Chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin-155 antibodies: A first case report in Peru
Chronic inflammatory demyelinating polyradiculoneuropathy is a clinically heterogeneous group of immune- mediated peripheral neuropathies that share neurophysiological manifesta-tions of demyelination and albuminocytologic dissociation. There are typical and atypical variants of this disease, some...
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| Language: | English |
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Medwave Estudios Limitada
2023-01-01
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| Series: | Medwave |
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| Online Access: | https://www.medwave.cl/investigacion/casos/2634.html |
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| author | Darwin Segura-Chávez Arantxa Sanchez-Boluarte Kelvin Alvarez-Toledo Jorge Caciano-López Isabel Tagle-Lostaunau Francisco Aquino-Peña Juan Sifuentes-Monge |
| author_facet | Darwin Segura-Chávez Arantxa Sanchez-Boluarte Kelvin Alvarez-Toledo Jorge Caciano-López Isabel Tagle-Lostaunau Francisco Aquino-Peña Juan Sifuentes-Monge |
| author_sort | Darwin Segura-Chávez |
| collection | DOAJ |
| description | Chronic inflammatory demyelinating polyradiculoneuropathy is a clinically heterogeneous group of immune- mediated peripheral neuropathies that share neurophysiological manifesta-tions of demyelination and albuminocytologic dissociation.
There are typical and atypical variants of this disease, some associated with antibodies against proteins of the node of Ranvier, such as neurofascin- 155.
We present the case of a 38- year- old male who presented with an eight- month history of par-esthesia and progressive weakness of four limbs associated with diplopia and dysphagia. The patient was conscious, with symmetric flaccid quadriparesis of distal predominance, hyp-otrophy in the dorsum and palm of both hands, generalized areflexia, postural low frequency, and high amplitude tremor in upper limbs of left predominance, appendicular dysmetria, dys-diadochokinesia, ophthalmoparesis to dextroversion in the right eye, absent gag reflex, ataxic gait with an increased base of support and positive Romberg's sign.
Cerebrospinal fluid showed albuminocytologic dissociation, and electromyography was com-patible with primarily demyelinating sensory- motor polyneuropathy.
Due to clinical suspicion, we requested anti- neurofascin- 155 antibodies, which tested positive.
The patient was treated with methylprednisolone at a dose of one gram per day for five days, followed by one milligram per kilogram for three months of prednisone, with progressive de-crease, which improved diplopia and dysphagia, with no effect on limb strength and even worsening of function. For this reason, treatment with rituximab was started in doses of two grams, presenting a substantial improvement in distal muscle strength, tremor, gait stability, coordination, and functionality measured with the modified Rankin scale. |
| first_indexed | 2024-04-10T00:23:41Z |
| format | Article |
| id | doaj.art-df0dbee17c54473b82ec68383479901b |
| institution | Directory Open Access Journal |
| issn | 0717-6384 |
| language | English |
| last_indexed | 2024-04-10T00:23:41Z |
| publishDate | 2023-01-01 |
| publisher | Medwave Estudios Limitada |
| record_format | Article |
| series | Medwave |
| spelling | doaj.art-df0dbee17c54473b82ec68383479901b2023-03-15T14:44:58ZengMedwave Estudios LimitadaMedwave0717-63842023-01-012201e2634e263410.5867/medwave.2023.01.2634Chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin-155 antibodies: A first case report in PeruDarwin Segura-Chávez0https://orcid.org/0000-0002-7016-9480Arantxa Sanchez-Boluarte1https://orcid.org/0000-0003-0876-7196Kelvin Alvarez-Toledo2https://orcid.org/0000-0002-6111-8489Jorge Caciano-López3https://orcid.org/0000-0002-9406-6881Isabel Tagle-Lostaunau4https://orcid.org/0000-0002-9052-6254Francisco Aquino-Peña5https://orcid.org/0000-0003-0922-9242Juan Sifuentes-Monge6https://orcid.org/0000-0003-4537-6864Centro Básico de Investigación en Enfermedades Neuromusculares y de Motoneurona, Instituto Nacional de Ciencias Neurológicas, Lima, PerúEscuela de Medicina, Facultad de medicina, Universidad Peruana Cayetano Heredia, Lima, PerúCentro Básico de Investigación en Demencias y Enfermedades Desmielinizantes del Sistema Nervioso Central, Instituto Nacional de Ciencias Neurológicas, Lima, PerúServicio de Neurología, Hospital Daniel Alcides Carrión, Lima, PerúCentro Básico de Investigación en Enfermedades Neuromusculares y de Motoneurona, Instituto Nacional de Ciencias Neurológicas, Lima, PerúCentro Básico de Investigación en Enfermedades Neuromusculares y de Motoneurona, Instituto Nacional de Ciencias Neurológicas, Lima, PerúCentro Básico de Investigación en Enfermedades Neuromusculares y de Motoneurona, Instituto Nacional de Ciencias Neurológicas, Lima, PerúChronic inflammatory demyelinating polyradiculoneuropathy is a clinically heterogeneous group of immune- mediated peripheral neuropathies that share neurophysiological manifesta-tions of demyelination and albuminocytologic dissociation. There are typical and atypical variants of this disease, some associated with antibodies against proteins of the node of Ranvier, such as neurofascin- 155. We present the case of a 38- year- old male who presented with an eight- month history of par-esthesia and progressive weakness of four limbs associated with diplopia and dysphagia. The patient was conscious, with symmetric flaccid quadriparesis of distal predominance, hyp-otrophy in the dorsum and palm of both hands, generalized areflexia, postural low frequency, and high amplitude tremor in upper limbs of left predominance, appendicular dysmetria, dys-diadochokinesia, ophthalmoparesis to dextroversion in the right eye, absent gag reflex, ataxic gait with an increased base of support and positive Romberg's sign. Cerebrospinal fluid showed albuminocytologic dissociation, and electromyography was com-patible with primarily demyelinating sensory- motor polyneuropathy. Due to clinical suspicion, we requested anti- neurofascin- 155 antibodies, which tested positive. The patient was treated with methylprednisolone at a dose of one gram per day for five days, followed by one milligram per kilogram for three months of prednisone, with progressive de-crease, which improved diplopia and dysphagia, with no effect on limb strength and even worsening of function. For this reason, treatment with rituximab was started in doses of two grams, presenting a substantial improvement in distal muscle strength, tremor, gait stability, coordination, and functionality measured with the modified Rankin scale.https://www.medwave.cl/investigacion/casos/2634.htmlanti-neurofascin 155distal acquired demyelinating symmetric neuropathy |
| spellingShingle | Darwin Segura-Chávez Arantxa Sanchez-Boluarte Kelvin Alvarez-Toledo Jorge Caciano-López Isabel Tagle-Lostaunau Francisco Aquino-Peña Juan Sifuentes-Monge Chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin-155 antibodies: A first case report in Peru Medwave anti-neurofascin 155 distal acquired demyelinating symmetric neuropathy |
| title | Chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin-155 antibodies: A first case report in Peru |
| title_full | Chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin-155 antibodies: A first case report in Peru |
| title_fullStr | Chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin-155 antibodies: A first case report in Peru |
| title_full_unstemmed | Chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin-155 antibodies: A first case report in Peru |
| title_short | Chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin-155 antibodies: A first case report in Peru |
| title_sort | chronic inflammatory demyelinating polyradiculoneuropathy with antineurofascin 155 antibodies a first case report in peru |
| topic | anti-neurofascin 155 distal acquired demyelinating symmetric neuropathy |
| url | https://www.medwave.cl/investigacion/casos/2634.html |
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