Lanitha Srikugan, MCRP

Rapid onset visual loss with limb involvement in a postpartum female invites a number of differential diagnoses, including infectious encephalomyelitis, vasculitis, an acute demyelinating disorder, and arterial/venous sinus thrombosis. We report an interesting, but initially puzzling, case of rapidly progressive optic neuritis and transverse myelitis in a postpartum Ghanaian female, later diagnosed as neuromyelitis optica (NMO).

Case Report

A 33‐year‐old Ghanaian female presented with bilateral eye pain worsened by eye movement, rapid visual loss, bifrontal headache, and right leg weakness for 48 hours. She was 8 weeks postpartum, with labor having been induced at 35 weeks due to hypertension; she was discharged on labetolol and no other medications. There was no other relevant past medical or family history, or illicit drug use.

On admission, her blood pressure was 148/99 mm Hg, pulse rate 92 beats per minute, temperature 37.8C, oxygen saturation 97% on room air, respiratory rate 14 breaths per minute, and Glasgow coma scale 15/15. Cranial nerve examination showed absent light perception in the right eye with a relative afferent pupillary defect, 20/30 Snellen visual acuity in the left eye, bilateral optic disc edema (worse on the right) and grade 1 hypertensive retinopathy, and slight facial asymmetry but otherwise grossly intact cranial nerves. Neurological examination of the limbs showed 5/5 power in upper limbs, but flexion was weaker (3/5) than extension (4/5) in lower limbs. There was hyperreflexia in the right arm and right leg with positive Babinski sign. Sensation, proprioception, and coordination were normal in both upper and lower limbs; a Romberg test was negative.

Other systems examinations were unremarkable. Complete blood count, standard electrolytes, erythrocyte sedimentation rate, C‐reactive protein, urinalysis, chest x‐ray, and an urgent head computed tomography (CT) with contrast were all noncontributory.

To cover a broad range of possible infective processes, ceftriaxone and acyclovir were started. In view of the rapid progression of her presentation she was transferred urgently to a specialty neurology hospital. There she was started on intravenous methylprednisolone to cover possible inflammatory causes, such as multiple sclerosis (MS) and vasculitis. However, her lower limb weakness progressed and she developed urinary retention. Lumbar puncture was performed; the opening pressure was 160 mm H₂O. Cerebrospinal fluid (CSF) analysis demonstrated elevated protein level (121 mg/dL), 16/mm³ lymphocytosis, normal glucose, and negative gram stain and culture. Conventional CSF polymerase chain reaction for herpes simplex, Epstein‐Barr, varicella‐zoster, cytomegalovirus, John Cunningham virus (to rule out progressive multifocal leukoencephalopathy), human herpesvirus 6 viruses, and toxoplasma were negative. Antinuclear antibody, anticytoplasmic antibody, antiganglioside antibodies (GM1, GQ1b), thrombophilia screen (activated protein C resistance ratio, lupus anticoagulant and anticardiolipin antibodies, antithrombin III, protein C, and S deficiency), B12, folate, ferritin, and human immunodeficiency virus (HIV) screen were also negative. Brain magnetic resonance imaging (MRI), including venography, was normal. However, T2‐weighted MRI images of the orbit showed signal hyperintensity of the left optic chiasm and optic nerves, and T2‐weighted images of the spinal cord showed hyperintensity within the upper 4 and lower 4 thoracic levels. The diagnosis of NMO was entertained.

Following intravenous steroids, the patient was treated with a tapering course of oral steroids. She began to make a slow recovery with the aid of intense physiotherapy. Having walked in at her initial admission, she returned home needing crutches 4 weeks later and the visual acuity in her right eye remained 20/120 at best. Her requirement for intermittent urinary self‐catheterization and rectal enemas decreased with time. She continued to be rehabilitated at home with input from the community physiotherapy team and remains in remission at 12 months.

Discussion

First described by Eugene Devic in 1894, NMO is a rare, chronic inflammatory, demyelinating disease of the central nervous system.1 It is more common among Asians and Africans, with a female predominance (females comprise over two‐thirds of patients and more than 80% of those with the relapsing form of the disease).2 The mean age of onset is 35 to 47 years.2 The prevalence and incidence of NMO has not been established, partly because the disease is underrecognized and often confused with MS.

Rapid onset visual loss with limb involvement in a postpartum female invites a number of differential diagnoses, including infectious encephalomyelitis, vasculitis, an acute demyelinating disorder, and arterial/venous sinus thrombosis. We report an interesting, but initially puzzling, case of rapidly progressive optic neuritis and transverse myelitis in a postpartum Ghanaian female, later diagnosed as neuromyelitis optica (NMO).

Case Report

A 33‐year‐old Ghanaian female presented with bilateral eye pain worsened by eye movement, rapid visual loss, bifrontal headache, and right leg weakness for 48 hours. She was 8 weeks postpartum, with labor having been induced at 35 weeks due to hypertension; she was discharged on labetolol and no other medications. There was no other relevant past medical or family history, or illicit drug use.

On admission, her blood pressure was 148/99 mm Hg, pulse rate 92 beats per minute, temperature 37.8C, oxygen saturation 97% on room air, respiratory rate 14 breaths per minute, and Glasgow coma scale 15/15. Cranial nerve examination showed absent light perception in the right eye with a relative afferent pupillary defect, 20/30 Snellen visual acuity in the left eye, bilateral optic disc edema (worse on the right) and grade 1 hypertensive retinopathy, and slight facial asymmetry but otherwise grossly intact cranial nerves. Neurological examination of the limbs showed 5/5 power in upper limbs, but flexion was weaker (3/5) than extension (4/5) in lower limbs. There was hyperreflexia in the right arm and right leg with positive Babinski sign. Sensation, proprioception, and coordination were normal in both upper and lower limbs; a Romberg test was negative.

Other systems examinations were unremarkable. Complete blood count, standard electrolytes, erythrocyte sedimentation rate, C‐reactive protein, urinalysis, chest x‐ray, and an urgent head computed tomography (CT) with contrast were all noncontributory.

To cover a broad range of possible infective processes, ceftriaxone and acyclovir were started. In view of the rapid progression of her presentation she was transferred urgently to a specialty neurology hospital. There she was started on intravenous methylprednisolone to cover possible inflammatory causes, such as multiple sclerosis (MS) and vasculitis. However, her lower limb weakness progressed and she developed urinary retention. Lumbar puncture was performed; the opening pressure was 160 mm H₂O. Cerebrospinal fluid (CSF) analysis demonstrated elevated protein level (121 mg/dL), 16/mm³ lymphocytosis, normal glucose, and negative gram stain and culture. Conventional CSF polymerase chain reaction for herpes simplex, Epstein‐Barr, varicella‐zoster, cytomegalovirus, John Cunningham virus (to rule out progressive multifocal leukoencephalopathy), human herpesvirus 6 viruses, and toxoplasma were negative. Antinuclear antibody, anticytoplasmic antibody, antiganglioside antibodies (GM1, GQ1b), thrombophilia screen (activated protein C resistance ratio, lupus anticoagulant and anticardiolipin antibodies, antithrombin III, protein C, and S deficiency), B12, folate, ferritin, and human immunodeficiency virus (HIV) screen were also negative. Brain magnetic resonance imaging (MRI), including venography, was normal. However, T2‐weighted MRI images of the orbit showed signal hyperintensity of the left optic chiasm and optic nerves, and T2‐weighted images of the spinal cord showed hyperintensity within the upper 4 and lower 4 thoracic levels. The diagnosis of NMO was entertained.

Following intravenous steroids, the patient was treated with a tapering course of oral steroids. She began to make a slow recovery with the aid of intense physiotherapy. Having walked in at her initial admission, she returned home needing crutches 4 weeks later and the visual acuity in her right eye remained 20/120 at best. Her requirement for intermittent urinary self‐catheterization and rectal enemas decreased with time. She continued to be rehabilitated at home with input from the community physiotherapy team and remains in remission at 12 months.

Discussion

First described by Eugene Devic in 1894, NMO is a rare, chronic inflammatory, demyelinating disease of the central nervous system.1 It is more common among Asians and Africans, with a female predominance (females comprise over two‐thirds of patients and more than 80% of those with the relapsing form of the disease).2 The mean age of onset is 35 to 47 years.2 The prevalence and incidence of NMO has not been established, partly because the disease is underrecognized and often confused with MS.

Rapid onset visual loss with limb involvement in a postpartum female invites a number of differential diagnoses, including infectious encephalomyelitis, vasculitis, an acute demyelinating disorder, and arterial/venous sinus thrombosis. We report an interesting, but initially puzzling, case of rapidly progressive optic neuritis and transverse myelitis in a postpartum Ghanaian female, later diagnosed as neuromyelitis optica (NMO).

Case Report

A 33‐year‐old Ghanaian female presented with bilateral eye pain worsened by eye movement, rapid visual loss, bifrontal headache, and right leg weakness for 48 hours. She was 8 weeks postpartum, with labor having been induced at 35 weeks due to hypertension; she was discharged on labetolol and no other medications. There was no other relevant past medical or family history, or illicit drug use.

On admission, her blood pressure was 148/99 mm Hg, pulse rate 92 beats per minute, temperature 37.8C, oxygen saturation 97% on room air, respiratory rate 14 breaths per minute, and Glasgow coma scale 15/15. Cranial nerve examination showed absent light perception in the right eye with a relative afferent pupillary defect, 20/30 Snellen visual acuity in the left eye, bilateral optic disc edema (worse on the right) and grade 1 hypertensive retinopathy, and slight facial asymmetry but otherwise grossly intact cranial nerves. Neurological examination of the limbs showed 5/5 power in upper limbs, but flexion was weaker (3/5) than extension (4/5) in lower limbs. There was hyperreflexia in the right arm and right leg with positive Babinski sign. Sensation, proprioception, and coordination were normal in both upper and lower limbs; a Romberg test was negative.

Other systems examinations were unremarkable. Complete blood count, standard electrolytes, erythrocyte sedimentation rate, C‐reactive protein, urinalysis, chest x‐ray, and an urgent head computed tomography (CT) with contrast were all noncontributory.

To cover a broad range of possible infective processes, ceftriaxone and acyclovir were started. In view of the rapid progression of her presentation she was transferred urgently to a specialty neurology hospital. There she was started on intravenous methylprednisolone to cover possible inflammatory causes, such as multiple sclerosis (MS) and vasculitis. However, her lower limb weakness progressed and she developed urinary retention. Lumbar puncture was performed; the opening pressure was 160 mm H₂O. Cerebrospinal fluid (CSF) analysis demonstrated elevated protein level (121 mg/dL), 16/mm³ lymphocytosis, normal glucose, and negative gram stain and culture. Conventional CSF polymerase chain reaction for herpes simplex, Epstein‐Barr, varicella‐zoster, cytomegalovirus, John Cunningham virus (to rule out progressive multifocal leukoencephalopathy), human herpesvirus 6 viruses, and toxoplasma were negative. Antinuclear antibody, anticytoplasmic antibody, antiganglioside antibodies (GM1, GQ1b), thrombophilia screen (activated protein C resistance ratio, lupus anticoagulant and anticardiolipin antibodies, antithrombin III, protein C, and S deficiency), B12, folate, ferritin, and human immunodeficiency virus (HIV) screen were also negative. Brain magnetic resonance imaging (MRI), including venography, was normal. However, T2‐weighted MRI images of the orbit showed signal hyperintensity of the left optic chiasm and optic nerves, and T2‐weighted images of the spinal cord showed hyperintensity within the upper 4 and lower 4 thoracic levels. The diagnosis of NMO was entertained.

Following intravenous steroids, the patient was treated with a tapering course of oral steroids. She began to make a slow recovery with the aid of intense physiotherapy. Having walked in at her initial admission, she returned home needing crutches 4 weeks later and the visual acuity in her right eye remained 20/120 at best. Her requirement for intermittent urinary self‐catheterization and rectal enemas decreased with time. She continued to be rehabilitated at home with input from the community physiotherapy team and remains in remission at 12 months.

Discussion

First described by Eugene Devic in 1894, NMO is a rare, chronic inflammatory, demyelinating disease of the central nervous system.1 It is more common among Asians and Africans, with a female predominance (females comprise over two‐thirds of patients and more than 80% of those with the relapsing form of the disease).2 The mean age of onset is 35 to 47 years.2 The prevalence and incidence of NMO has not been established, partly because the disease is underrecognized and often confused with MS.