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A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome.

Okoh HC, Lubana SS, Langevin S, Sanelli-Russo S, Abrudescu A - Case Rep Rheumatol (2015)

Bottom Line: Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate.Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease.We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG.

View Article: PubMed Central - PubMed

Affiliation: Icahn School of Medicine at Mount Sinai, Queens Hospital Center, Jamaica, NY 11432, USA.

ABSTRACT
Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG.

No MeSH data available.


Related in: MedlinePlus

Nerve conduction studies (see Table 1). Findings: evaluation of the right peroneal motor nerve showed prolonged distal onset latency (13.8 ms), reduced amplitude (Ankle, 0.0 mV), reduced amplitude (B Fib., 0.0 mV), and reduced amplitude (Poplt., 0.0 mV). F wave studies indicate that the right peroneal F wave has no response.
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fig1: Nerve conduction studies (see Table 1). Findings: evaluation of the right peroneal motor nerve showed prolonged distal onset latency (13.8 ms), reduced amplitude (Ankle, 0.0 mV), reduced amplitude (B Fib., 0.0 mV), and reduced amplitude (Poplt., 0.0 mV). F wave studies indicate that the right peroneal F wave has no response.

Mentions: Four days after admission, the patient's neurological status worsened. She became progressively weaker and lethargic and was only able to state her name. She had difficulty swallowing, speaking, taking deep breaths, and coughing. She was intubated for airway protection and placed on nasogastric tube (NGT) feeding. Neurologist evaluated the patient; exam was notable for absent reflexes in addition to progressive lower extremity weakness; GBS was suspected. She also developed ophthalmoparesis with inability of the eyes to cross the midline bilaterally. Stool studies were negative for Campylobacter jejuni and were guaiac negative. Lumbar puncture and plasma exchange were recommended. Cerebral spinal fluid (CSF) analysis revealed protein of 35 mg/dL; WBC was 8/cumm. Oligoclonal bands and myelin basic protein were absent, with Anti-GQ-1 antibody titers less than 1 : 100. West Nile viral titers were negative. Nerve conduction studies revealed absence of F wave response in right peroneal nerve, prolonged distal onset latency, and severe conduction block (Figure 1); although CSF results were inconsistent with GBS as the protein was not elevated, a diagnosis was made clinically. Treatment with plasma exchange was begun 5 days after admission. She received 5 plasma exchanges with no improvement.


A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome.

Okoh HC, Lubana SS, Langevin S, Sanelli-Russo S, Abrudescu A - Case Rep Rheumatol (2015)

Nerve conduction studies (see Table 1). Findings: evaluation of the right peroneal motor nerve showed prolonged distal onset latency (13.8 ms), reduced amplitude (Ankle, 0.0 mV), reduced amplitude (B Fib., 0.0 mV), and reduced amplitude (Poplt., 0.0 mV). F wave studies indicate that the right peroneal F wave has no response.
© Copyright Policy - open-access
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4553270&req=5

fig1: Nerve conduction studies (see Table 1). Findings: evaluation of the right peroneal motor nerve showed prolonged distal onset latency (13.8 ms), reduced amplitude (Ankle, 0.0 mV), reduced amplitude (B Fib., 0.0 mV), and reduced amplitude (Poplt., 0.0 mV). F wave studies indicate that the right peroneal F wave has no response.
Mentions: Four days after admission, the patient's neurological status worsened. She became progressively weaker and lethargic and was only able to state her name. She had difficulty swallowing, speaking, taking deep breaths, and coughing. She was intubated for airway protection and placed on nasogastric tube (NGT) feeding. Neurologist evaluated the patient; exam was notable for absent reflexes in addition to progressive lower extremity weakness; GBS was suspected. She also developed ophthalmoparesis with inability of the eyes to cross the midline bilaterally. Stool studies were negative for Campylobacter jejuni and were guaiac negative. Lumbar puncture and plasma exchange were recommended. Cerebral spinal fluid (CSF) analysis revealed protein of 35 mg/dL; WBC was 8/cumm. Oligoclonal bands and myelin basic protein were absent, with Anti-GQ-1 antibody titers less than 1 : 100. West Nile viral titers were negative. Nerve conduction studies revealed absence of F wave response in right peroneal nerve, prolonged distal onset latency, and severe conduction block (Figure 1); although CSF results were inconsistent with GBS as the protein was not elevated, a diagnosis was made clinically. Treatment with plasma exchange was begun 5 days after admission. She received 5 plasma exchanges with no improvement.

Bottom Line: Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate.Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease.We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG.

View Article: PubMed Central - PubMed

Affiliation: Icahn School of Medicine at Mount Sinai, Queens Hospital Center, Jamaica, NY 11432, USA.

ABSTRACT
Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG.

No MeSH data available.


Related in: MedlinePlus