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Clinical

Neurology

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14

2 Diseases Mainly Affecting the Brain


and its Coverings

Characteristics of Diseases of the Brain

Definition:
Diseases of the brain are characterized by both general and localizing mani-
festations. General symptoms and signs include headache, disturbances of
consciousness, (generalized) epileptic seizures, an organic mental syn-
drome, meningism, and signs of elevated intracranial pressure (vomiting,
bradycardia). Localizing signs include focal neurological and neuropsycho-
logical deficits, visual disturbances, cranial nerve deficits, and focal epilep-
tic seizures. None of these features are obligatory for diagnosis, and they
may be present in varying combinations and degrees of severity.

Congenital and Perinatally Acquired Diseases of the Brain

Definition:
Both genetic defects and disturbances occurring during pregnancy may lead
to developmental disorders of the brain (and of the remainder of the ner-
vous system, as well as other organs of the body). These may already be ev-
ident in the newborn infant (e.g., microcephaly), or they may become evi-
dent only in the course of further development. The same is true of brain
injuries occurring during delivery, which are of two types: hemorrhages,
and more or less diffuse hypoxic injuries. The more common modes of pre-
sentation in the early postnatal phase are abnormalities of muscle tone and
pathological reflexes. Later manifestations include delayed psychomotor
development, motor deficits (para- or hemiparesis) and involuntary move-
ments (e.g., athetosis). Epileptic seizures in children and adolescents are
not uncommonly an expression of a congenital or perinatally acquired dis-
ease of the brain.

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Congenital and Perinatally Acquired Diseases of the Brain 15

The Neurological Examination in > an abnormally brisk hand grasp re-


Infancy and Early Childhood flex,
> increased tonic reflexes,
Techniques used in the neurological > increased neck reflexes,
examination of adults are generally > en bloc rotation on testing of the
not applicable to infants and very trunk postural reflex and the head-
young children. Information about on-trunk reflex.
the childs functional state is more
usefully derived from observation of By the end of the 4th month, the infant
spontaneous behavior and of com- should be able to control its head
plex motor reflexes (104, 311). while sitting, lift its head while
In the infant, spontaneous posture prone, and use both hands for play.
should be noted, as well as any bodily The Moro reflex fades, and the Lan-
asymmetries or constantly main- dau and parachute reflexes make
tained postures. It should also be their first appearance. The phenom-
noted whether the head is asymmet- ena listed above are pathological in
ric (plagiocephalic) or otherwise ab- this stage also.
normal in shape (p. 44). The head cir- By the end of the 6th month, the fol-
cumference, body weight, and body lowing findings are highly suggestive
length should be measured, entered or, if pronounced, definitely indica-
into a table for future reference, and tive of a cerebral movement disorder:
compared with normal values for age retained tonic neck reflex and Moro
and sex (Fig. 2.1). reflex, and absent Landau, labyrin-
Primitive motor function in children thine positional, and parachute re-
is initially governed by a number of flexes. The child should be able to lift
reflex mechanisms. These are listed in its head while supine, turn from su-
Table 2.1, and their temporal develop- pine to prone, sit up with support,
ment and clinical significance are turn toward an external noise, and
briefly described. use the whole hand, including the
The stages of normal motor develop- thumb.
ment are shown in Fig. 2.2. By the end of the 9th month, the child
In the initial months of life, the follow- should be able to sit unaided, and the
ing motor abnormalities may indicate lumbar kyphosis becomes less pro-
the presence of a cerebral movement nounced. The following findings are
disorder (cerebral palsy): suggestive of a cerebral movement
> feeding problems,
disorder, in addition to the pathologic
> hypotonia,
reflexes already mentioned: absence
> paucity of movement,
of the body righting and limping re-
> spastic extension of the legs when
actions, and presence of a trunk pos-
the child is lifted, tural reflex.
> intense adductor spasmse.g., dur-
ing diaper changes,
> absence of head lifting while prone
persisting into the 3rd month of
life,

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16 2 Diseases Mainly Affecting the Brain and its Coverings

a 0 3 6 9 12 15 18 21 24 30 36 42 48
cm cm
%
Body length (supine) 97
110 110
Girls 0 4 years 90
75
50
100 25 100
10
3

90 90

kg

80

%
70 97 20
90
75
60
50
25 15
10
50
3

kg
10

5 Weight 5

0 3 6 9 12 15 18 21 24 30 36 42 48
Age (months)

Fig. 2.1ah Head circumference, body length, and weight in childhood and adoles-
cence. (Adapted from: Berner Datenbuch der Pdiatrie: Praktische Richtlinien, Therapie, Ernh-
rungsgrundlagen, Referenzwerte, 4th ed., Stuttgart: Fischer, 1992).

Mumenthaler, Neurology 2004 Thieme


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Congenital and Perinatally Acquired Diseases of the Brain 17

b
0 3 6 9 12 15 18 21 24 30 36 42 48
cm cm
55 55
Head circumference
54 54
Girls 0 4 years
%
53 53
97
52 90 52

51 75 51
50
50 50
25
49 49
10
48 48
3
47 47

46 46

45 45

44 44

43 43

42 42

41 41

40 40

39 39

38 38

37 37

36 36

35 35
Mother ............. cm
34 34
Father ............. cm
33 33

32 32

0 3 6 9 12 15 18 21 24 30 36 42 48
Age (months)

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18 2 Diseases Mainly Affecting the Brain and its Coverings

c
0 3 6 9 12 15 18 21 24 30 36 42 48
cm % cm
97
Body length (supine) 90
110 110
Boys 0 4 years 75
50
25
100 10 100
3

90 90

kg

80

25
70
%
97

90 20
60
75
50
50 25
10 15
3

kg

10 10

5 Weight 5

0 3 6 9 12 15 18 21 24 30 36 42 48
Age (months)

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Congenital and Perinatally Acquired Diseases of the Brain 19

d
0 3 6 9 12 15 18 21 24 30 36 42 48
cm cm
56 56
Head circumference
55 Boys 0 4 years % 55
97
54 54
90
53 53
75
52 52
50
51 25 51

50 10 50

49 3 49

48 48

47 47

46 46

45 45

44 44

43 43

42 42

41 41

40 40

39 39

38 38

37 37

36 36
Mother ............. cm
35 35
Father ............. cm
34 34

33 33

0 3 6 9 12 15 18 21 24 30 36 42 48
Age (months)

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20 2 Diseases Mainly Affecting the Brain and its Coverings

e
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
cm cm
97
90
170 Height 75 170
Girls 1 18 years 50
25
160 160
10
Mother ............. cm 3
150 Father ............. cm 150
%

140 140

130
kg
90
120
%
97 80
110

70
100 90

75 60
90
50
25
80 50
10
3
70 40

kg 30

Weight
20 20
B:
B2 : 3 % 97 %
10 P: 10
P2 : 3% 97 %
Mother .......... kg
Father .......... kg Menarche: 3% 97 %
0 0
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
Age (years)

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Congenital and Perinatally Acquired Diseases of the Brain 21

f
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
cm cm

58 58
Head circumference
57 Girls 1 15 years % 57
97
56 56
90
55 55
75

54 50 54

25
53 53

52 10 52

51 51
3
50 50

49 49

48 48

47 47

46 46

45 45

44 44

43 43
Mother ............. cm
42 Father ............. cm 42

41 41

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
Age (years)

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22 2 Diseases Mainly Affecting the Brain and its Coverings

g
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
cm cm
97
190 190
90
Height
Boys 1 18 years 75
180 180
50
25
170 10 170
Mother ............. cm 3
160 Father ............. cm % 160

150
kg
140

% 90
130
97

120 80
90

110 75 70

50
100
25 60
90 10
3 50
80
40
70

30
kg
20 Weight 20

P:
10 P2: 3 % 97 % 10
Mother .......... kg Testis ml :
Father .......... kg >
ml : 3 %
3 97 %
0 0
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
Age (years)

Mumenthaler, Neurology 2004 Thieme


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Congenital and Perinatally Acquired Diseases of the Brain 23

h
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
cm cm

60 60
Head circumference
59 Boys 1 15 years 59
%
58 97 58

57 90 57

75
56 56

55 50 55
25
54 54
10
53 53
3
52 52

51 51

50 50

49 49

48 48

47 47

46 46

45 45
Mother ............. cm
44 Father ............. cm 44

43 43

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
Age (years)

Mumenthaler, Neurology 2004 Thieme


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All rights reserved. Usage subject to terms and conditions of license.
Mumenthaler, Neurology 2004 Thieme

Table 2.1 Reflexes in infancy and childhood

24
Reflex Mode of testing and response Time normally In cerebral palsy Remarks
present

2
z Dolls eyes Infant awake and recumbent, passive Birth to 10th day Persistent

Diseases Mainly Affecting the Brain and its Coverings


# phenomenon turning of the head. Gaze stays in the
#
original direction
Positional reactions

#
Postural reflexes

#
# Step reflex Child held up under axillae, with soles First few weeks of
# lightly touching the ground. Stepping life
# movements, body carried along by exa-
miner
#
# Crossed Child supine, passive maximal flexion of Always abnormal Note tone in foot Spinal reflex
#
# stretch reflex one hip and knee. Extension of opposite
# leg and foot
#
# Crossed Child supine, passive maximal flexion of Birth to 7th12th Abnormal only Differential diagnosis:
| flexion reflex one hip and knee. Flexion of opposite leg month after the first movement accompanying
year hip contracture of other
etiology
z z Support Legs: standing the child up on its feet, Increasing from Increased, Physiological astasia in 2nd
# # reactions or the examiners pressing on the soles birth to 4th6th persistent and 3rd month
# #
# # of the feet, induces extension of legs. month
# # Arms: pressure induces extension
# #
# # Foot placing Child held up under axillae, dorsum of Only in the first
# # reflex foot lightly touches edge of table. Child few weeks of life
# # actively raises leg and plants foot on
# #
# # table
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Mumenthaler, Neurology 2004 Thieme

z z Tonic hand Examiner touches palm with finger. Birth to 3rd month Increased after Possibly abnormal after 3rd
# # grasp reflex Clenching of fist 3rd month month, definitely abnormal
# # after 6th month
# #
# # Tonic foot Touching the sole of the foot induces Birth to 12th Absent at birth, Marked clawing of the toes
# #
# # grasp reflex clawing of the toes month later increasing may impair gait
# # (toe reflex)
# #
# # Tonic spine Child prone, examiner strokes the skin of First few months Increased Usually barely perceptible
# # reflex

Congenital and Perinatally Acquired Diseases of the Brain


the back. Child turns to stroked side, ex- of life
Positional reactions

# # tends ipsilateral leg, and flexes contralat-


# #
Postural reflexes

# # eral leg

# # Tonic neck Child prone or supine, head slowly pas- Birth to 5th6th Increased; pre-
# # reflex, sively turned. Altered posture or tone. month; absent sent in sleep af-
# # asymmetric On the side to which the head is turned, during sleep ter 6th month
# #
# # the arm is extended and the leg is ex-
# # tended at the knee and ankle. On the op-
# # posite side, the arm is flexed and the leg
# # is flexed in all joints
# #
# # Tonic neck Child supine, head passively flexed. Flex- Birth to 5th6th Increased; pre-
# #
# # reflex, ion of both arms and flexion of both legs month; absent sent in sleep af-
# | symmetric at hips, possibly also at other joints during sleep ter 6th month
#
# Tonic labyrin- Child supine, head passively flexed. Ac- Never pure Increased Spastic children do not like
# thine reflex tive retraction of head and shoulders, to lie prone!
| contraction of trunk and hip extensors,
opening of mouth
Child prone, head passively flexed. Flex- Rarely pure Increased
ion of head, flexion and adduction of
arms and legs

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Mumenthaler, Neurology 2004 Thieme

Table 2.1 Reflexes in infancy and childhood (Cont.)

26
Reflex Mode of testing and response Time normally In cerebral palsy Remarks
present

2
z

Diseases Mainly Affecting the Brain and its Coverings


# Labyrinthine Child turned prone or held upside-down Appears in 2nd Absent
# postural reflex by the feet. Retraction of head month; from Delayed
# (head) 3rd4th to 6th
#
# month
# Child sitting, trunk flexed to one side: Persistent from Sometimes asymmetrical
# head kept vertical the 3rd or 4th (in presence of hemi-
# month onward, paresis)
#
# though masked by
# other movements
Righting reflexes

#
# Landau reflex Child held horizontal in abdominal sus- 4th18th month Absent, delayed,
# pension: head retracted, trunk and legs sometimes pro-
extended. Passive flexion of head: all longed
#
# joints flexed
#
# head-on-trunk Child supine, head turned passively and Birth to 12th Delayed, pro- Rotation en bloc
# reflex rapidly to one side. After 1/3 rotation, month tracted, rotation requires treatment
# the trunk follows with a torsional move- en bloc
# ment
#
#
# Trunk postural Child supine, shoulder (or pelvis) pas- Birth to 4th6th Increased, some- Test is negative when the
# reflex sively rotated. 1st stage: the trunk fol- month times prolonged pelvis begins to rotate only
# lows en bloc. 2nd stage: the trunk fol- 4th6th to 14th after 80 of shoulder rota-
# lows with torsion month; birth to tion
#
# Supine position, hips flexed to right 6th month
# angle, rotation of trunk on thighs
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Mumenthaler, Neurology 2004 Thieme

z Moro reflex Child supine. A sudden blow on the bed Birth to 4th7th Positive for lon- Absent in the first few
# induces movement of the arms to the month ger period months in cases of severe
# sides and then forwards, as if in an em- brain injury
#
# brace, with hands open. The same re-
# sponse occurs when the patient is held
# supine in the air and the head is sud-
# denly let go
z Body righting

Congenital and Perinatally Acquired Diseases of the Brain


Attempting to push the child out of any When sitting, from Delayed, absent
#
# reaction body position induces contraction of the 7th month onward or deficient
# ipsilateral muscles
Balance reactions

#
# Limping Attempting to push the child out of any When sitting, from Delayed, absent When standing, better to-
# reaction body position induces a buttressing 7th month onward or deficient ward the affected side;
# movement, possibly across the midline when sitting, child may use
#
unaffected arm across mid-
# line to affected side
#
# Parachute reflex Child in ventral suspension or kneeling From the 6th to Absent, delayed, An abnormal reflex, even if
# position and suddenly pushed forward. 9th month onward or incomplete asymmetric or if the hands
# Extension of arms, palms open are clenched; a statokinetic
#
| reaction

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