Hanan Fathy

• Female patient Waad Ahmed Youssof , 2 years old

from Elamria, admitted on 22/2/09.
• C/O:
 An attack of disturbed sensorium, sweating
Transient loss of vision
(day of admission)
Present history:

• The patient is a known case of glycogen storage disease type III, she is on regular
starch supplementation every six hours, the mother missed the night supplements.
In the morning the mother found her child irresponsive and with profuse sweating, she
tried giving her her missed amount of starch but the child was unable to take oral
feeding.The mother sought medical advice at Elamria hospital RBS checking
revealed very low level, she was given I.V fluids and her blood glucose level was
checked regularly, the patient improved but she didn't`t regain full conscious and
hence was referred to Elsaatby hospital,
Upon admission the patient had disturbed sensoium her blood sugar level was low, she
was given I.V glucose for correction of hypoglycemia her condition gradually
improved, however upon regaining concious the patient complained that she can`t
see. She remained so for over night then in the morning she regained full
conciousness and good vision.
C.T brain during period of loss of vision : unremarkable
Ophthalmology consultation : no gross abnormality can be seen and MRI brain was
recommended.
Past history: the patient was diagnosed at the age of 8 months as having GSDIII( via liver biopsy in
Elmonofia).
• Previous attacks of hypoglycemia but with no loss of vision
Family history:
Her sister was diagnosed
With the same
Condition
Perinatal history : C/S delivary, smooth prenatal, natal and post natal periods.
Developmental history: the patient can walk alone she can climb up stairs step by step , she can say sentences
composed of 3-4 words.
Nutritional history: she eats family food plus regular intake of corn starch ever 6 hrs
Vaccination history : according to schedule.
• The patient is conscious, cooperative well oriented, mild pallor,
no jaundice no cyanosis ,no abnormal facial features no special
decubitus.
• HR:110 b/min( regular, average force and volume, no special character,
vessel wall not felt, peripheral pulsations felt) RR:32cycle/min
Temp :37

Wt:11 kg(average for age) Ht:81 cm

HC:48 cm
Head and neck: normal facies,neck freely mobile no palpable.Ns,
carotids felt bilaterally equally pulsating, thyroid not felt.
• Chest and heart are clinically free
• Abdomin is lax .The liver is felt 3 cm below the
costal margin firm in consistency, sharp border,
smooth surface not tender not pulsating the left lope
is felt about 3 cm in the mid line the upper border is
in the fifth space mid clavicular line.
• Spleen is not felt abdominally, upper pole in the
eighth space mid axillary line.
• Normal female genitalia
• Extremites: normal no LL oedema
• Neurological examination:
• The patient is fully conscious ,cooperative oriented
• Cranial nerves are intact (no clinical evidence of cranial nerve palsy)
• Motor system: good muscle status, normal tone ,no abnormal
movements, muscle power (pt not cooperative ), normal gait.
• No ataxia
• Reflexes : deep reflexes are easily elicited and brisky, abdominal
reflexes intact , flexor planter reflex
• No clonus.
• Sensations (what was possible to examine)superficial sensations intact,
deep sensations couldn't`t be assessed
• No signs of meningeal irritation.
• The patient is a known case of GSDIII.
• Admitted in an attack of hypoglycemia.
• She suffered from transient loss of
vision.
• The patient has hepatomegally, and ?
exaggerated deep reflexes.
•
Routine chemical investigations :
Except for hypoglycemia , no abnormal findings

CK:1056 u/l LDH:240u/l

• Early clinical signs in hypoglycemia reflect the appearance of
physiological protective mechanisms initiated by hypothalamic sensory
nuclei .

• Such symptoms include sweating, also termed diaphoresis,

tachycardia, anxiety and hunger.

• If unheeded, these symptoms give way to a more serious CNS

disorder progressing through confusion, lethargy and delirium followed
by seizures and coma.

• Prolonged hypoglycemia may lead to irreversible brain damage.

• In newborns, hypoglycemia is the most common
metabolic disorder, and there are many reports describing
hypoglycemia-induced brain injury in this phase of life.

• Neonatal hypoglycemia can result in long-term disabilities

like epilepsy and visual impairment.

• The injuries include the parietal region frequently, but

mainly the occipital region. The explanation for this
selective vulnerability of occipital lobes in neonates is a
regional deficit in expression and function of the glucose
membrane transporter proteins.
• The mechanisms that explain the neuronal damage during
hypoglycemic episodes involves cytotoxic oedema, shrinkage
of the extracellular space and failure of ionic pumps of the
cell membrane after energy depletion.

• Anderson et al. induced a hypoglycemic state in ten healthy

patients and studied, by the f-MRI that contrasted in
dependence of blood oxygen level, the signs changes in
visual cortex after passive visual stimulation.

• They observed that during moderate hypoglycemia, this

method showed a reduced activity in the considered cortical
area and they suggested also that in other regions of cortex,
the variations in signal on f-MRI could reveal the regional
vulnerability of the brain to hypoglycemia.
• The acute sequel of mild hypoglycemic
episodes includes decrements in motor skills,
visual acuity, auditory processing, mood, and
a variety of cognitive processes
Diabetes Care 31:2072-2076, 2008
DOI: 10.2337/dc08-1441
© 2008 by the American Diabetes
• Hypoglycemia reduces the blood-
oxygenation level dependent signal in
primary auditory and visual cortex: A
functional magnetic resonance imaging
study

• J Neurosci Res. 2007 Feb 15;85(3):575-82.