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Table of Contents    
LETTERS TO EDITOR
Year : 2018  |  Volume : 66  |  Issue : 5  |  Page : 1496-1498

Peripartum seizures in Albright's osteodystrophy: Is it hypocalcemia or embolic stroke?


Department of General Medicine, Grant Medical College and Sir JJ hospital, Mumbai, Maharashtra, India

Date of Web Publication17-Sep-2018

Correspondence Address:
Dr. Vidya S Nagar
Department of General Medicine, Grant Medical College and Sir JJ hospital, Mumbai - - 400 008, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.241336

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How to cite this article:
Chatterjee R, Nagar VS, Kumbhare D, Sajjan B. Peripartum seizures in Albright's osteodystrophy: Is it hypocalcemia or embolic stroke?. Neurol India 2018;66:1496-8

How to cite this URL:
Chatterjee R, Nagar VS, Kumbhare D, Sajjan B. Peripartum seizures in Albright's osteodystrophy: Is it hypocalcemia or embolic stroke?. Neurol India [serial online] 2018 [cited 2018 Oct 23];66:1496-8. Available from: http://www.neurologyindia.com/text.asp?2018/66/5/1496/241336




Sir,

A 20-year old female patient was transferred to the emergency medicine unit after an episode of convulsion during labor in her first pregnancy, which was carried to term. She had had a single antenatal visit to a primary health center prior to this in the 8th month of her pregnancy, with no documentation of any raised blood pressure or any other comorbidity. On reviewing the obstetrician's notes, it was found that she had an uneventful stay in the labor ward with no evidence of elevated blood pressure and normal routine laboratory investigations and had suffered a first episode of generalized tonic clonic convulsion while bearing down toward the end of labor. This had been associated with a sudden drop in oxygen saturation with a drop in blood pressure to 80/60 mm of mercury from a baseline of 120/70 mm of mercury. The baby had been delivered successfully, after which she was rushed to the medical emergency. On evaluation, she was found to be stuporous with a Glasgow coma scale (GCS) of 8/15(E2V2M4). There was mild peripheral cyanosis. The cardiovascular evaluation was normal. Fluid resuscitation was started with isotonic saline at 20ml/kg/h after which her blood pressure stabilized to 110/70mm of Hg. Oxygen saturation also improved to 97% on oxygen by mask at 4 liters/min. On detailed evaluation after stabilization, she was found to have a short stature with short metacarpals on both hands and feet, with the classic knuckle-knuckle–dimple-dimple sign present in the hands [Figure 1]. On neurological evaluation, her best verbal response had improved to speaking inappropriate words. There were reduced movements of the right side of the body compared to the left. The tone was normal in all 4 limbs. There was hyperreflexia of all deep tendon reflexes in bilateral upper and lower limbs, along with upgoing plantars bilaterally. Routine laboratory investigations revealed normal blood sugar, complete blood count, urea and electrolytes, renal and liver functions, and a normal coagulation profile. Serum calcium was low, with the total level of 6.57 mg/dl, the ionized calcium level being 4.1 mg/dl, and the protein bound calcium level being 2.47 mg/dl. Serum phosphorus was elevated at 7.2 mg/dl. Calcium was corrected by intravenous infusion. A computed tomography of the brain was done in view of the presence of lateralizing signs on examination but did not reveal any significant abnormality [Figure 2]. An MRI of the brain was planned, which revealed areas of diffusion restriction in the bilateral frontal, parietal, occipital regions, centrum semiovale, splenium, some part of the isthmus of the corpus callosum and left cerebellar hemisphere that were hyperintense on T2 weighted images suggestive of acute infarcts [Figure 3]a-e]. The areas of diffusion restriction were spread all over the neuro-parenchyma including bilateral cerebral and cerebellar regions. Multiple vascular territories were involved. There was a characteristic starfield pattern suggestive of embolic shower as a possible etiology. The electrocardiogram was suggestive of sinus tachycardia. There was no evidence of calf swelling, and Homan's and Moses' signs were negative. A venous Doppler of bilateral lower limbs showed no evidence of thrombosis. The X-ray of the chest was normal, with no active infiltrates or focal oligemia to suggest pulmonary embolism. The patient was shifted to the medical intensive care unit. She had no further episodes of seizures. Her blood pressure and oxygen saturation stabilized. Her sensorium improved subsequently over the next 48 hours, with supportive management alone, to a GCS of 15/15. On further evaluation of the causes for her hypocalcemia, serum parathyroid hormone was raised at 103pg/ml. This coupled with the classic features on physical examination and X-rays of the hands and feet were suggestive of pseudo-hypoparathyroidism (Albright's hereditary osteodystropy). A two-dimensional echocardiogram to look for the source of emboli revealed no obvious structural heart defect. In view of a high clinical suspicion, echocardiography was reviewed after injecting agitated saline, which showed appearance of bubbles in the left atrium on two-dimensional echo during the Valsalva maneuver. This was suggestive of a latent patent foramen ovale or some arteriovenous shunting across the pulmonary vasculature. In view of the clinical picture of cardiorespiratory insufficiency during the active part of labor, with an embolic stroke and the presence of right-to-left shunting with no evidence of venous thrombosis, a possibility of amniotic fluid embolism paradoxically causing embolic stroke during labor was considered. The patient subsequently had a complete neurological recovery with no residual neurological deficit and was discharged on day 12th of admission.
Figure 1: (a) Archibaldæs sign in the left hand. Short third metacarpal causes knuckle--knuckle--dimple--dimple sign. (b and c) Short 3rd and 4th metacarpals. (d) Short 3rd and 4th metatarsals in both feet

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Figure 2: Normal CT scan of the brain after the seizure episode

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Figure 3: (a) T2 weighted images show multiple hyperintense lesions in bilateral cerebral hemispheres in a starfield pattern. (b) Same lesions show diffusion restriction on diffusion weighted images. (c) Embolic shower on coronal T2 weighted images. (d) Cerebellar infarct on diffusion weighted images. (e) Infarct in the splenium of the corpus callosum on sagittal T1 weighted images

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Woo et al., reported a case of amniotic fluid embolism causing an embolic stroke.[1] The authors described a similar case of peripartum cardio–respiratory arrest with a starfield pattern on the MRI of the brain and an incidentally discovered patent foramen ovale. They hypothesized that the reversal of shunt through this defect occurred during labor because of the increase in intrathoracic pressure during bearing down. They simulated this with a reversal of shunt on two-dimensional echocardiography while performing the Valsalva maneuver. The coexistence of pseudo-hypoparathyroidism and resultant hypocalcemia was a red herring in our case. The temporal evolution of events and typical imaging findings coupled with the history point toward amniotic fluid embolism to the brain as the cause of the initial seizure. The paradoxical shunting of emboli to the systemic circulation might even have spared a high load of emboli to the lungs and resulted in a less-severe cardio-respiratory arrest.

Amniotic fluid embolism is at present a purely clinical diagnosis. Currently, three sets of accepted criteria emanate from the United States, the United Kingdom, and Japan.[2] All these criteria agree on the presence of acute onset of cardio-respiratory arrest that occurs peripartum in the absence of comorbidities such as eclampsia or postpartum hemorrhage that the symptoms may be attributed to. The Japanese criteria consider any event within 12 h of active labor; the US criteria consider symptoms that initiate within 30 min of active labor.

One review places acute onset of neurological deficit in the form of loss of consciousness as the most common presentation of amniotic fluid embolism, that is reported in up to 66.7% of the cases.[3] The same authors describe severe residual neurological sequelae in 15% of the cases and minor neurological deficits in 25% of the cases.

Neurological outcomes of amniotic fluid embolism are not well defined in the existing literature. Isolated reports exist documenting the use of therapeutic hypothermia with a good resultant neurological outcome.[4] The existing recommendations for the management of amniotic fluid embolism emphasize upon the prompt initiation of advanced cardiac life support protocols and adequate oxygenation and hemodynamic support.[5]

The present data put the aggregate mortality of amniotic fluid embolism at 20.4%.[6] Neurological manifestations and outcomes are not well known. A comprehensive review of the same is required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Woo Y-S, Hong S-C, Park S-M, Cho K-H. Ischemic stroke related to an amniotic fluid embolism during labor. J Clin Neurosci 2015;22:767-8.  Back to cited text no. 1
    
2.
Kobayashi H, Akasaka J, Naruse K, Sado T, Tsunemi T, Niiro E, et al. Comparison of the different definition criteria for the diagnosis of amniotic fluid embolism. J Clin Diagn Res 2017;11:QC18-QC21.  Back to cited text no. 2
    
3.
Skolnik S, Ioscovich A, Eidelman LA, Davis A, Shmueli A, Aviram A, et al. Anesthetic management of amniotic fluid embolism - A multi-center, prospective, cohort study. J Matern Neonatal Med 2017;1-5. doi: 10.1080/14767058.2017.1404024.  Back to cited text no. 3
    
4.
Barriuso V, Pombar X, Bankowski HA. The use of therapeutic hypothermia in the management of amniotic fluid embolism. Obstet Med 2013;6:92-3.  Back to cited text no. 4
    
5.
Pacheco LD, Saade G, Hankins GDV, Clark SL. Amniotic fluid embolism: Diagnosis and management. Am J Obstet Gynecol 2016;215:B16-24.  Back to cited text no. 5
    
6.
Benson MD. Amniotic fluid embolism mortality rate. J Obstet Gynaecol Res 2017;43:1714-8.  Back to cited text no. 6
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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