Journal of Obstetrics and Gynaecology

ISSN: 0144-3615 (Print) 1364-6893 (Online) Journal homepage: http://www.tandfonline.com/loi/ijog20

Hemiparesis as an unusual presentation of
haemolysis, elevated liver enzymes, low platelet
count (HELLP) syndrome
D Elsandabesee, R Hamzeh & A Pozyczka
To cite this article: D Elsandabesee, R Hamzeh & A Pozyczka (2004) Hemiparesis as an unusual
presentation of haemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome,
Journal of Obstetrics and Gynaecology, 24:8, 926-927
To link to this article: http://dx.doi.org/10.1080/01443610400019179

Published online: 02 Jul 2009.

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926

Obstetric case reports

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Theoretically, the enhanced thrombogenesis can lead to a
hypercoagulable state that could potentially worsen DIC; however,
studies in animals suggest no increase in risk of DIC (Bregengaard
et al., 1993). Thrombotic events such as myocardial infarction,
cerebrovascular ischaemia, deep vein thrombosis and pulmonary
embolism have been reported, but appear to be related to risk
factors for these conditions and the severity of the condition for
which NovoSeven is used other than to use of NovoSeven or dosage
(Roberts, 1998). The major drawback is that the product is
expensive.
Alternative treatments such as hysterectomy lead to loss of
fertility, an issue for many woman. Uterine artery embolisation
(Drife, 1997) had also been used in limited cases but is not available
in many units and will probably never be used outside specialist
centres. In addition, it is an invasive procedure with potential risks
of infection, vascular injury and radiation exposure.
In summary, we have demonstrated a new therapy for the
treatment of postpartum bleeding in association with a traumatised
placental bed. It provides an easy therapy to add to the therapeutic
options for this difficult problem.

References
Bregengaard C., Diness V. and Hedner U. (1993) Effect of recombinant
factor VIIa on endotoxin-induced disseminated intravasular coagulation in rabbits. Thrombosis and Haemostasis, 69, 749.
Drife J. (1997) Mangement of primary postpartum haemorrage. British
Journal of Obstetrics and Gynaecology, 104, 275 – 277.
O’Connell N.M., Perry D.J., Hodgson A.J., et al. (2003) Recombination
FVIIa in the management of uncontrolled haemorrage. Transfusion,
43, 1711 – 1716.
Roberts H.R. (1998) Clinical experience with activated factor VII: focus
on safety aspects. Blood Coagulation and Fibrinolysis, 9(Supplement),
S115 – S118.
Segal S., Shemesh I.Y., Blumenthal R., et al. (2003) Treatment of
obstetric haemorrhage with recombinant activated factor VII
(rFVIIa). Archives of Gynecology and Obstetrics, 268, 266 – 267.
Vlot A.J., Ton E., Mackaay A.J.C., Kramer M.H.H. and Gaillard
C.A.J.M. (2000) Treatment of a severely bleeding patient without
pre-existing coagulopathy with activated factor VII. American
Journal of Medicine, 108, 421 – 422.
White B., McHale J., Ravi N. et al. (1999) Successful use of
recombinant FVIIa (NovoSeven) in the management of intractable
post-surgical intra-abdominal haemorrage. British Journal of Haematology, 107, 677 – 682.

Correspondence to: Dr Muchabayiwa Gidiri, Department of Obstetrics and Gynaecology, Hull and East Yorkshire Women and Children’s Hospital,
Anlaby Road, Hull HU3 2JZ, UK. Tel: 01482 382769; Fax: 01482 382781; E-mail: fmgidiri@doctors.org.uk
DOI: 10.1080/01443610400019120

Hemiparesis as an unusual presentation of
haemolysis, elevated liver enzymes, low platelet
count (HELLP) syndrome
D. ELSANDABESEE, R. HAMZEH and A. POZYCZKA
Department of Obstetrics and Gynaecology, James Paget Hospital, Norfolk, UK

Case report
A 35-year-old woman in her second pregnancy had a normal
delivery followed by manual removal of the placenta. The
pregnancy was uneventful and there was no medical or surgical
history of relevance. Five hours after delivery, the woman
complained of headache and muscle weakness on the left side
followed by three episodes of tonic and clonic seizures. The blood
pressure was 124/74 mmHg and remained normal. There was no
proteinuria. Flaccid paralysis was demonstrated on the left side
with extensor plantar response. An average score of 14 was given on
the Glasgow Coma Scale. The provisional diagnosis was that of
cerebrovascular stroke; however, the woman was treated as a case
of eclampsia until the diagnosis of stroke could be confirmed.
Initial blood results revealed marked rise in liver enzymes,
bilirubin and uric acid (alanine transaminase 550 IU/l, alkaline
phosphatase 314 IU/l, bilirubin 45 umol/l, urate 0.6 umol/l).
Platelet count was found to drop progressively to reach 66 6 109/l
(220 6 109 prior to manual removal of placenta). Coagulation
screen was normal, apart from raised D-dimer (8174 ng/ml).
Peripheral blood film showed no evidence of haemolysis. The
diagnosis of haemolysis, elevated liver enzymes, low platelet count
(HELLP) or more strictly ELLP syndrome was made. CT scan
revealed three areas of intracerebral haemorrhage (ICH) involving
the right frontal, temporal and parietal lobes with midline shift.
The patient was transferred to a tertiary referral Neuroscience
Critical Care Unit for further management. Conservative management with physiotherapy as well as haematological and neurological monitoring was adopted. MRI excluded lateral and sagital sinus
thrombosis. Platelet count and liver function tests improved
gradually and the woman was transferred back to our hospital to
be managed with physiotherapy for left hemiparesis. She showed

progressive improvement and was discharged 23 days after the
incident.

Discussion
The importance of not missing the diagnosis of HELLP syndrome
lies in the potentially disastrous consequences of the condition.
Right upper quadrant pain is the presenting symptom in 86 – 92%
of cases. The majority of patients (90%) will give a history of
malaise while 45 – 86% complain of nausea and/or vomiting (Rath
et al., 2000).
A striking feature in this case report is the unusual presentation
as hemiparesis. ICH is a recognised complication and has been
identified as the most common cause of death in patients with
HELLP syndrome (Hashiguchi et al., 2001). We claim, however,
that no cases published in the literature presented with hemiparesis.
We conducted a MEDLINE search for the English-language
articles dealing with HELLP syndrome and intracranial haemorrhage or stroke published from 1983 to 2003. None of the three
cases reports identified (Gliemroth et al., 2000; Hashiguchi et al.,
2001; Ezri et al., 2002) presented in this way.
Another unusual feature is the occurrence of ICH in the presence
of normal blood pressure. Although up to 20% of patients present
no hypertension (Rath et al., 2000), there have been no reports of
ICH in normotensive patients with HELLP. Using strict diagnostic
criteria, this case would be considered partial, as opposed to
complete HELLP syndrome. It should be borne in mind, however,
that haemolysis is the most difficult feature to capture in a
laboratory process (Rath et al., 2000). Although a higher incidence
of maternal complications has been highlighted with complete
HELLP syndrome, this case report demonstrates that there is no

Obstetric case reports
place for complacence in the management of partial HELLP
syndrome.

References
Ezri T., Abouleish E., Lee C. and Evron S (2002) Intracranial subdural
hematoma following dural puncture in a parturient with HELLP
syndrome. Canadian Journal of Anaesthesia, 49, 820 – 823.

927

Gliemroth J., Knopp U., Kehler U. et al., (2000) HELLP syndrome
with haemoglobin vasospasm. Journal of Clinical Neuroscience, 7,
59 – 62.
Hashiguchi K., Inamura T., Irita K. et al. (2001) Late occurrence of
diffuse cerebral swelling after intracerebral hemorrhage in a patient
with HELLP syndrome, case report. Neurologica Medico-Chirurgica,
41, 144 – 148.
Rath W., Faridi A. and Dudenhausen J.W. (2000) HELLP syndrome.
Journal of Perinatal Medicine, 28, 249 – 260.

Correspondence to: D. Elsandabesee, 31 Jenkins Green, Lowestoft, NR32 4WX, UK. E-mail: deyamonir@hotmail.com

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DOI: 10.1080/01443610400019179

Methylmalonic acidaemia: a rare metabolic disorder
in pregnancy
O. A. ADEYEMI1, T. GIRISH,1 S. MUKHOPADHYAY1, S. A. OLCZAK2 and Z. AHMED3
Departments of 1Obstetrics, 2Medicine and Paediatrics, 3Pilgrim Hospital, Boston, Lincolnshire, UK

Case report
A 23-year-old primigravid woman was booked for antenatal care at
9 weeks’ gestation in a district general hospital. As a neonate she
had been diagnosed to be suffering from a rare metabolic disorder:
methylmalonic acidaemia due to deficiency of the methylmalony
CoA mutase enzyme (a vitamin B12-dependent form of the disease).
She developed epilepsy at the age of 7 years, possibly secondary to
MMA (methylmalonic acidaemia)-related central nervous system
effects in early childhood. Convulsions, which had the features of
both absence and generalised seizures, were controlled with
carbamazepine.
She was on a high-dose combined pill for contraception as she
was on antiepileptic drugs, but missed a pill and became pregnant.
Her diet was unremarkable. She smoked 10 – 15 cigarettes a day.
She had one sister who does notg have MMA. She had not suffered
any metabolic decompensation for many years.
She weighed 60.3 kg with a body mass index of 23. Her general
and systemic examation was unremarkable. She was booked for
antenatal care at 9 weeks’ gestation. Booking bloods, dating and
anomaly scans were normal. Downs and neural tube defect
screening tests were normal. The partner’s carrier status was
unknown. The patient was advised that the neonate would be tested
for methylmalonic acidaemia by blood spots and urine examination.
She was seen in combined obstetric/medical clinic on a
fortnightly basis. Specialist advice was sought from a consultant
in adult inborn errors of metabolism at a tertiary centre. Dietary
protein was restricted with adequate carbohydrate supplementation. Oral cyanocobalamin therapy 500 mg twice a day was
continued throughout pregnancy. Oral L-carnitine was supplemented at 1 mmol/10 ml per day. Because essential fatty acid deficiency
is common in MMA, she was prescribed Maxepa capsules. Seizure
control was achieved with carbamazepine 800 mg twice a day, the
dose was being adjusted in relation to serum levels. Steroid inhalers
were continued for asthma.
Full blood count, urea and electrolytes and liver function tests
were within the normal range throughout pregnancy. The MMA/
creatinine ratio was raised throughout pregnancy. Plasma total and
free carnitine were decreased, suggesting relative carnitine deficiency. Propionyl carnitine/palmitoyl carnitine ratio was 5.8.
She presented at 20 weeks’ gestation with vaginal discharge and a
high vaginal swab did not reveal any pathological organisms. At 25
weeks’ gestation she was admitted with abdominal pain. An

endocervical swab confirmed a chlamydial infection, which was
treated adequately with clarithromycin. She was followed-up in the
genitourinary clinic. She was admitted at 27 weeks’ gestation with
vomiting and backache, which settled with intravenous fluids and
anti-emetics. At 32 weeks’ gestation she was admitted with preterm
prelabour rupture of membranes and was managed conservatively.
Dexamethasone was administered, and regular temperature
monitoring, twice-weekly full blood count and daily cardiotocogram were carried out. At 34 weeks’ gestation, induction of labour
was commenced in view of suspected chorioamnionitis on clinical
grounds, although the white cell count was within normal range.
She underwent emergency caesarean section for failure to progress
in labour and delivered a live female baby weighing 1.9 kg with an
apgar score of 9 and 10 at 1 and 5 minutes, respectively. Umbilical
cord pH and base excess were within normal range. The baby was
admitted to neonatal unit for observation. The baby’s urine showed
no evidence of methylmalonic acid excretion. The baby was
discharged home with multivitamins iron and folic acid. The
patient had an uneventful postpartum recovery.
At 6 weeks’ follow up the baby was making appropriate
developmental progress. At 5 months’ follow up the baby weighed
6.15 kg, which was around the 50th centile for her corrected age.
Her examination was unremarkable with a satisfactory developmental progress.

Discussion
Methylmalonic acidaemia is a rare genetic metabolic autosomal
recessive disorder. Methylmalonic acid is derived from propionic
acid as a part of the catabolic pathway of isoleucine, valine,
threonine and methionine. A racemase enzyme converts the Disomer of MMA to L-isomer and the latter is converted to succinic
acid by a mutase, which required adenosyl cobalamin as the coenzyme. Our patient had a deficiency of MMA CoA mutase, which
is the most common variant of the disease.
Accumulation of methylmalonic acid in the body leads to
metabolic acidosis, ketosis, hyperammonaemia and relative carnitine deficiency (as it is excreted as methyl carnitine). Neurological
manifestations include seizures, encephalopathy and stroke (Behrman et al., 1996).
Rational treatment of this condition is by a low-protein diet that
is deficient in MMA precursors. Large doses of vitamin B12 are
needed in the B12-responsive form of the disease. Acidosis may need