F

Special Article

Magnetic Resonance Spectroscopy in Pediatric Neurology
Sheffali Gulati, Tariq Shah 1, Shaji Menon, Rama Jayasundar 1 and Veena Kalra
Departments of Pediatrics and 1Nuclear Magnetic Resonance, All India Institute of Medical Sciences, New Delhi,
India.

Abstract, In the last three decades a range of non-invasive biophysical techniques have been developed, of which Magnetic
Resonance (MR) has proved to be the most versatile. Its non-invasive and safe nature has made it the most important
diagnostic and research tool in clinical medicine. MR Spectroscopy (MRS) is the only technique in clinical medicine that provides
non-invasive access to living chemistry in situ. This article focuses mainly on proton MRS in brain and also phosphorus MRS
in calf muscle, with particular reference to the pediatric population, the normal spectrum and its use in various disease conditions
in the practice of pediatric neurology. Few representative case studies among different disease groups have also been detailed.
[Indian J Pediatr 2003; 70 (4) : 317-325]

Key words : Magnetic resonance imaging; Magnetic resonance Spectroscopy (MRS); Proton MRS; Phosphorus MRS
Physiological studies both under normal and pathological
conditions have always been important to medical
science. While studies under normal conditions are crucial
for understanding the functioning of a system, those
under pathological conditions are important for studying
the mechanisms of disease in order to design appropriate
treatments. Many major technological advances have
been applied in biological and clinical research: the
resulting improvements in instrumentation have helped
in conducting more accurate and elegant experiments to
enable the living system to be interrogated. Many
metabolic studies to date, however, have been invasive to
varying degrees. This implies that the metabolism is often
disrupted during the process of extraction of the sample
and consequently, the results may not be representative of
the in vivo situation.
In the last three decades, however, a range of noninvasive biophysical techniques has been developed, of
which Magnetic Resonance (MR) has proved to be the
most versatile, t Its non-invasive and safe nature has
made it the most important diagnostic and research tool
in clinical medicine. In biological studies, MR imaging
(MRI) is not a completely new technique since there are
other imaging m e t h o d s available. H o w e v e r , MR
Spectroscopy (MRS) is the only technique in clinical
medicine that provides non-invasive access to living
chemistry in situ.
The technique of MR utilises the magnetic properties
of atomic nuclei. The first MR experiments were
conducted by the groups of Bloch and Purcell in 1946, for
which they were jointly awarded the Nobel Prize in 1952.
From being a m e t h o d for measuring the magnetic

Reprint requests : Prof. Veena Kalra, Head, Department of
Pediatrics,All India Institute of MedicalSciences,New Delhi, India.
Fax No. 91-11-26862663.E-mail : kalra_veena@hotmaiLcom
Indian Joumal of Pediatrics, Volume 70---April, 2003

properties of atomic nuclei, MR has evolved into a
powerful analytical tool in both laboratory and clinical
settings. This has been made possible largely through the
use of m o d e r n computer systems, combined with
technical advances in magnet design. A variety of
biological investigations have been carried out using MR,
ranging from tissue metabolism, tissue charaterization,
blood flow and functional studies to drug binding studies
in pharmaceutical research.
In the proton spectra from brain, metabolites such as
N-acetylaspartate (NAA), Creatine/Phosphocreafine (Cr/
PCr), Choline (Cho) containing compounds, glycine,
glutamine, glutamate, taurine, aspartate, myo-inositol and
alanine can be observed. While NAA is considered a
neuronal marker, Cr/PCr is involved in high energy
metabolism, and Cho is involved in membrane turnover.
In pathology, increase or decrease of metabolites or
presence of new metabolites are usually observed.
Although a number of nuclei can be studied using MR,
proton is the most widely studied nucleus. This article
focuses on proton MRS in brain, with particular reference
to the pediatric population.
MRS can be useful in evaluation of ischemic penumbra
of stroke, brain development (myelin and neuronal
dysgenesis), head trauma (evaluation of cerebral damage
not visible with MRI), degenerative disorders
(identification of microscopic pathology not visible with
MRI); and metabolic disease (metabolic disturbances with
specific metabolic patterns). 2~ The differential diagnostic
uses of metabolites of MRS is detailed in Table 1

Normal Finding in MRS
It is well known that MR imaging is highly sensitive in
assessment of state of myelination during postnatal brain
development. 4,5 This seems to be true for proton MR
317

Sheffali Gulati et al
s p e c t r o s c o p y also as t h e r e are s t r i k i n g d i f f e r e n c e s in
s p e c t r a w i t h r e s p e c t to a c h i l d ' s age. T h e v o l u m e l o c a l i z e d
s p e c t r a of w h i t e m a t t e r i n h e a l t h y c h i l d r e n are d o m i n a t e d
by three prominent peaks from the methyl groups : NAA
a t 2 p p m , C r / P C r a t 3 p p m a n d C h o a t 3.2 p p m .

C o m p a r i s o n of relative p e a k intensities of N A A , C r / P C r
and Cho during normal brain maturation
reveals
considerable changes in Ch and NAA. Whereas a
s p e c t r u m in n e o n a t a l b r a i n at the o n s e t of m y e l i n a t i o n
shows high signal intensity for the Cho containing

TABLE 1. D i f f e r e n t i a l D i a g n o s t i c U s e s of M e t a b o l i t e s in M R S 2,3
Metabolite
( N o r m a l cerebral
concentration)

Characteristics

Decreased

Increased

1. Creatine (Cr)
and phosphocreatine
(PCr) (8raM)

a. Marker of intact brain
metabolism.
b. Biosynthesis in kidney
and liver

9
9
9
9
9

SIADH
Hypoxia
Stroke
Tumor
Trauma

9

Hyperosmolar increasing
with age

2. Lactate (Lac)
(MmM : not visible
because of its low
concentration)

a. End product of anaerobic
metabolism.
b. Marker of ischemia
c. Interruption of Krebs
cycle and inhibition of
pyruvate dehydrogenase
increases brain lactate level

9 Unknown

9
9
9
9
9
9

Hypoxia
Stroke
ICH
Hydrocephalus
Near drowning
Lymphoma
Tumor

3. Myoinositol
(mI) (5mM)

a. Astrocyte marker
b. Cell volume regulator
c. Increase in osmotic stress
of brain

9
9
9
9
9

Stroke
Tumor
Hypoxia
Hyponatremia
SIADH

9
9
9
9

Neonates
Renal failure
Diabetes mellitus
Recovered hypoxia

4. N-Acetyl Aspartate
(NAA) (8-9mm)

a. Most intense signal
in normal brain.
b. Normal NAA indicator of
normal neuronal and
mitochondrial function

9
9
9
9

9
9
9
9

Canavan's
Axonal recovery
(MELAS, MS)
Infant development

9
9
9

Hypoxia-Alexander
Infancy
Ischemia-herpes
Epilepsy
MS
Tumor
Trauma
Any degenerative disease
Hepatic encephalopathy
Stroke
SIADH
Cryptococcoma

9
9
9
9

Trauma
Tumor
Stroke (common)
Neonates

9

9

Contamination from outside
volume e.g. scalp
Lymphoma
Necrotic area in tumor
Tuberculoma
Some type of Brain abscess
TOXO
Trauma

9
9
9

Acute hepatic encephalopathy
Hypoxia
Near drowning

9

9

5. Choline (Cho) (1.5raM)

a. Membrane synthesis
b. Membrane degradation
is the main source
c. Dietary and systemic
sources diffuses freely
into brain

9
9
9
9

6. Lipids

a. Presence always pathological
(contamination not considered)
b. Indicative of severe pathological
process liberating membrane lipids

9 Not detectable

9
9
9
9
9

Glutamine (Gin) and
Glutamate (Glu)
(Gin approx 5raM,
Glu approx 10 mM)

a. Astrocyte marker
b. Increase in state with
dystrocyte replacement
of neurons
c. These act as neurotoxins,
inhibition of redox cycle leads
to accumulation of lactate

9 Trauma
9 SIADH
9 Hyponatremia

ICH : Intracranial hemorrhage; MS : Multiple sclerosis; TOXO : Toxoplasmosis; MELAS : Myopathy, encephalopathy, lactic acidosis with stroke
like episodes; SIADH : Syndrome of inappropriate antidiuretic hormone secretion

318

Indian Journal of Pediatrics, Volume 70---April, 2003

Role of Magnetic Resonance Spectroscopy in Pediatric Neurology
compounds and relatively low signal intensity for NAA,
this pattern is reversed in children over one year of age, in
whom myelination is complete. Other visible compounds
are glutamate, glutamine glycine, taurine and inositol.
These are seen depending on their concentration.6

Epilepsy
A decrease in level of NAA is helpful in accurate
presurgical localization of epileptic focus 3. MRS is useful
in temporal lobe epilepsy prior to surgery. In mesial
temporal sclerosis, there is a decrease in NAA signal from
the affected temporal lobe. Gray matter heterotopias are
found in 10% children with retractory seizure. These can
be diagnosed by MRS.

Brain Tumors
Wang et al reported that for intraaxial tumors, lower NAA
to choline ratios are associated with more aggressive
tumor type7 When discriminant analysis with NAA and
Cr/Pcr to Cho ratio is done. Diagnosis can be established
with approximately 83% certainty for cerebellae tumor s.
Pediatric cerebeUar tumors are dominated by three major
t u m o r types primitive neuroectodermal tumor,
astrocytoma and e p e n d y m o m a . Most primitive
neuroectodermal tumors have low NAA/Cho and Cr /
Pcr Cho ratio. The C r / P c r Cho ratio is higher in
ependymoma than in astrocytoma. Preus MC et al (in
society of magnetic resonance in medicine meeting,
A u g u s t 1994) reported that five major type of
supratentorial tumor in adults can be distinguished by
using in vivo proton MRS.9Reduction or absence of NAA
is seen in tumors derived from astrocytes like glioma,
meningiomas and craniopharyngeoma. Lactate is seen in
cystic areas and necrotic tissues, m,n
There are five biochemical defects common to the
majority of brain tumors, as measured by MRS. They are
as follows : NAA is decreased; lactate is seen, lipid is seen
(especially in areas of necrosis); creatine and phosphocreatine (PCr) is decreased; and choline is increased.

Neurocysticercosis (NCC)
Evaluation of inflammatory granulomas using MRS by
Jayasundar R et al revealed an extremely low level of
metabolites together with a poor signal/noise ratio in
NCC. Lipids were seen in 86% of tuberculomas while in
NCC and nonspecific inflammatory granuloma, lipids
were seen in 20-21% cases?2
Intracranial Tuberculomas
Gupta et al reported the result of localized proton MR
spectroscopy carried out on two patients with intracranial
tuberculoma,la A large resonance was observed between
0.7 and 1.6 ppm which was assigned to (CH2) group of
saturated fatty acids. This peak was attributed to large
lipid fraction present in tubercle bacilli. Distinct
resonances of serine and phenolic lipids were seen in ex
vivo and in vitro studies of tuberculous granuloma. The

Indian Journal of Pediatrics, Volume 70--April, 2003

lipid extract of granuloma and mycobacterium
tuberculosis showed phenolic lipids at 7.1 and 7.4 ppm, a
constituent of cell wall of the bacteria in a tuberculoma.
From these studies, it is clear that intracranial tuberculomas are characterized by a spectral pattern that
involves primarily lipid resonance. Jayasundar et al have
also reported the predominant occurrence of the lipid
resonances in tuberculomas,m3

Brain Abscess
Kim et al performed MR spectroscopy in seven patients

with pyogenic brain abscess and in seven patients with
necrotic or cystic brain tumor. Six of seven patients with
abscess showed resonance from lactate, valine, alanine,
leucine, acetate succinate and unidentified metabolites
(2.2, 2.9, 3.2, 3.4, and 3.8 ppm). In contrast, six of seven
patients with turaor showed only a resonance attributed
to lactate. Dev et al used localized MR spectroscopy on 24
patients with pyogenic brain abscess. Post aspiration
studies were performed for 12 patients treated with
combined medical and surgical therapy and 2 patients
treated medically. Lactate and amino acid were seen in all
of the spectra for all patients, regardless of time of
spectroscopy after the onset of combined medical and
surgical therapy. Acetate and pyruvate disappeared after
1 week of combined therapy. Grand et al suggested that
the presence of amino acid resonance could provide a
means for identifying abscesses and distinguishing these
masses from necrotic region of brain tumor) 3

Head Trauma
MR spectroscopy provides a way to evaluate tissue
viability and to characterize the metabolic disorders
associated with each type of injury. Result of MR
spectroscopy may provide insight as to how patients
should be treated and whether treatment is effective, thus
helping neurosurgeons make clinical decision. Wan et al
demonstrate that the injured region had highly increased
lactate signal (Lactate / creatine = 0.8 approximately,
three to five times normal) and decreased NAA (NAAJ
N A ~ = 0.26), creatine (creatinei / creatiner = 0.25) and
choline (cholinei/cholinec = 0.2 signals (subscripts T and
"c" denotes signals from injured and control region
respectively. Increase in lactate is related to tissue
swelling and ischemia after trauma, and decrease of
choline, creatine and NAA is related to edema, which has
a dilution effect.9J3

Stroke
MR spectrum in areas of stroke and in diseases like sickle
cell disease or MELAS shows an elevated lactate even
before neuroimaging can pick up these abnormal areas.
Areas of transient ischemic attack may show decrease in
N A A / C r ratio but, lactate is not detected. Follow up
MRS studies show a return of NAA level to normal
indicating the recovery of partial neuronal injury7

319

Sheffali Gulati et al

Rett Syndrome (RS) and Autism

Adrenoleukodystrophy (ALD)

Hashimoto et al performed proton MRS of the brain in 3
cases of RS in comparison with autism and controls. RS is
a clinically defined disorder characterized by autistic
behaviour and cognitive and motor skill loss early in life.
The older patient with RS demonstrated decreased NAA
/Cho and N A A / C r ratio when compared with the autism
and control groups, whereas the younger patients did not
demonstrate these decreased metabolite ratios. This data
suggest that there m a y be a s e c o n d a r y d e g e n e r a t i v e
process of late onset RS which is pathophysiologically
different from autism25,~

Childhood ALD is an X-linked disorder with a defect in
peroxisome that involves impaired function of single
specific e n z y m e (Lignoceryl-coenzyme A ligase). This
defect leads to accumulation of very-long chain fatty acids
(VLCFA) in central nervous system, adrenal cortex and
testes. Neurological symptoms include attention disorder,
seizures, visual and auditory disturbances progressively
leading to vegetative state and eventual death. Tzika et al
reported that MRS from white matter lesion showed 65%
reduction in the ratio of N A A / t C r ; 55% increase in the
ratio of choline-containing compounds (Cho) to tCr, 94%
increase in ratio of glutamate, glutamine or inositol to tCr
and lactate accumulation. 23

Pelizaeus-Merzbacher disease
P e l i z a e u s - M e r z b a c h e r disesae is a rare m y e l i n a t i o n
disorder with muscular hypotonia, psychomotor
retardation and ocular nystagmus. ~7The basic defect is an
X-linked recessive disorder with loss of myelin due to an
in b o r n a b n o r m a l i t y of p r o t e o l i p i d p r o t e i n , a major
protein component of myelin. MR spectroscopy in this
case revealed drastically diminished NAA and elevated
choline in affected cerebellar white matter. 6

Canavan's Disease
C a n a v a n ' s disease is an i n b o r n error of m e t a b o l i s m
characterized by aspartoacylase deficiency with cystic
degeneration of oligodendroglia and astrocyte swelling.
The patients present with megalencephaly, blindness,
spasticity and p s y c h o m o t o r retardation. Aydinli et al
demonstrated elevated levels of N-acetylaspartate (NAA)
compare to choline and creatine in the frontal region
w h i t e matter. MRS f r o m areas of d e m y e l i n a t i o n
demonstrate increase in N A A / C h o ratio, this is not only
due to reduction of choline containing compound in brain
but also due to increase of NAA concentration. The in
vivo measurement of NAA, choline and creatine in the
brain by MRS offers an additional non invasive diagnostic
test for establishing the diagnosis of Canavan's disease.
CSF, plasma and urine also shows high level of NAA due
to deficiency of aspartoacylase. 1s,~9

Metachromatic Leukodystrophy (MLD)
MLD is an autosomal of recessive lysosomal storage
disease caused by deficiency of lysosomal activity of
arylsutatase A, which results in an a c c u m u l a t i o n of
sulfated lipids in myelin sheath in both peripheral and
central n e r v o u s system. The late infantile f o r m is
c h a r a c t e r i z e d by loss of m o t o r and c o g n i t i v e skills
behaviour problems starting before 4 years progressing to
death. P r o t o n MRS r e v e a l e d a m a r k e d r e d u c t i o n of
neuronal marker NAA in affected white and gray matter
and elevated lactate in demyelinated areas in contrast to
other Leucodystrophies MLO patients w h o showed a
generalized increase of brain myo-inositol (2-t0-3 fold in
w h i t e m a t t e r ) , i n d i c a t i n g a specific role in the
pathophysiology of demyelination of MLD. 2~
320

Alexander Disease
Alexander disease is a leukodystrophy characterized by
presence of numerous Rosenthal fibers (RFs) i.e. inclusion
bodies in astrocyte. The clinical manifestation includes
psychomotor deterioration and megalencephaly.
Elevation of ~, ]3-crystallin and head shock protein 27
(HSP 27) in CSF and frontal dominant leukodystrophy is
diagnostic. Takanashi et al demonstrated decrease in NAA
with a lactate peak in frontal region but a normal NAA
level without a lactic peak in posterior region. 24

Megalencephalic Leukoencephalopathy with Subcortical Cysts/Van der Knaap
Vacuolating m e g a l e n c e p h a l i c l e u k o e n c e p h a l o p a t h y
(VML) with subcortical cysts is a n e u r o d e g e n e r a t i v e
disorder clinically characterized by megalencephaly with
onset in the first year of life, progressive ataxia, spasticity
and relatively spared cognitive function. Conventional
MRI findings consist of diffusely abnormal cerebral white
matter with subcortical cysts. Recent single-voxel proton
MR spectroscopy studies have s h o w n mild metabolic
abnormalities in the white matter. 1H-MRSI examinations,
which can provide simultaneously metabolic information
from m a n y different brain regions, s h o w e d inhomogeneous decreases in all normally detected metabolites
with significant widespread decreases in the ratio of Na c e t y l a s p a r t a t e to c r e a t i n e + p h o s p h o c r e a t i n e and
concomitant small increases in lactate in the white matter
of both hemispheres. Metabolic abnormalities w e r e
milder in the frontal white matter and more severe in the
posterior white matter. The 1H-MRSI pattern of the gray
matter was normal in both patients. 25

Mitochondrial Disorders
Leigh Disease (Subacute Necrotizing Encephalomyelopathy)
Leigh disease is a recessively inherited familial disorder of
infancy and childhood with variable clinical expression.
This disease is caused by one of the several possible
deficiencies that affect the oxidation of pyruvate, resulting
in abnormally elevated levels of p y r u v a t e and lactate.
Indian Journal of Pediatrics, Volume 70--April, 2003

Role of Magnetic Resonance Spectroscopy in Pediatric Neurology
Laboratory finding includes metabolic acidosis with
elevated lactate and pyruvate concentration in blood and
CSF. Characteristic necrosis are found in spinal cord,
brain stem, and basal ganglia, especially putamen. MRS
reveals elevated levels of lactate in the head of nucleus
caudatus. The levels of NAA are low in areas of stroke
associated with neuronal loss. MRS is also useful in
assessing Leigh disease and in monitoring its treatment.
Because at times blood and CSF lactate level do not reflect
the brain lactate level. MRS is the most reliable method to
monitor lactic acidosis in the brain. MRS can even detect
areas of early ischemic changes earlier than MRI.6,9
MELAS (Myopathy, EncephaIopathy Lactic Acidosis
with Stroke like lesion)

MRS can detect area of poor perfusion and stroke earlier
than other neuroimaging modalities. These areas show
elevated levels of lactate. Successful treatment with
sodium dichloracetate and improvement in perfusion
lead to reduction in lactate/creatine ratio. The decrease in
NAA in area of hypoperfusion may return back to normal
on reperfusion indicating reversible damage. 6,9

Hepatic Encephalopathy
Proton MRS in hepatic e n c e p h a l o p a t h y shows an
elevation of Glutamine levels and a reduction in choline,
myoinositol levels. A decrease in levels of myoinositol is
seen in patients even with overt neurological impairment,
therefore it is possible to screen for subclinical hepatic
encephalopathy with MRS.
Friedreich Ataxia
Friedreich's ataxia (FA) is the most common form of
autosomal recessive spinocerebellar ataxia and is often
associated with a cardiomyopathy. The disease is caused
by an expanded intronic GAA repeat, which results in
deficiency of a mitochondrial protein called frataxin. 3~p
nuclear MRS has shown that these patients have lower
rates of mitochondrial ATP production in skeletal muscles
than controls. This can be used to see response of newer
possible therapeutic modalities.3~

Case Studies
1.

Attention - Deficit-Hyperactivity Disorder (ADHD)
Toft PB indicated a particular vulnerability to hypoxic
injury of striatal neurons in the developing brain.
Striatum is important for context recognition and
behaviour. An elevation of lactate in striatum a part of
frontostriatal circuit, which plays a vital role in human
behaviour in neonatal period following adverse perinatal
events like asphyxia, germinal matrix hemorrhage and
intrauterine growth retardation may play important role
in development of ADHD in later life.26

2.

Dyslexia
Dyslexia is characterized by language disorder in which
reading ability is compromised because of poor
phonologic skills. MRS in these patients revealed a
difference in spectrum between them and normal control
subjects in the temporo-parietal lobe. The cerebellum is
also, biochemically, assymetrical on MRS in dyslexic men
suggesting altered development of this organ.
Instructional intervention therapy that improved
phonological performance in dyslexic boys was
associated with low levels of lactate in left anterior
quadrand of brain compared to untreated patients?7~8

Tuberous Sclerosis
MRS spectroscopy of tubers in patients with tuberous
sclerosis revealed significant increase in myoinositol/
creatine ratios in the tubers. The N A A / c r e a t i n e / P c r
ratios in the tubers showed a significant decrease, and
was considered to reflect a reduction of cranial neurons.
The increase in myoinositol/creatine/Pcr ratio in tubers
was considered to be a reflection of increase in glial
tissue.29

Indian Joumal of Pediatrics, Volume 70---April, 2003

3.

K, a 13-year-old girl presented with right focal
seizures with secondary generalization from the age
of 10 years. She had multiple ring lesions on CT
scan of head. Besides anticonvulsants she was given
2 courses of albendazole therapy and one course of
praziquantel therapy. In left parietal region a lesion
persisted for over I year for which MRI and MRS
were done (Fig. la, lb). This was suggestive of
neurocysticercosis which can be easily distinguished from a tuberculoma ( Fig. 2a, 2b)
A, an 8-year-old boy presented with progressively
diminishing vision for 6 months and regression of
motor milestones. His head circumference was
normal, pupils normal size and normally reacting to
light, generalized hypertonia and brisk reflexes. His
MRI showed bilateral symmetrical changes in
posterior parieto-occipital white matter. His Aryl
sulfatase was normal but very long chain fatty acids
were increased (Amount of C 26:0 and the ratios of
C 24/22 and C 26/22 were higher than normal).
Results were consistent with X linked adrenoleukodystrophy. His MRI and MRS findings are depicted
in Fig. 3a, 3b. The spectrum from occipital lobe
shows low level of Cr and Cho and drastic decrease
in NAA though in literature high Cho and lactate
have also been reported. The decrease in NAA in
ALD is more than observed in other leukodystrophies.
Am, a 6-year-old boy presented with progressively
increasing difficulty in walking for 2.5 years,
generalized seizures and a progressively increasing
large head size. His head circumference was 58 cm
and had mild hypertonia bilateral lower limbs with
brisk reflexes. His MRI showed extensive white
matter hyperintensities scattered symmetrically
bilaterally throughout the white matter. These

321

Sheffali Gulati et al

Fig. 2a
i

3.5

41

I

I

2.5

Chemical

r

1,5

shift

r

!

0.5

i' ppm

Fig. lb
Fig. l(a). T2 weighted MRI image from a child with persistent
Neurocysticercosis. (b) The proton spectrum shows
reduced levels of NAA, Cr and Cho. The peak observed at
2.00 ppm is identified as N-acetyl aspartate (NAA), at
3.00prom Creatine / Phosphocreatnine (Cr) and 3.2 ppm
Choline (Cho).

4.

322

involved the subcortical white matter as well as the
p e r i v e n t r i c u l a r region. The c h a n g e s w e r e m o r e
marked in the frontal, temporal and parietal region.
S u b c o r t i c a l c y s t s w e r e also p r e s e n t . H i s a r y l
s u l f a t a s e a n d v e r y l o n g c h a i n fatty acids w e r e
n o r m a l . A d i a g n o s i s of Van d e r K n a a p or
Megalencephalic leukoencephalopathy with
subcortical cysts was made. His MRI and MRS are
depicted in Fig 4a, 4b.
BK, a 1 2 - y e a r - o l d child p r e s e n t e d w i t h
progressively worsening ataxia since the age of 5
years, scoliosis, no telangiectasia, bilateral upgoing
plantars, just elicitable ankle jerks and a negative
R o m b e r g . He h a d no r e c u r r e n t s i n o p u l m o n a r y
infections. N e r v e c o n d u c t i o n v e l o c i t y s h o w e d
peripheral neuropathy. A diagnosis of Friedreich's
ataxia was made. His MRS of calf muscle before and

--

|

I

3.5

9

l

~

2.5

1.5

Chemical

shi f t

"

9

I

I

0.5

/ ppm

Fig. 2b
Fig. 2 (a). T2 weighted MR/image from a child with Tuberculoma.
(b) The proton spectrum shows a huge lipid peak with no
signal from NAA, Cr and Cho.

5.

after 6 m o n t h s of t r e a t m e n t with t h i a m i n e and
amantadine hydrochloride is s h o w n in Fig 5a, 5b.
There was no improvement on treatment.
RJ, a 10-year-old boy presented with hyperactivity
and normal IQ. He had combined type of A D H D
w i t h significant inattention, h y p e r a c t i v i t y a n d
Indian Journal of Pediatrics, Volume 70~April, 2003

Role of Magnetic Resonance Spectroscopy in Pediatric Neurology

3 5

2.5

Chemical

1.5

0.5
f

shift :"
Fig. 3b

Fig. 3 (a). TI weighted MRI image from a left occipital lobe in a child
with Adrenoleukodystrophy. (b) The proton spectrum
shows low level of Cr and Cho and drastic decrease in
NAA. The decrease in NAA in ALD is more than observed
in other leukodystrophies.

3.5

2.5

Chemical

I 5

~.5

shift / ppm
Fig. 4b

Fig. 4 (a). TI weighted MRI image from a left parietal lobe in a child
with Van der Knaap or megalencephalic leukoencephalopathy with subcortical cysts. (b) the proton spectrum
shows reduced level of all metabolites.

Fig. 5a

5

0

-5

-~0

-i5

Chemical shift / ppm

~

5

6

-5

-i0

-t5

Chemical shift / ppm

Fig. 5. Phosphorus spectrum from calf muscle from a child with Friedreich's ataxia (a) Pretreatment (b) Post treatment follow up. The
spectrum identifies prominent phosphocreatine, with J3ATP, ?ATP, o.ATP inorganic phosphate.

Indian Joumal of Pediatrics, Volume 70--April, 2003

323

Sheffali Gulati et al

Fig. 6b

1Ill

3.5
3.5

2.5

1.5

0.5

Chemical s h i f t

/ ppm

2 5

1 5

Chemical s h i f t

0.5

/ ppm

Fig. 6d

Fig. 6c
Fig. 6. T2 weighted MRI image from a (a) right prefrontal cortex (b) left prefrontal cortex from a child with Attention Deficit Hyperactivity
Disorder. The proton spectrum acquired from right frontal lobe (c) is normal but abnormal spectrum with reduced metabolites level
observed in the left frontal lobe (d).

i m p u l s i v i t y d i a g n o s e d b y D S M IV c r i t e r i a . H i s
Single photon Emission Computed Tomography
(SPECT) showed left basifrontal hypoperfusion.
H i s M R I a n d M R S are d e p i c t e d in Fig. 6a-d.

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Contributors
VK and SG were pediatric neurologists incharge of the cases, SG and
SM drafted the manuscript, VK critically reviewed the manuscript
and will act as guarantor for the paper, TS performed the MRS in all
patients and RJ critically reviewed the manuscript

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