posterior fossa.
CEREBROSPINAL FLUID IN DISEASE

C
SF examination in patients with neurologic disorders can provide valuable information
about the nature of the disease process. This is particularly true in infections (meningitis,
encephalitis), autoimmune disorders (multiple sclerosis, Guillain-Barré polyneuritis),
tumors, and hemorrhage. (CSF: Cerebro Spinal Fluid; Líquido Cefaloraquídeo (LCR) in
spanish).)
Normal CSF obtained from the lumbar subarachnoid space is clear and colorless, is under 50 to
200 mm of CSF pressure in the recumbent relaxed state, and contains three cells (lymphocytes)
or fewer per cubic millimeter, 15 to 45 mg of protein, and 60 to 80 mg/dl of glucose.
In bacterial meningitis the CSF is cloudy and turbid, is under considerably increased pressure
(200 to 500 mm of CSF pressure), and contains an increased number of cells, almost all
polymorphonuclear leukocytes (2000 to 10,000/mm3), increased protein (100 to 1000 mg), and
low glucose (below 20 mg/dl). Examination of fluid by Gram stain and culture reveals the
organism responsible for the meningitis.
In viral encephalitis the CSF usually is clear, is under normal or slightly elevated pressure, and
contains either a normal or a slightly increased number of cells (five to several hundred, mostly
lymphocytes), normal or slightly increased protein (50 to 200 mg/dl), and normal glucose.
Gram staining shows no bacteria. Culture of the CSF may reveal the viral agent involved.
In multiple sclerosis the CSF is clear, is under normal pressure, and contains a normal or an
increased number (50 to 300) of cells, predominantly lymphocytes, normal or moderately
increased protein including oligoclonal and myelin basic proteins, increased gamma globulins,
and normal glucose.
In Guillain-Barré the CSF is characterized by albuminocytologic dissociation in which protein is
moderately to markedly elevated in the presence of normal cells. The fluid is clear, is under
normal pressure, and contains a normal amount of glucose.
In brain tumors the CSF is clear, is under increased pressure, and contains a normal or
increased number of cells, an increased amount of protein, and normal glucose. Spinning of the
CSF may reveal the presence of tumor cells in the sediment. Seeding of tumor cells along the
meninges is associated with an increase in cells and protein. Lumbar puncture is
contraindicated in the presence of increased intracranial pressure to avoid herniation.
In spinal cord tumors the CSF may have a yellowish tinge as a result of the marked increase in
protein, is under normal pressure, and contains a normal or slightly increased number of cells,
a marked increase in protein, and normal glucose. Tumor cells may be found in the sediment.
In subarachnoid hemorrhage the CSF is bloody, is under markedly increased pressure, and
contains a large number of red blood cells, a very high amount of protein (as a result of the
presence of blood), and low glucose.
Table 30-1 summarizes CSF findings in health and disease.
VENTRICULOMEGALY
Enlargement of the ventricles (ventriculomegaly) usually is associated with one of the following
conditions: (1) overproduction of CSF, as occurs in tumors of the choroid plexus (choroid plexus
papilloma), (2) atrophy of the brain with secondary (compensatory) enlargement of the
ventricles (hydrocephalus ex vacuo), as in Alzheimer's disease; (3) developmental failure of
growth of the cerebral mantle (the brain between the ventricle and the brain surface), as in the
condition known as colpocephaly; or (4) obstruction of CSF flow or absorption, as in obstructive
hydrocephalus.
The mechanism of ventriculomegaly in hypersecreting tumors of the choroid plexus (Figure 30-
1) is not clear. It may be due to overproduction of CSF in excess of resorption, overproduction
of protein, or both.
Ventriculomegaly associated with brain atrophy may be focal (as in infarction) (Figure 30-2) or
generalized (as in Alzheimer's disease and hypoxic ischemic encephalopathy) and is a
compensatory mechanism that fills the space created by the loss of brain substance. Hence, it is
called hydrocephalus ex vacuo. It usually is associated with concomitant enlargement of the
subarachnoid spaces.
Developmental ventriculomegaly is due to failure of growth of the cerebral mantle. In an 8-
week-old embryo, the ventricles are large and the cerebral mantle is thin. With normal
development, the cerebral mantle grows faster than do the ventricles, so that by mid gestation
the ventricles become relatively small. If the cerebral mantle fails to grow normally, the
ventricles remain relatively large, a condition known as colpocephaly (Figure 30-3), a term
coined by Yakovlev and Wadsworth in 1946 to refer to disproportionate enlargement of the
occipital horns.
 341 Functional Neuroanatomy
Text and Atlas
HYDROCEPHALUS
Hydrocephalus is a condition characterized by an increased amount of CSF in the ventricles
(Figure 30-4). Hippocrates was one of the first physicians to deal with hydrocephalus,
advocating the use of laxatives and sneeze-inducing substances for its treatment. The surgical
approach to the treatment of hydrocephalus, though suggested by Hippocrates and others, was
not accepted as the most effective mode of treatment until the nineteenth century.
There are two types of hydrocephalus: communicating and noncommunicating. In
communicating hydrocephalus there is free communication between the ventricles and the
subarachnoid space. The obstruction to the flow of CSF in this type of hydrocephalus is usually
distal to the ventricular system, in the subarachnoid spaces (as a result of fibrosis from previous
infection) or the arachnoid granulations (as a result of a lack of or abnormalities in those
structures). This results in CSF accumulation and enlargement of all the ventricular cavities as
well as the subarachnoid spaces.
In noncommunicating hydrocephalus CSF in the ventricular cavities cannot reach the
subarachnoid spaces because of obstruction of CSF flow in the foramen of Monro (Figure 30-5),
the aqueduct of Sylvius (Figure 30-6), or the foramina of Magendie and Luschka. Obstruction of
the foramen of Monro—for example, by tumor—blocks the flow of CSF from the lateral ventricle
to the third ventricle, resulting in an accumulation of CSF and enlargement of the lateral
ventricle on the side of obstruction (Figure 30-5). Obstruction of the aqueduct of Sylvius by
tumor, inflammation, or congenital atresia results in accumulation of CSF and enlargement of the
ventricular cavities draining into the aqueduct (third ventricle and both lateral ventricles)
(Figure 30-6). Obstruction at the foramina of Magendie and Luschka by tumor, inflammation, or
congenital atresia results in CSF accumulation and enlargement of the fourth, third, and both
lateral ventricles.

Table 30-1. Cerebrospinal Fluid Findings in Health and Disease

Condi- color Pressu- Cells/mm3 Protein Glucose other

tion re (mg/dl) (mg/dl)
(mmCS
F)

Nor- Clear 50– 0–3 15–45 60– —

mal 200 80

Bacte- Clou ↑ ↑(neutroph ↑ ↓ Organis

rial dy ils) m by
me- Gram
ningi- stain
tis and
culture

Viral Clear Nor Normal or Nor- Nor Orga-

encep mal ↑(lymphoc mal or mal nism by
halitis or ↑ ytes) ↑ culture

Mul- Clear Nor Normal or Norma Nor Oligoclo

tiple mal ↑ l or ↑ mal nal
scle- (increa bands,
rosis sed and
gamma myelin
globuli basic
ns) proteins

Gui- Clear Nor Normal ↑ Nor Albu-

llain- mal mal minocy-
Barré tologic
syn- disasso-
dro- ciation
me
 342

Brain Clear ↑ Normal ↑ Nor Tumor

tumor or↑ mal cells in
sedi-
ment

Spinal Ye- Nor Normal or ↑ Nor Tumor

tumor llow mal ↑ mal cells in
sedi-
ment

Suba- Bloo ↑ ↑ (red ↑ ↓ —

rach- dy cells)
noid
hemo
rrhage

In adults in whom the skull sutures have closed, hydrocephalus is associated with a marked
increase in intracranial pressure. This is associated with headache, vomiting, dizziness, a
decrease in the state of consciousness, and edema of the optic disks. In these patients, the
lateral margins of the lateral ventricles become rounded and there is an outflow of CSF across
the ependyma into the periventricular spaces (transependymal flow) (Figure 30-7). Pressure
exerted on the corticospinal fibers that innervate the lower extremities, which travel in
proximity to the lateral ventricles, results in lower extremity weakness.
If hydrocephalus develops in early childhood, before closure of the skull sutures, the skull
yields to the increased pressure by widening of the sutures and a progressive increase in head
circumference. A rapid increase in intracranial pressure in these children may result in a
decreased level of consciousness and alertness, vomiting, irritability, and the “setting-sun”
sign, in which the upper lids are retracted and the globes are directed downward.
Normal-Pressure Hydrocephalus (Hakim-Adams Syndrome)
Normal-pressure hydrocephalus (a type of communicating hydrocephalus) is a disorder of the
elderly characterized by uniform enlargement of the ventricular system without a concomitant
increase in CSF pressure or intracranial pressure. The pathophysiology of normal-pressure
hydrocephalus is poorly understood. Impaired resorption of CSF is believed to be the cause of
CSF accumulation and ventricular enlargement. Clinically, the condition is characterized by
dementia, urinary incontinence, and gait disturbance. These signs sometimes improve after
shunting of the CSF to extracranial sites. Normal-pressure hydrocephalus is thus considered a
treatable dementing disorder. The condition can be diagnosed by radioisotope scans, which
demonstrate reflux of the radioisotope into the ventricles after its injection into the lumbar
subarachnoid space.

Figure 30-1 Figure 30-2

Figure 30-1. Parasagittal gadolinium enhanced magnetic resonance image (MRI) showing choroid plexus
papilloma and ventriculomegaly.
Figure 30-2. Coronal section of the brain showing cerebral infarct and secondary focal ventriculomegaly.
 343 Functional Neuroanatomy
Text and Atlas

Figure 30-3 Figure 30-4.

Figure 30-3. T1-weighted parasagittal MRI showing disproportionate enlargement of the occipital horn in
colpocephaly.
Figure 30-4. T2-weighted axial MRI showing enlarged ventricular cavities (ventriculomegaly) due to
hydrocephalus.

Figure 30-5 Figure 30-6

Figure 30-5. T1-weighted axial MRI showing unilateral enlargement of the lateral ventricle with displacement of
the septum pellucidum across the midline due to obstruction of the foramen of Monro by atresia.
Figure 30-6. T1-weighted midsagittal MRI showing selective enlargement of the lateral and third ventricles due to
aqueductal stenosis. The fourth ventricle is normal in size.

Figure 30-7 Figure 30-829

Figure 30-7. T2-weighted axial MRI showing transependymal flow of cerebrospinal fluid to the adjacent brain
substance in hydrocephalus.
Figure 30-8. Computed tomography scan showing accumulation of cerebrospinal fluid in the subarachnoid space
over the frontal lobe and in the interhemispheric fissure as seen in benign external hydrocephalus. (Failure of
origin in the figure)
Benign External Hydrocephalus
Benign external hydrocephalus is a disorder of childhood characterized by the accumulation of
CSF in the subarachnoid space over the brain surface, particularly over the frontal lobes and in
the interhemispheric fissure, without significant involvement of the ventricular cavities (Figure
30-8). The condition was first described in the eighteenth century by Underwood, who also
observed its benign nature. The condition was rediscovered after the advent of newer imaging
techniques. It is a self-limited condition which usually resolves spontaneously without sequelae.

29 Image with failure of origin.

 344

IDIOPATHIC INTRACRANIAL HYPERTENSION (PSEUDOTUMOR CEREBRI) AND

BENIGN INTRACRANIAL HYPERTENSION
Idiopathic intracranial hypertension (IIH), which was described by Quincke in 1891, is a
disorder characterized by increased intracranial pressure without hydrocephalus or brain
tumor. It is more common in adult obese women of childbearing age and affects both sexes
equally in childhood. These patients complain of headache, papilledema, and transient visual
obscuration. Imaging studies usually show small ventricles. The condition responds to
acetazolamide (Diamox), a carbonic anhydrase inhibitor, and to corticosteroids, both of which
reduce or inhibit the formation of CSF. Studies of CSF hydrodynamics in pseudotumor cerebri
differentiate two types: type I with normal CSF conductance and type II with very low
conductance and high CSF pressure. Type I is believed to result from extracellular brain
edema, and type II from impaired CSF resorption through the arachnoid granulations. CSF
hydrodynamic studies suggest that patients with type II IIH share a common physiologic
mechanism with patients who have normal-pressure hydrocephalus.
INTRAVENTRICULAR NEUROEPITHELIAL CYSTS
Intraventricular cysts are rare developmental cysts lined by neuroepithelium. The precise
origin of these cysts is controversial. They are believed to arise from choroid plexus tissue
derived from primitive neuroepithelium. They have been reported to occur in all the ventricular
cavities, but most commonly in the third ventricle (colloid cysts of the third ventricle). A variety
of names have been used to describe these cysts, including epithelial cysts, ependymal cysts,
choroid plexus cysts, choroidal epithelial cysts, and subarachnoid ependymal cysts. The term
neuroepithelial cysts was introduced by Fulton and Bailey in 1929. Intraventricular cysts contain
a clear serous liquid resembling CSF with a mildly elevated protein content. The fluid in colloid
cysts of the third ventricle is usually viscid with a gelatinous or mucinous appearance.
Intraventricular cysts are clearly visible on magnetic resonance imaging (Figure 30-9). They
usually are asymptomatic and are found accidentally on neuroimaging studies. Some may
enlarge and become symptomatic.
THE BOBBLE-HEAD DOLL SYNDROME
The bobble-head doll syndrome is a disorder of childhood characterized by a to-and-fro, 2- to
3-Hz rhythmic nodding of the head similar to that in a doll with a weighted head attached to a
coil-spring neck. The movement disappears in the supine position and during sleep. This
disorder was described by Benton in 1966. In most cases the syndrome is associated with an
intraventricular cyst in the region of the anterior third ventricle (Figure 30-10) or an arachnoid
cyst in the suprasellar region. The phenomenon results from intermittent obstruction of the
foramen of Monro by the cyst. The head bobbing is believed to be a learned behavior which
relieves the obstruction by means of posterior displacement of the cyst away from the foramen
of Monro. The syndrome has less commonly been described in association with aqueductal
stenosis and shunt obstruction. The syndrome is treated by shunting or fenestration of the cyst.

Figure 30-9 Figure 30-10

Figure 30-9. T1-weighted parasagittal MRI showing a
Figure 30-10. T2-weighted axial MRI showing a third
neuroepithelial cyst in the posterior part of the lateral
ventricle cyst in the bobble-head doll syndrome.
ventricle.

Figure 30-11. T1-weighted midsagittal MRI showing

features of the Dandy-Walker syndrome.

DANDY-WALKER SYNDROME (MALFORMATION)

The Dandy-Walker malformation (Figure 30-11) consists of the triad of (1) large cystic dilatation
of the posterior part of the fourth ventricle, (2) complete or partial agenesis of the cerebellar
vermis, and (3) enlargement of the posterior fossa with upward displacement of the tentorium,
torcula, and transverse sinus. Hydrocephalus, though common, is not an essential feature of the
syndrome. The syndrome was described by Sutton in 1887 and was recognized as a distinct
entity in 1914 by Dandy and Blackfan, who attributed it to atresia of the foramina of Magendie
 345 Functional Neuroanatomy
Text and Atlas
and Luschka. In 1942 Taggart and Walker documented the entity and supported the proposed
etiology of atresia. The term Dandy-Walker syndrome was proposed in 1954 by Benda, who
recognized that atresia of the foramina of Magendie and Luschka is not an essential feature of
the syndrome. The pathogenesis of the syndrome remains controversial. The syndrome arises
early in gestation, at about 4 weeks, and involves multiple developmental defects of the central
nervous system. Foraminal atresia may be a contributing factor in some cases. The cystic
dilatation of the fourth ventricle is attributed to the persistence of the anterior membranous area
that forms the roof of the fetal fourth ventricle, which ordinarily regresses and disappears as the
choroid plexus and vermis develop.
Various treatment modalities have been tried with varying success, including
ventriculoperitoneal shunting, opening of the fourth ventricle, and excision of the cyst
membrane. Current treatment consists of shunting of the cyst to the peritoneum
(cystoperitoneal shunt) combined with shunting of the lateral ventricles to the peritoneum
(ventriculoperitoneal shunt).
TERMINOLOGY
Alzheimer's disease.
A type of cortical dementia named after Alois Alzheimer, the German neuropsychiatrist and
pathologist who described the disease in 1906. The term Alzheimer's disease was coined by
Ernst Kraepelin, a German psychiatrist, in 1910.
Aqueduct of Sylvius.
A narrow passage linking the third and fourth ventricles. Named after Franciscus de la Boe
Sylvius, who described it in 1650.
Bobble-head doll syndrome.
A syndrome of to-and-fro rhythmic movement of the head associated with anterior third
ventricle cysts or tumors. The movement is believed to be a learned behavior that relieves
obstruction of the foramen of Monro.
Colpocephaly.
A developmental condition characterized by failure of development of the cerebral mantle and
secondary ventriculomegaly with disproportionate enlargement of the occipital horns of the
lateral ventricle. The term was coined by Yakovlev and Wadsworth in 1946.
Communicating hydrocephalus.
A type of hydrocephalus in which obstruction to CSF flow occurs between the roof of the fourth
ventricle and the arachnoid granulations.
Dandy-Walker syndrome.
A developmental malformation characterized by large cystic dilatation of the fourth ventricle,
agenesis of the cerebellar vermis, and upward displacement of the tentorium cerebelli, torcula,
and transverse sinus. The condition was first described by J. B. Sutton in 1887 and was
recognized as a distinct entity by Dandy and Blackfan in 1914 and by Taggert and Walker in
1942. The term Dandy-Walker syndrome was proposed by Benda in 1954.
Foramen of Luschka.
Paired openings in the lateral recesses of the fourth ventricle through which cerebrospinal fluid
flows from the fourth ventricle to the cisterna magna. Named after Hubert von Luschka, a
German anatomist, in 1863.
Foramen of Magendie.
The median aperture in the roof of the fourth ventricle, connecting it with the cisterna magna.
Named after Fran¸ois Magendie, a French physiologist who described it in 1842.
Foramen of Monro.
The site of communication between the lateral and third ventricles. Named after Alexander
Monro, a Scottish anatomist who described it in 1753.
Guillain-Barré syndrome.
An acute inflammatory demyelinating polyneuropathy. Described by George Guillain, Jean
Alexander Barré, and Andre Strohl, French physicians, in 1916.
Hydrocephalus (Greek hydro, “water”; kephalé, “head”).
Dilatation of the cerebral ventricles. Known to Hippocrates, it was described accurately by
Vesalius in 1550.
Hydrocephalus ex vacuo.
An increase in the volume of CSF and ventriculomegaly secondary to brain atrophy.
Noncommunicating hydrocephalus.
A type of hydrocephalus caused by obstruction of cerebrospinal fluid flow between the sites of
its formation and the roof of the fourth ventricle.
Pseudotumor cerebri.
A condition consisting of a rise in intracranial pressure in the absence of an intracranial mass or
hydrocephalus. Known by other terms, including idiopathic intracranial hypertension, hydrops,
serous meningitis, Julien-Marie-See syndrome, Dupré's syndrome, and Symonds syndrome.
First described by Quincke in 1891.
Setting-sun sign.
Depression of the eyeball with failure of upward gaze and retraction of upper lid. Seen in
children with hydrocephalus and pressure on the dorsal tectum.
Visual obscuration.
Transient dimming of vision caused by increased intracranial pressure.
SUGGESTED READINGS
Benda CE: The Dandy-Walker syndrome or the so-called atresia of the foramen of Magendie. J Neuropathol Exp Neurol 1954;
13:14–29.
 346

Benson DF et al: Diagnosis of normal pressure hydrocephalus. N Engl J Med 1970; 283:609–615.
Benton JW et al: The bobble-head doll syndrome. Neurology 1966; 16:725–729.
Coker SB: Bobble-head doll syndrome due to trapped fourth ventricle and aqueduct. Pediatr Neurol 1986; 2:115–116.
Czervionke LF et al: Neuroepithelial cysts of the lateral ventricle: MR appearance. AJNR 1987; 8:609–613.
Dandy WE, Blackfan KD: Internal hydrocephalus: An experimental, clinical, and pathological study. Am J Dis Child 1914;
8:406–482.
Dell S: Further observation on the “bobble-head doll syndrome.” J Neurol Neurosurg Psychiatry 1981; 44:1046–1049.
Hart MN et al: The Dandy-Walker syndrome: A clinicopathological study based on 28 cases. Neurology 1972; 22:771–780.
Herskowitz J et al: Colpocephaly: Clinical, radiologic, and pathogenetic aspects. Neurology 1985; 35:1594–1598.
Leech RW, Goldstein E: Hydrocephalus: Classification and mechanisms. In Leech RW, Brumback RA (eds): Hydrocephalus:
Current Clinical Concepts. St. Louis, Mosby 1991:45–70.
New PFJ, Davis KR: Intraventricular noncolloid neuroepithelial cysts. AJNR 1981; 2:569–576.
Norman MG et al: Dandy Walker syndrome. In Norman MG et al. (eds): Congenital Malformations of the Brain: Pathological,
Embryological, Clinical, Radiological, and Genetic Aspects. New York, Oxford University Press, 1995:343–347.
Norman MG et al: Hydrocephalus. (eds): Congenital Malformations of the Brain: Pathological, Embryological, Clinical, Radiological, and
Genetic Aspects. New York, Oxford University Press, 1995:333–339.
Papazian O et al: The history of hydrocephalus. Int Pediatr 1991; 6:233–235.
Pryse-Phillips W: Companion to Clinical Neurology. Boston, Little, Brown, 1995.
Puden RH: The surgical treatment of hydrocephalus: An historical review. Surg Neurol 1981; 15:15–26.
Sahar A et al: Choroid plexus papilloma: Hydrocephalus and cerebrospinal fluid dynamics. Surg Neurol 1980; 13:476–478.
Sarnat HB: Dandy-Walker malformation. In Norman MG et al (eds): Cerebral Dysgenesis: Embryology and Clinical Expression. New
York, Oxford University Press, 1992:305–316.
Sutton JB: The lateral recesses of the fourth ventricle: Their relation to certain cysts and tumors of the cerebellum and to
occipital meningocele. Brain 1887; 9:352–361.
Taggart JK, Walker AE: Congenital atresia of the foramens of Luschka and Magendie. Arch Neurol Psychiatr 1942; 48:583–612.
Wiese JA et al: Bobble-head doll syndrome: Review of the pathophysiology and CSF dynamics. Pediatr Neurol 1985; 1:361–366.
Williams MA, Razumovsky AY: Cerebrospinal fluid circulation, cerebral edema, and intracranial pressure. Curr Opin Neurol
1996; 6:847–853.
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31   %     
  
 & 
Major Sensory Pathways
Pathway for Conscious Proprioception
Pathways for Nonconscious Proprioception
Pathway for Pain and Temperature
Trigeminal Pathways
Major Motor Pathways
Cortical Origin
Subcortical Origin
KEY CONCEPTS
1) Posterior column fibers ascend ipsilateral to their side of entry into the spinal cord and
synapse on the nuclei gracilis and cuneatus in the medulla oblongata. Second-order fibers
from the nuclei gracilis and cuneatus cross in the medulla oblongata (sensory, lemniscal
decussation) to form the medial lemniscus. Medial lemniscal fibers terminate on neurons in
the ventral posterior lateral nucleus of the thalamus.
2) The dorsal spinocerebellar tract reaches the cerebellum via the restiform body. The
ventral spinocerebellar tract reaches the cerebellum via the brachium conjunctivum.
3) The spinothalamic fibers are somatotopically organized so that sacral originating fibers are
lateral in the tract and cervical originating fibers are medial in the tract. Spinothalamic
fibers terminate on neurons in the ventral posterior lateral nucleus of the thalamus.
4) Trigeminal pathways convey exteroceptive and proprioceptive sensations from the face.
5) Corticospinal fibers originate principally from the motor and premotor areas, descend
throughout the neuraxis, mostly decussate in the medulla oblongata (motor decussation),
and terminate on interneurons or alpha motorneurons in the spinal cord.
6) Corticopontocerebellar fibers constitute the largest component of corticofugal fibers. They
originate principally from primary sensory and motor cortices and synapse on pontine
nuclei. Second-order neurons from the pontine nuclei terminate in the cerebellum.
7) Cortically originating (cortifugal) motor pathways include the corticospinal (pyramidal),
corticopontocerebellar, corticobulbar, corticothalamic, corticostriate, and
corticohypothalamic tracts.
8) Subcortically originating fibers include the rubrospinal, vestibulospinal and reticulospinal.
MAJOR SENSORY PATHWAYS
Pathway for Conscious Proprioception

T
he pathway for kinesthesia (position and vibration sense) and discriminative touch (well-
localized touch and two-point discrimination) is the posterior column–medial lemniscus
system (Figure 31-1).
Nerve fibers that contribute to this pathway have their cell bodies in the dorsal root ganglia. The
receptors for this system are (1) cutaneous mechanoreceptors (hair follicles and touch pressure
receptors) which convey the sensations of touch, vibration, hair movement, and pressure and
(2) proprioceptive receptors (muscle spindle, Golgi tendon organ, and joint receptors). Muscle
receptors (muscle spindles and Golgi tendon organs) are the primary receptors that convey
position sense. Joint receptors may be concerned with signaling joint movement but not joint
position.