Original Article
Spectrum of neurological complications in HELLP syndrome Birinder S. Paul, Sunil K. Juneja1, Gunchan Paul2, Shweta Gupta1 Departments of Neurology, 1Obstetrics and Gynecology, 2Critical Care Division, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
Abstract
Address for correspondence: Dr. Gunchan Paul, Associate Intensivist Critical Care Division, Dayanand Medical College, Ludhiana ‑ 141 001, Punjab, India. E‑mail: [email protected]
Background: Hemolysis (H), elevated liver enzymes (EL), and low platelets (LP), HELLP syndrome is the extended spectrum of severe preeclampsia and is associated with high mortality. A large proportion of mortality can be attributed to catastrophic central nervous system events. Aims: The purpose of this study was to access the clinical manifestations, radiological abnormalities and outcome in patients of HELLP syndrome with neurological manifestations. Setting: Obstetric unit and neurology intensive critical unit (ICU) of an academic medical center. Study Design: Retrospective study. Subjects and Methods: Case records of all obstetrical patients who were admitted between January 2012 and December 2012 were screened and data was collected from those patients who were diagnosed with HELLP syndrome with neurological complications. It was entered into a structured performa and analyzed using percentages. Results: During the study period; 1,166 deliveries were conducted, 108 patients had pregnancy‑induced hypertension (PIH); and of the 12 patients with HELLP, eight (66%) patients had neurological complications. The presenting neurological features were seizures (four), focal neurological deficits (two), and encephalopathy (two). Of the eight patients, in six patients neuroimaging showed features of posterior reversible encephalopathy syndrome (PRES), three of them had associated hemorrhage, and two patients had isolated intracranial hemorrhage. All except two were discharged home. Conclusions: Neurological complications are not uncommon in patients with HELLP syndrome and a high index of suspicion is essential. Aggressive multidisciplinary approach is the key to reduce the morbidity and mortality. Key words: Eclampsia, intracranial hemorrhage, hemolysis (H), elevated liver enzymes (EL), and low platelets (LP) syndrome, neurological complications, posterior reversible encephalopathy syndrome
Received : 04‑03‑2013 Review completed : 24‑07‑2013 Accepted : 11‑10‑2013
Introduction
This syndrome is rare but can occur as an isolated condition in 0.2-0.6% of all pregnancies.[2] The reported mortality in HELLP syndrome varies from 1 to 25%, and is mainly attributed to disseminated intravascular coagulation (DIC), liver failure, adult respiratory distress syndrome, acute kidney injury, sepsis, stroke or cardiopulmonary arrest.[3] Concerning the neurological complications, there have been only a few case reports. This is an observational study of neurological manifestations in patients of HELLP syndrome. [1]
Hemolysis (H), elevated liver enzymes (EL), but low platelets (LP) define HELLP syndrome which is an extended spectrum of severe preeclampsia or eclampsia. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.121909
Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Subjects and Methods Study population consisted of 1,246 obstetrical patients 467
Paul, et al.: Neurological complications in HELLP
hospitalized between January and December 2012. Case records of these patients were reviewed. Definitions Complete HELLP syndrome was defined as microangiopathic hemolytic anemia in women with severe preeclampsia, serum lactate dehydrogenase (LDH) >600 IU/L, platelet count 70 IU/L. Partial HELLP was defined as the presence of only one or two of the features of LDH, AST or platelets.[4] Based on the severity, HELLP syndrome was categorized into three classes using the University of Mississippi criteria: [5] Class 1 with severe thrombocytopenia (platelets 600 IU/L); Class 2 required similar criteria except that thrombocytopenia was moderate (50,000-100,000/mL); and Class 3 included patients with mild thrombocytopenia (1-1.5 lac/mL), mild hepatic dysfunction (AST and/or ALT >40 IU/L) and hemolysis (LDH >600 IU/L). Pregnancy‑induced hypertension (PIH) was classically defined as the triad of hypertension (>140/90 mmHg), proteinuria, and edema after 20 weeks of gestation.
Posterior reversible encephalopathy syndrome (PRES) was defined as cerebral white matter edema seen as hypodensity on computed tomography (CT) or T2 weighted magnetic resonance imaging (MRI) showing hyperintensity at grey‑white matter junction with no diffusion restriction. Patients fulfilling the above criteria were included in the study and the following variables were entered in a structured proforma: i) Obstetrical features (age, parity, perinatal history, clinical presentation, laboratory findings, treatment details and complications) and associated comorbid conditions like hypertension, diabetes, and cigarette smoking; ii) neurological manifestations (headache, impairment of consciousness, seizures, focal neurological deficit and imaging abnormalities); iii) maternal outcome (discharge or death).
Results During the 2 year period; 1,166 deliveries were conducted in our institute, 108 patients had PIH and 12 had HELLP syndrome. The frequency of HELLP syndrome in the total cohort was 0.9%. Of the 12 patients with HELLP syndrome, eight (66%) patients had neurological complications. The mean maternal age was 30.5 years. Five patients (62.5%) were multigravida and three (37.5%) were primigravida. Table 1 gives the demographic profile, salient features of antenatal history,
Table 1: Demographic data, presenting symptoms, salient features of history, lab parameters, and hospital stay of eight cases of HELLP syndrome
Case Age (years) Gravidity Time of presentation Presenting complaint Hypertension after 20 weeks Proteinuria Pedal edema Platelets (/µL) Coagulopathy (INR) LDH (IU/L) SGOT (IU/L) SGPT (IU/L) PBF‑evidence of hemolysis Urea (mg/dL) Creatinine (mg/dL) Uric acid Other complications Platelet transfusion
I
II
III
IV
28 P2L2 20 hr postpartum Seizures
28 P3L3A2SB1 24 hr postpartum Left hemiparesis
27 P3L2SB1 4 hr postpartum Seizures
‑
+
+
27 G1P1 36 weeks gestation Right hemiparesis and global aphasia +
‑ ‑ 2,5000 1.1 2,633 2,645 1,555 +
? + 56,000 1.02 1,659 435 347
+ + 38,000 0.98 1,433 784 685 +
+ + 33,000 1.19 2,650 1,222 448 +
+ + 36,000 1.2 1,879 303 349 +
77 2 12.8 ARF, shock
134 2.8
123 2.4
85 2.02
ARF, shock
ARF, shock, poor GCS, ventilatory support
ARF, shock, ventilatory support Yes
47 1.98 7.6 Shock
Yes
V
VI
VII
VIII
24 P1SB1 36 hr postpartum Hypotension, hemperitonium
33 G2P1L1 36 weeks gestation Seizures
‑
+
? + 34,000 1.27 2,231 988 405
‑ ‑ 53,000 1.34 1,779 603 440 +
+ + 48,000 1.2 2,891 1,263 529 +
40 0.95 9.5
71 3.2 6.8 ARF, shock, early retinal detachment Yes
39 1.77 10 Ventilatory support
27 24 P1L1 G2P1SB1 6 hr 30 weeks postpartum gestation Hypotension, Seizures hemoperitonium, hematuria + +
Yes
Yes
? - Antenatal records not available in the case files, INR - International normalized ratio, LDH - Lactate dehydrogenase, SGOT - Serum glutamic‑oxaloacetic transaminase, SGPT - Serum glutamic‑pyruvic transaminase, PBF - Peripheral blood film, ARF - Acute renal failure, HELLP - Hemolysis (H), elevated enzymes (EL), and low platelets (LP)
468
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Paul, et al.: Neurological complications in HELLP
clinical and laboratory findings. Six (75%) patients had PIH and two patients (case II and VIII) had history of preeclampsia in previous pregnancies. Associated comorbidities were not observed in any. Six patients had complete HELLP, while the remaining two had partial HELLP, due to lack of peripheral blood film findings, although LDH levels were above 600 IU/L.
managed conservatively with antihypertensives, antiepileptics and antiedema measures along with supportive care. Of the eight patients, four patients had still births (case II had twin pregnancy with one intrauterine death).
Five (65%) patients developed neurological features in the postpartum period; while three patients (35%) presented in antepartum period. The presenting symptoms varied, the most common being generalized tonic‑clonic seizures (four, 50%), hemiplegia (two, 25%), and encephalopathy (two, 25%). However, all the eight patients developed seizures during their hospital course. The neurological symptoms, neuroimaging findings and outcomes are summarized in Table 2. Two (25%) patients had isolated intracranial hemorrhage [Figures 1 and 2], six (75%) patients had features of PRES [Figures 3a, 4, and 5a], and three of them were associated with hemorrhage as well [Figures 3b and 5b].
The acronym of HELLP was first coined by Wenstein in 1982 to describe the presence of hemolysis (H), elevated liver enzymes (EL), and low platelets (LP) in a woman considered of having preeclampsia or eclampsia. [6] Using the University of Mississippi criteria[5] all the patients fulfilled the three characteristic features of this syndrome with six in Class I and two patients in Class II.
The clinical course of six patients was complicated by shock, five patients had acute kidney injury and three required mechanical ventilation. Platelet transfusions were given to five patients (case V and VII during laparotomy for hemoperitoneum, case II during parietal craniotomy for evacuation of intracranial hemorrhage (ICH), case IV and VIII for caesarean section). Maternal outcome was good (75%) in all except two patients (case III and VIII). All except case II were
Discussion
The mean maternal age in this series was 30.5 years comparable to the age reported in the series by Isler and colleagues.[7] The mean age of women with pregnancy related ICH was younger than women with ICH not related to pregnancy (28 vs 38 years).[8] Kittner, et al., also reported relative risk of puerperal ICH to be 28.3 compared to 2.5 during antepartum period.[9] In our series of the five patients with ICH, three patients presented in the postpartum period suggesting that postpartum is a high risk period for developing neurological complications, more so in multigravida with HELLP syndrome. Another study reported a high incidence in the postpartum period, while the study from Taiwan in Chinese women reported a higher incidence (58%) of pregnancy‑related ICH in prepartum period.[10,11]
Table 2: Neurological symptoms, radiological findings, and outcome of eight cases of HELLP syndrome
Case
I
II
III
IV
V
VI
VII
VIII
Altered sensorium Headache Seizures
+
+
+
+
+
+
+
+
Neurological deficit CT/MRI findings
+ +
+ + Day 1 Neck rigidity Left hemiparesis Bilateral Intracranial diffuse SAH hemorrhage involving the with mass interhemispheric effect fissure, sylvian (Figure 2) fissure. cortical sulci (Figure 1)
Angiography/ Normal venography Intervention
Not done
Maternal outcome
Discharge
Discharge
+ ‑
+ + Day 2 Right hemiparesis and global aphasia PRES with basal ganglion bleed
Day 1‑changes suggestive of PRES in praietooccipital regions and brainstem (Figure 3a) Day 4‑intracranial hemorrhage in right parietooccipital region (Figure 3b) Not done Normal
Evacuation of ICH Death
+ Day 3 ‑ Bilateral parieto occipital PRES
Not done
Termination of pregnancy
Exploratory laparotomy
Discharge
Discharge
+ +
+ Day 5 ‑ Blurring of vision Bilateral Bilateral parieto parietal, occipital frontal PRES occipital cortical and subcortical changes suggestive of PRES (Figure 4) Not done Not done Exploratory laparotomy Discharge Discharge
+ + ‑ Hemorrhage in the brainstem with PRES (Figure 5a and b)
Not done Termination of pregnancy by LSCS Death
CT - Computed tomography, MRI - Magnetic resonance imaging, SAH - Subarachnoid hemorrhage, ICH - Intracranial hemorrhage, LSCS - Lower segment cesarean section, PRES – Posterior reversible encephalopathy syndrome, HELLP – Hemolysis (H), elevated enzymes (EL), and low platelets (LP)
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Paul, et al.: Neurological complications in HELLP
Figure 1: Noncontrast computed tomography (CT) of case I showing hyperdensity in the interhemispheric fissure and bilateral cortical sulci suggestive of diffuse subarachnoid hemorrhage
a
b
Figure 3: (a) Magnetic resonance imaging fluid‑attenuated inversion recovery (MRI FLAIR) image of case III demonstrates hyperintense signal in bilateral parietooccipital lobes also extending to the brain stem, suggestive of posterior reversible encephalopathy syndrome (PRES). (b) Four days later repeat MRI was done due to deterioration in sensorium. FLAIR images demonstrated hemorrhage in the right parietooccipital area
a
b Figure 5: (a) MR image (T2 weighted) of case VIII shows bilateral hyperintense signal in parietooccipital area suggestive of PRES. (b) MRI (Gradient Echo) shows hemorrhage in the brainstem
Majority reports of PRES in the obstetrics and gynecology literature are a complication of severe preeclampsia/ eclampsia. We could find only two case reports of PRES in patients with HELLP.[12] 470
Figure 2: Noncontrast CT of case II shows a large intracerebral hemorrhage in the right parietal lobe, with mass effect
Figure 4: MRI FLAIR image of case VII demonstrates vasogenic edema suggestive of PRES in the occipital, parietal, and frontal lobes; also involving the thalamus on both sides
PRES is a transient clinicoradiological syndrome, first noted in patients with hypertensive encephalopathy.[13] The exact pathogenesis of PRES remains incompletely understood and the most likely mechanism is vasogenic edema secondary to an acute increase in arterial blood pressure, which overwhelms the autoregulatory capacity of the cerebral vasculature causing arteriolar vasodilatation and endothelial dysfunction. [14,15] This compromised capacity of autoregulation of the cerebral vasculature has been postulated as the mechanism of ICH in eclampsia and the increased concentrations of oxyhemoglobin derived from hemolysis in patients with HELLP might exaggerate this vascular response. [16,17] Also, thrombocytopenia in the setting of HELLP could contribute to increased risk of hemorrhage in these patients. As the name suggests, there is predilection of vasogenic edema in PRES for the posterior white matter, because the anterior cerebral circulation has higher sympathetic innervations than the Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Paul, et al.: Neurological complications in HELLP
vertebrobasilar system, which is protective against the damaging hypertension.[18] However, edema may not be limited to the posterior circulation and changes may be widespread involving the brainstem also, as in our cases V and VII [Figures 3a and 4]. Although complete reversibility of clinical and radiological features is the defining feature of PRES; however, ischemic injury and irreversible damage can occur. Hefzy et al., [19] studied the frequency of hemorrhage in PRES among various clinical conditions and reported an incidence of 5% in patients with eclampsia/delayed eclampsia. In our study one patient had initial imaging that was negative, while the other two had widespread changes of PRES along with hemorrhage.
2.
Two‑third of our patients were discharged with no neurological deficit and follow‑up MRI showed complete resolution of previous changes, consistent with the diagnosis of PRES. The mainstay of treatment is recognition and removal of the precipitating factor and supportive care. Our patients were aggressively managed with anticonvulsants, antiedema measures, antihypertensives and termination of pregnancy (case IV and VI). Previous studies suggest a significantly higher incidence of postpartum hemorrhagic complications in patients with platelets count
Spectrum of neurological complications in HELLP syndrome Birinder S. Paul, Sunil K. Juneja1, Gunchan Paul2, Shweta Gupta1 Departments of Neurology, 1Obstetrics and Gynecology, 2Critical Care Division, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
Abstract
Address for correspondence: Dr. Gunchan Paul, Associate Intensivist Critical Care Division, Dayanand Medical College, Ludhiana ‑ 141 001, Punjab, India. E‑mail: [email protected]
Background: Hemolysis (H), elevated liver enzymes (EL), and low platelets (LP), HELLP syndrome is the extended spectrum of severe preeclampsia and is associated with high mortality. A large proportion of mortality can be attributed to catastrophic central nervous system events. Aims: The purpose of this study was to access the clinical manifestations, radiological abnormalities and outcome in patients of HELLP syndrome with neurological manifestations. Setting: Obstetric unit and neurology intensive critical unit (ICU) of an academic medical center. Study Design: Retrospective study. Subjects and Methods: Case records of all obstetrical patients who were admitted between January 2012 and December 2012 were screened and data was collected from those patients who were diagnosed with HELLP syndrome with neurological complications. It was entered into a structured performa and analyzed using percentages. Results: During the study period; 1,166 deliveries were conducted, 108 patients had pregnancy‑induced hypertension (PIH); and of the 12 patients with HELLP, eight (66%) patients had neurological complications. The presenting neurological features were seizures (four), focal neurological deficits (two), and encephalopathy (two). Of the eight patients, in six patients neuroimaging showed features of posterior reversible encephalopathy syndrome (PRES), three of them had associated hemorrhage, and two patients had isolated intracranial hemorrhage. All except two were discharged home. Conclusions: Neurological complications are not uncommon in patients with HELLP syndrome and a high index of suspicion is essential. Aggressive multidisciplinary approach is the key to reduce the morbidity and mortality. Key words: Eclampsia, intracranial hemorrhage, hemolysis (H), elevated liver enzymes (EL), and low platelets (LP) syndrome, neurological complications, posterior reversible encephalopathy syndrome
Received : 04‑03‑2013 Review completed : 24‑07‑2013 Accepted : 11‑10‑2013
Introduction
This syndrome is rare but can occur as an isolated condition in 0.2-0.6% of all pregnancies.[2] The reported mortality in HELLP syndrome varies from 1 to 25%, and is mainly attributed to disseminated intravascular coagulation (DIC), liver failure, adult respiratory distress syndrome, acute kidney injury, sepsis, stroke or cardiopulmonary arrest.[3] Concerning the neurological complications, there have been only a few case reports. This is an observational study of neurological manifestations in patients of HELLP syndrome. [1]
Hemolysis (H), elevated liver enzymes (EL), but low platelets (LP) define HELLP syndrome which is an extended spectrum of severe preeclampsia or eclampsia. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.121909
Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Subjects and Methods Study population consisted of 1,246 obstetrical patients 467
Paul, et al.: Neurological complications in HELLP
hospitalized between January and December 2012. Case records of these patients were reviewed. Definitions Complete HELLP syndrome was defined as microangiopathic hemolytic anemia in women with severe preeclampsia, serum lactate dehydrogenase (LDH) >600 IU/L, platelet count 70 IU/L. Partial HELLP was defined as the presence of only one or two of the features of LDH, AST or platelets.[4] Based on the severity, HELLP syndrome was categorized into three classes using the University of Mississippi criteria: [5] Class 1 with severe thrombocytopenia (platelets 600 IU/L); Class 2 required similar criteria except that thrombocytopenia was moderate (50,000-100,000/mL); and Class 3 included patients with mild thrombocytopenia (1-1.5 lac/mL), mild hepatic dysfunction (AST and/or ALT >40 IU/L) and hemolysis (LDH >600 IU/L). Pregnancy‑induced hypertension (PIH) was classically defined as the triad of hypertension (>140/90 mmHg), proteinuria, and edema after 20 weeks of gestation.
Posterior reversible encephalopathy syndrome (PRES) was defined as cerebral white matter edema seen as hypodensity on computed tomography (CT) or T2 weighted magnetic resonance imaging (MRI) showing hyperintensity at grey‑white matter junction with no diffusion restriction. Patients fulfilling the above criteria were included in the study and the following variables were entered in a structured proforma: i) Obstetrical features (age, parity, perinatal history, clinical presentation, laboratory findings, treatment details and complications) and associated comorbid conditions like hypertension, diabetes, and cigarette smoking; ii) neurological manifestations (headache, impairment of consciousness, seizures, focal neurological deficit and imaging abnormalities); iii) maternal outcome (discharge or death).
Results During the 2 year period; 1,166 deliveries were conducted in our institute, 108 patients had PIH and 12 had HELLP syndrome. The frequency of HELLP syndrome in the total cohort was 0.9%. Of the 12 patients with HELLP syndrome, eight (66%) patients had neurological complications. The mean maternal age was 30.5 years. Five patients (62.5%) were multigravida and three (37.5%) were primigravida. Table 1 gives the demographic profile, salient features of antenatal history,
Table 1: Demographic data, presenting symptoms, salient features of history, lab parameters, and hospital stay of eight cases of HELLP syndrome
Case Age (years) Gravidity Time of presentation Presenting complaint Hypertension after 20 weeks Proteinuria Pedal edema Platelets (/µL) Coagulopathy (INR) LDH (IU/L) SGOT (IU/L) SGPT (IU/L) PBF‑evidence of hemolysis Urea (mg/dL) Creatinine (mg/dL) Uric acid Other complications Platelet transfusion
I
II
III
IV
28 P2L2 20 hr postpartum Seizures
28 P3L3A2SB1 24 hr postpartum Left hemiparesis
27 P3L2SB1 4 hr postpartum Seizures
‑
+
+
27 G1P1 36 weeks gestation Right hemiparesis and global aphasia +
‑ ‑ 2,5000 1.1 2,633 2,645 1,555 +
? + 56,000 1.02 1,659 435 347
+ + 38,000 0.98 1,433 784 685 +
+ + 33,000 1.19 2,650 1,222 448 +
+ + 36,000 1.2 1,879 303 349 +
77 2 12.8 ARF, shock
134 2.8
123 2.4
85 2.02
ARF, shock
ARF, shock, poor GCS, ventilatory support
ARF, shock, ventilatory support Yes
47 1.98 7.6 Shock
Yes
V
VI
VII
VIII
24 P1SB1 36 hr postpartum Hypotension, hemperitonium
33 G2P1L1 36 weeks gestation Seizures
‑
+
? + 34,000 1.27 2,231 988 405
‑ ‑ 53,000 1.34 1,779 603 440 +
+ + 48,000 1.2 2,891 1,263 529 +
40 0.95 9.5
71 3.2 6.8 ARF, shock, early retinal detachment Yes
39 1.77 10 Ventilatory support
27 24 P1L1 G2P1SB1 6 hr 30 weeks postpartum gestation Hypotension, Seizures hemoperitonium, hematuria + +
Yes
Yes
? - Antenatal records not available in the case files, INR - International normalized ratio, LDH - Lactate dehydrogenase, SGOT - Serum glutamic‑oxaloacetic transaminase, SGPT - Serum glutamic‑pyruvic transaminase, PBF - Peripheral blood film, ARF - Acute renal failure, HELLP - Hemolysis (H), elevated enzymes (EL), and low platelets (LP)
468
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Paul, et al.: Neurological complications in HELLP
clinical and laboratory findings. Six (75%) patients had PIH and two patients (case II and VIII) had history of preeclampsia in previous pregnancies. Associated comorbidities were not observed in any. Six patients had complete HELLP, while the remaining two had partial HELLP, due to lack of peripheral blood film findings, although LDH levels were above 600 IU/L.
managed conservatively with antihypertensives, antiepileptics and antiedema measures along with supportive care. Of the eight patients, four patients had still births (case II had twin pregnancy with one intrauterine death).
Five (65%) patients developed neurological features in the postpartum period; while three patients (35%) presented in antepartum period. The presenting symptoms varied, the most common being generalized tonic‑clonic seizures (four, 50%), hemiplegia (two, 25%), and encephalopathy (two, 25%). However, all the eight patients developed seizures during their hospital course. The neurological symptoms, neuroimaging findings and outcomes are summarized in Table 2. Two (25%) patients had isolated intracranial hemorrhage [Figures 1 and 2], six (75%) patients had features of PRES [Figures 3a, 4, and 5a], and three of them were associated with hemorrhage as well [Figures 3b and 5b].
The acronym of HELLP was first coined by Wenstein in 1982 to describe the presence of hemolysis (H), elevated liver enzymes (EL), and low platelets (LP) in a woman considered of having preeclampsia or eclampsia. [6] Using the University of Mississippi criteria[5] all the patients fulfilled the three characteristic features of this syndrome with six in Class I and two patients in Class II.
The clinical course of six patients was complicated by shock, five patients had acute kidney injury and three required mechanical ventilation. Platelet transfusions were given to five patients (case V and VII during laparotomy for hemoperitoneum, case II during parietal craniotomy for evacuation of intracranial hemorrhage (ICH), case IV and VIII for caesarean section). Maternal outcome was good (75%) in all except two patients (case III and VIII). All except case II were
Discussion
The mean maternal age in this series was 30.5 years comparable to the age reported in the series by Isler and colleagues.[7] The mean age of women with pregnancy related ICH was younger than women with ICH not related to pregnancy (28 vs 38 years).[8] Kittner, et al., also reported relative risk of puerperal ICH to be 28.3 compared to 2.5 during antepartum period.[9] In our series of the five patients with ICH, three patients presented in the postpartum period suggesting that postpartum is a high risk period for developing neurological complications, more so in multigravida with HELLP syndrome. Another study reported a high incidence in the postpartum period, while the study from Taiwan in Chinese women reported a higher incidence (58%) of pregnancy‑related ICH in prepartum period.[10,11]
Table 2: Neurological symptoms, radiological findings, and outcome of eight cases of HELLP syndrome
Case
I
II
III
IV
V
VI
VII
VIII
Altered sensorium Headache Seizures
+
+
+
+
+
+
+
+
Neurological deficit CT/MRI findings
+ +
+ + Day 1 Neck rigidity Left hemiparesis Bilateral Intracranial diffuse SAH hemorrhage involving the with mass interhemispheric effect fissure, sylvian (Figure 2) fissure. cortical sulci (Figure 1)
Angiography/ Normal venography Intervention
Not done
Maternal outcome
Discharge
Discharge
+ ‑
+ + Day 2 Right hemiparesis and global aphasia PRES with basal ganglion bleed
Day 1‑changes suggestive of PRES in praietooccipital regions and brainstem (Figure 3a) Day 4‑intracranial hemorrhage in right parietooccipital region (Figure 3b) Not done Normal
Evacuation of ICH Death
+ Day 3 ‑ Bilateral parieto occipital PRES
Not done
Termination of pregnancy
Exploratory laparotomy
Discharge
Discharge
+ +
+ Day 5 ‑ Blurring of vision Bilateral Bilateral parieto parietal, occipital frontal PRES occipital cortical and subcortical changes suggestive of PRES (Figure 4) Not done Not done Exploratory laparotomy Discharge Discharge
+ + ‑ Hemorrhage in the brainstem with PRES (Figure 5a and b)
Not done Termination of pregnancy by LSCS Death
CT - Computed tomography, MRI - Magnetic resonance imaging, SAH - Subarachnoid hemorrhage, ICH - Intracranial hemorrhage, LSCS - Lower segment cesarean section, PRES – Posterior reversible encephalopathy syndrome, HELLP – Hemolysis (H), elevated enzymes (EL), and low platelets (LP)
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Paul, et al.: Neurological complications in HELLP
Figure 1: Noncontrast computed tomography (CT) of case I showing hyperdensity in the interhemispheric fissure and bilateral cortical sulci suggestive of diffuse subarachnoid hemorrhage
a
b
Figure 3: (a) Magnetic resonance imaging fluid‑attenuated inversion recovery (MRI FLAIR) image of case III demonstrates hyperintense signal in bilateral parietooccipital lobes also extending to the brain stem, suggestive of posterior reversible encephalopathy syndrome (PRES). (b) Four days later repeat MRI was done due to deterioration in sensorium. FLAIR images demonstrated hemorrhage in the right parietooccipital area
a
b Figure 5: (a) MR image (T2 weighted) of case VIII shows bilateral hyperintense signal in parietooccipital area suggestive of PRES. (b) MRI (Gradient Echo) shows hemorrhage in the brainstem
Majority reports of PRES in the obstetrics and gynecology literature are a complication of severe preeclampsia/ eclampsia. We could find only two case reports of PRES in patients with HELLP.[12] 470
Figure 2: Noncontrast CT of case II shows a large intracerebral hemorrhage in the right parietal lobe, with mass effect
Figure 4: MRI FLAIR image of case VII demonstrates vasogenic edema suggestive of PRES in the occipital, parietal, and frontal lobes; also involving the thalamus on both sides
PRES is a transient clinicoradiological syndrome, first noted in patients with hypertensive encephalopathy.[13] The exact pathogenesis of PRES remains incompletely understood and the most likely mechanism is vasogenic edema secondary to an acute increase in arterial blood pressure, which overwhelms the autoregulatory capacity of the cerebral vasculature causing arteriolar vasodilatation and endothelial dysfunction. [14,15] This compromised capacity of autoregulation of the cerebral vasculature has been postulated as the mechanism of ICH in eclampsia and the increased concentrations of oxyhemoglobin derived from hemolysis in patients with HELLP might exaggerate this vascular response. [16,17] Also, thrombocytopenia in the setting of HELLP could contribute to increased risk of hemorrhage in these patients. As the name suggests, there is predilection of vasogenic edema in PRES for the posterior white matter, because the anterior cerebral circulation has higher sympathetic innervations than the Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Paul, et al.: Neurological complications in HELLP
vertebrobasilar system, which is protective against the damaging hypertension.[18] However, edema may not be limited to the posterior circulation and changes may be widespread involving the brainstem also, as in our cases V and VII [Figures 3a and 4]. Although complete reversibility of clinical and radiological features is the defining feature of PRES; however, ischemic injury and irreversible damage can occur. Hefzy et al., [19] studied the frequency of hemorrhage in PRES among various clinical conditions and reported an incidence of 5% in patients with eclampsia/delayed eclampsia. In our study one patient had initial imaging that was negative, while the other two had widespread changes of PRES along with hemorrhage.
2.
Two‑third of our patients were discharged with no neurological deficit and follow‑up MRI showed complete resolution of previous changes, consistent with the diagnosis of PRES. The mainstay of treatment is recognition and removal of the precipitating factor and supportive care. Our patients were aggressively managed with anticonvulsants, antiedema measures, antihypertensives and termination of pregnancy (case IV and VI). Previous studies suggest a significantly higher incidence of postpartum hemorrhagic complications in patients with platelets count
![[Development of symptoms and perinatal complications in HELLP syndrome].](/assets/img/hold.png)
