Limits...
Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment.

Chroni E, Veltsista D, Gavanozi E, Vlachou T, Polychronopoulos P, Papathanasopoulos P - BMC Neurol (2015)

Bottom Line: Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs.Two-year follow-up showed no further deterioration.Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, School of Medicine, University of Patras, Patras, Greece. echroni@yahoo.com.

ABSTRACT

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) as a pure sensory variant is rarely encountered. Therefore the best treatment option is hard to define.

Case presentations: We reported two middle-aged patients of Caucasian origin, one female and one male, who over a period of several months presented limbs and gait ataxia. Clinical and neurophysiological examination revealed only sensory abnormalities. A diagnosis of atypical CIDP was suggested, considering the elevated CSF protein level and the presence of anti-gangliosides antibodies. Ten and 15 days respectively after initiation of prednisolone treatment both patients experienced exacerbation of sensory symptoms and emerging of muscle weakness. Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs. Two-year follow-up showed no further deterioration.

Conclusion: Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

No MeSH data available.


Related in: MedlinePlus

Clinical assessment scores of a 58 year old woman are plotted against treatment regime at different time-points.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
getmorefigures.php?uid=PMC4359520&req=5

Fig1: Clinical assessment scores of a 58 year old woman are plotted against treatment regime at different time-points.

Mentions: The first patient was initially treated with prednisolone (60 mg p.o. daily) but within 10 days she reported increase of unsteadiness and numbness which also involved the trigeminal nerve region. Nevertheless, according to the instructions she completed a two-month period of steroid treatment. At that time her sensory deficits enhanced, she manifested weakness of quadriceps and wrist extensor muscles (expanded MRC score reduced from 70 to 66) and became unable to walk without bilateral support. She scored 6 on the ONLS (2 on the arm scale). Prednisolone was tapered off and intravenous immunoglobulin (IVIg) therapy was started at a dose of 0.4 mg/kg/day. The second day of infusion the patient manifested severe headache, nausea, fever, nuchal rigidity and confusion. A second CSF analysis showed neutrophilic pleocytosis (700 white blood cells/mL, normal glucose and 0.93 g/L protein). Following thorough laboratory investigation a diagnosis of aseptic meningitis was made and IVIg treatment was discontinued. A course of rituximab (i.v. infusions at a dosage of 375 mg/m2 weekly for 4 weeks) was tried as a second-line treatment according to recent literature [12]. Three months following steroid withdrawal the patient’s clinical status returned to pre-steroid level (Figure 1). Two years later, following another two rituximab infusions, the patient was able to walk unaided and reported only minimal distal numbness of her hands and feet.Figure 1


Pure sensory chronic inflammatory polyneuropathy: rapid deterioration after steroid treatment.

Chroni E, Veltsista D, Gavanozi E, Vlachou T, Polychronopoulos P, Papathanasopoulos P - BMC Neurol (2015)

Clinical assessment scores of a 58 year old woman are plotted against treatment regime at different time-points.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4359520&req=5

Fig1: Clinical assessment scores of a 58 year old woman are plotted against treatment regime at different time-points.
Mentions: The first patient was initially treated with prednisolone (60 mg p.o. daily) but within 10 days she reported increase of unsteadiness and numbness which also involved the trigeminal nerve region. Nevertheless, according to the instructions she completed a two-month period of steroid treatment. At that time her sensory deficits enhanced, she manifested weakness of quadriceps and wrist extensor muscles (expanded MRC score reduced from 70 to 66) and became unable to walk without bilateral support. She scored 6 on the ONLS (2 on the arm scale). Prednisolone was tapered off and intravenous immunoglobulin (IVIg) therapy was started at a dose of 0.4 mg/kg/day. The second day of infusion the patient manifested severe headache, nausea, fever, nuchal rigidity and confusion. A second CSF analysis showed neutrophilic pleocytosis (700 white blood cells/mL, normal glucose and 0.93 g/L protein). Following thorough laboratory investigation a diagnosis of aseptic meningitis was made and IVIg treatment was discontinued. A course of rituximab (i.v. infusions at a dosage of 375 mg/m2 weekly for 4 weeks) was tried as a second-line treatment according to recent literature [12]. Three months following steroid withdrawal the patient’s clinical status returned to pre-steroid level (Figure 1). Two years later, following another two rituximab infusions, the patient was able to walk unaided and reported only minimal distal numbness of her hands and feet.Figure 1

Bottom Line: Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs.Two-year follow-up showed no further deterioration.Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, School of Medicine, University of Patras, Patras, Greece. echroni@yahoo.com.

ABSTRACT

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) as a pure sensory variant is rarely encountered. Therefore the best treatment option is hard to define.

Case presentations: We reported two middle-aged patients of Caucasian origin, one female and one male, who over a period of several months presented limbs and gait ataxia. Clinical and neurophysiological examination revealed only sensory abnormalities. A diagnosis of atypical CIDP was suggested, considering the elevated CSF protein level and the presence of anti-gangliosides antibodies. Ten and 15 days respectively after initiation of prednisolone treatment both patients experienced exacerbation of sensory symptoms and emerging of muscle weakness. Steroids were then substituted by rituximab in the first patient and intravenous immunoglobulin in the second patient resulting in gradual decrement of symptoms and signs. Two-year follow-up showed no further deterioration.

Conclusion: Caution should be exercised when treating cases of pure sensory polyneuropathy with high dose steroids since an unfavorable outcome is possible.

No MeSH data available.


Related in: MedlinePlus