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Neuralgic amyotrophy associated to hepatitis E virus infection
462
Letters to the Editor / Med Clin (Barc). 2015;145(10):458–463
5. Cordier JF, Loire R, Brune J. Amyloidosis of the tracheobronchial tree. N Engl J Med. 1962;266:587–91. 6. Petermann W, Barth J, Schluter E. Localized amyloidosis of central airways. Eur J Respir Dis. 1987;71:210–2. 7. Thompson PJ, Citron KM. Amyloid and the lower respiratory tract. Thorax. 1983;38:84–7. 8. Shinoi K, Shiraishi Y, Yahata JI. Amyloid tumor of the trachea and the lung, resembling bronchial asthma. Dis Chest. 1962;42:442–5. 9. Chew HC, Low SY, Eng P, Agasthian T, Cheah FK. Cough and persistent wheeze with long-standing asthma. Chest. 2007;132:727–31. 10. Puchtler H, Sweat F. On the binding of Congo red by amyloid. J Histochem Cytochem. 1962;10:355–64.
Neuralgic amyotrophy associated to hepatitis E virus infection夽 Neuralgia amiotrófica en relación con infección por el virus de la hepatitis E Dear Editor, Neuralgic amyotrophy (NA) is characterized by severe pain with subsequent onset of weakness, muscle atrophy and, occasionally, sensory deficit; characteristically symptoms that affect the brachial plexus nerve trunks. It is suggested that the NA has an autoimmune basis triggered by clinical phenomena such as trauma, surgery, immunization and more frequently in connection with previous infectious processes.1 A case of NA preceded by hepatitis E virus (HEV) infection is detailed next. Male, 41 years of age, without any relevant medical history, complaining of malaise, 38.5 ◦ C fever and choluria of 2-day progression. Lab reports showed impaired liver function tests (LFTs): alkaline phosphatase: 241 U/L, aspartate aminotransferase: 481 U/L, alanine aminotransferase: 1270 U/L, gamma-glutamyltransferase: 1189 U/L and total bilirubin: 4.2 mg/dl. The patient is diagnosed with acute hepatitis with subsequent normalization of LFTs. Serological studies confirmed acute HEV infection after demonstrating the presence of anti-HEV IgM (index: 13.2 arbitrary units, cut-off
Ana Pando Sandoval ∗ , Miguel Angel Ariza Prota, Marta García Clemente, Pere Casan Área del Pulmón, Instituto Nacional de Silicosis (INS), Hospital Universitario Central de Asturias (HUCA), Facultad de Medicina, Universidad de Oviedo, Oviedo, Asturias, Spain ∗ Corresponding author. E-mail address: ana [email protected] (A. Pando Sandoval).
value > 1). Quantification of HEV RNA, performed 2 months after the onset of the clinical condition, was undetectable. After 5 days of starting the symptoms secondary to hepatitis, the patient had severe pain in the right shoulder with poor response to analgesic treatment; 5 days later, functional impairment was added for lifting the right upper limb, with no sensory symptoms. On physical examination, only a motor deficit was observed in the right upper limb abduction when raising it over his shoulder accompanied by right winged scapula with no sensitivity alterations and preservation of tendon reflexes. The neurophysiological study showed the presence of a subacute neurogenic pattern with signs of denervation–reinnervation active at right serratus anterior muscle level, without observing other disorders of the brachial plexus. NA diagnosis was established by involvement of the right long thoracic nerve in connection with acute HEV infection. With an incidence of 1.64 cases per 100,000 persons/year, NA mainly affects males between the third and seventh decade. It presents a single phase progression with acute onset predominantly affecting the upper trunk of the brachial plexus and bilateral involvement in 30% of patients. The pathogenesis of NA is multifactorial, although studies point to an autoimmune basis since most patients report an infectious process prior to development of the neurological symptoms. Nevertheless, there are NA cases where a trigger cannot be identified.1
Table 1 Description of cases of acute neuralgic amyotrophy associated with infection by hepatitis E virus. Age (years)
Sex
Interval (days) between the onset of hepatitis symptoms and the neurological symptoms
Unilateral brachial plexus involvement/bilateral
AST (IU/l)
Prognosis
382 283 534 495 566 387
Male Male Male Male Male Male
5 NA 0 3 1 NA
Bilateral Bilateral Bilateral Bilateral Bilateral Unilateral
NA 147 247 155 234 1612
378 358 408 368 348 309
Male Male Female Male Male Male
1 90 4 15 7 0
Bilateral Bilateral Bilateral Bilateral Bilateral Bilateral
113 NA 177 64 86 517
523 41 New case
Male Male
7 5
Bilateral Unilateral
179 481
Residual motor deficit at 18 months NA Resolution in 2 years Residual weakness Persistence of pain for 10 months Persistence of pain and muscular atrophy at 4 months Residual weakness Residual weakness Residual weakness Residual weakness Residual weakness Progressive recovery of strength and weakness NA Residual weakness for shoulder elevation
AST, aspartate aminotransferase; NA, not available.
夽 Please cite this article as: Martínez Rodríguez L, Carvajal P, Morís G. Neuralgia amiotrófica en relación con infección por el virus de la hepatitis E. Med Clin (Barc). 2015;145:462–463.
Letters to the Editor / Med Clin (Barc). 2015;145(10):458–463
HEV is an emerging pathogen in the developed world, is an RNA virus with a broad genetic variability, classically described in outbreaks by enterofecal transmission in Third World countries, but in developed countries is considered a zoonosis associated with eating undercooked pork products.10 HEV infection has been associated with the development of different neurological conditions, such as Guillain–Barre syndrome (GBS), transverse myelitis or acute encephalitis3 . In recent years several cases of NA associated with acute HEV infection have been described (Table 1).2–8 A recent study found that 10% of 47 retrospectively analyzed NA diagnosed cases showed acute HEV infection, although unlike our case, all cases showed bilateral involvement of the brachial plexus.8 The pathophysiology of neural damage in cases of NA associated with HEV is unknown, hypothesizing both, a direct viral infection of the brachial plexus as well as the presence of HEV molecular mimicry as responsible for the initiation of the aberrant immune response, similar to the cases of GBS associated with HEV.8 Characteristically, type 3 is the viral genotype associated with the neurological symptoms, the dominant genotype in Europe. Acute HEV infection is confirmed by the presence of anti-HEV IgM, positive between 3 and 6 months after contact with the virus and viral RNA quantification by PCR which rapidly becomes negative in immunocompetent patients.10 The HEV must be taken into account as causal agent in cases of NA affecting young men with bilateral symptoms due to upper brachial plexus involvement accompanied by alteration of LFTs. References 1. Van Alfen N. Clinical and pathophysiological concepts of neuralgic amyotrophy. Nat Rev Neurol. 2011;7:315–22.
463
2. Kamar N, Bendall RP, Peron JM, Cintas P, Prudhomme L, Mansuy JM, et al. Hepatitis E virus and neurologic disorders. Emerg Infect Dis. 2011;17: 173–9. 3. Motte A, Franques J, Weitten T, Colson P. Hepatitis E-associated Parsonage–Turner syndrome, France. Clin Res Hepatol Gastroenterol. 2014;38:e11–4. 4. Fong F, Illahi M. Neuralgic amyotrophy associated with hepatitis E virus. Clin Neurol Neurosurg. 2009;111:193–5. 5. Rianthavorn P, Thongmee C, Limpaphayom N, Komolmit P, Theamboonlers A, Poovorawan Y. The entire genome sequence of hepatitis E virus genotype 3 isolated from a patient with neuralgic amyotrophy. Scand J Infect Dis. 2010;42:395–400. 6. Cheung MC, Maguire J, Carey I, Wendon J, Agarwal K. Hepatitis E – an unexpected problem at home. Scand J Gastroenterol. 2012;47:253. 7. Deroux A, Brion JP, Hyerle L, Belbezier A, Vaillant M, Mosnier E, et al. Association between hepatitis E and neurological disorders: two case studies and literature review. J Clin Virol. 2014;60:60–2. 8. Van Eijk JJ, Madden RG, van der Eijk AA, Hunter JG, Reimerink JH, Bendall RP, et al. Neuralgic amyotrophy and hepatitis E virus infection. Neurology. 2014;82:498–503. 9. Carli P, Landais C, Poisnel E, Cournac JM, Aletti M, Paris JF, et al. Shoulder pain in a 30-year-old man. Rev Med Interne. 2012;33:111–4. 10. Soriano V, Barreiro P. Hepatitis E: an emerging viral disease. Med Clin (Barc). 2014;143:22–4.
Laura Martínez Rodríguez a , Paula Carvajal b , Germán Morís a,∗ a Servicio de Neurología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain b Servicio de Neurofisiología Clínica, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain ∗ Corresponding author. E-mail address: [email protected] (G. Morís).
Letters to the Editor / Med Clin (Barc). 2015;145(10):458–463
5. Cordier JF, Loire R, Brune J. Amyloidosis of the tracheobronchial tree. N Engl J Med. 1962;266:587–91. 6. Petermann W, Barth J, Schluter E. Localized amyloidosis of central airways. Eur J Respir Dis. 1987;71:210–2. 7. Thompson PJ, Citron KM. Amyloid and the lower respiratory tract. Thorax. 1983;38:84–7. 8. Shinoi K, Shiraishi Y, Yahata JI. Amyloid tumor of the trachea and the lung, resembling bronchial asthma. Dis Chest. 1962;42:442–5. 9. Chew HC, Low SY, Eng P, Agasthian T, Cheah FK. Cough and persistent wheeze with long-standing asthma. Chest. 2007;132:727–31. 10. Puchtler H, Sweat F. On the binding of Congo red by amyloid. J Histochem Cytochem. 1962;10:355–64.
Neuralgic amyotrophy associated to hepatitis E virus infection夽 Neuralgia amiotrófica en relación con infección por el virus de la hepatitis E Dear Editor, Neuralgic amyotrophy (NA) is characterized by severe pain with subsequent onset of weakness, muscle atrophy and, occasionally, sensory deficit; characteristically symptoms that affect the brachial plexus nerve trunks. It is suggested that the NA has an autoimmune basis triggered by clinical phenomena such as trauma, surgery, immunization and more frequently in connection with previous infectious processes.1 A case of NA preceded by hepatitis E virus (HEV) infection is detailed next. Male, 41 years of age, without any relevant medical history, complaining of malaise, 38.5 ◦ C fever and choluria of 2-day progression. Lab reports showed impaired liver function tests (LFTs): alkaline phosphatase: 241 U/L, aspartate aminotransferase: 481 U/L, alanine aminotransferase: 1270 U/L, gamma-glutamyltransferase: 1189 U/L and total bilirubin: 4.2 mg/dl. The patient is diagnosed with acute hepatitis with subsequent normalization of LFTs. Serological studies confirmed acute HEV infection after demonstrating the presence of anti-HEV IgM (index: 13.2 arbitrary units, cut-off
Ana Pando Sandoval ∗ , Miguel Angel Ariza Prota, Marta García Clemente, Pere Casan Área del Pulmón, Instituto Nacional de Silicosis (INS), Hospital Universitario Central de Asturias (HUCA), Facultad de Medicina, Universidad de Oviedo, Oviedo, Asturias, Spain ∗ Corresponding author. E-mail address: ana [email protected] (A. Pando Sandoval).
value > 1). Quantification of HEV RNA, performed 2 months after the onset of the clinical condition, was undetectable. After 5 days of starting the symptoms secondary to hepatitis, the patient had severe pain in the right shoulder with poor response to analgesic treatment; 5 days later, functional impairment was added for lifting the right upper limb, with no sensory symptoms. On physical examination, only a motor deficit was observed in the right upper limb abduction when raising it over his shoulder accompanied by right winged scapula with no sensitivity alterations and preservation of tendon reflexes. The neurophysiological study showed the presence of a subacute neurogenic pattern with signs of denervation–reinnervation active at right serratus anterior muscle level, without observing other disorders of the brachial plexus. NA diagnosis was established by involvement of the right long thoracic nerve in connection with acute HEV infection. With an incidence of 1.64 cases per 100,000 persons/year, NA mainly affects males between the third and seventh decade. It presents a single phase progression with acute onset predominantly affecting the upper trunk of the brachial plexus and bilateral involvement in 30% of patients. The pathogenesis of NA is multifactorial, although studies point to an autoimmune basis since most patients report an infectious process prior to development of the neurological symptoms. Nevertheless, there are NA cases where a trigger cannot be identified.1
Table 1 Description of cases of acute neuralgic amyotrophy associated with infection by hepatitis E virus. Age (years)
Sex
Interval (days) between the onset of hepatitis symptoms and the neurological symptoms
Unilateral brachial plexus involvement/bilateral
AST (IU/l)
Prognosis
382 283 534 495 566 387
Male Male Male Male Male Male
5 NA 0 3 1 NA
Bilateral Bilateral Bilateral Bilateral Bilateral Unilateral
NA 147 247 155 234 1612
378 358 408 368 348 309
Male Male Female Male Male Male
1 90 4 15 7 0
Bilateral Bilateral Bilateral Bilateral Bilateral Bilateral
113 NA 177 64 86 517
523 41 New case
Male Male
7 5
Bilateral Unilateral
179 481
Residual motor deficit at 18 months NA Resolution in 2 years Residual weakness Persistence of pain for 10 months Persistence of pain and muscular atrophy at 4 months Residual weakness Residual weakness Residual weakness Residual weakness Residual weakness Progressive recovery of strength and weakness NA Residual weakness for shoulder elevation
AST, aspartate aminotransferase; NA, not available.
夽 Please cite this article as: Martínez Rodríguez L, Carvajal P, Morís G. Neuralgia amiotrófica en relación con infección por el virus de la hepatitis E. Med Clin (Barc). 2015;145:462–463.
Letters to the Editor / Med Clin (Barc). 2015;145(10):458–463
HEV is an emerging pathogen in the developed world, is an RNA virus with a broad genetic variability, classically described in outbreaks by enterofecal transmission in Third World countries, but in developed countries is considered a zoonosis associated with eating undercooked pork products.10 HEV infection has been associated with the development of different neurological conditions, such as Guillain–Barre syndrome (GBS), transverse myelitis or acute encephalitis3 . In recent years several cases of NA associated with acute HEV infection have been described (Table 1).2–8 A recent study found that 10% of 47 retrospectively analyzed NA diagnosed cases showed acute HEV infection, although unlike our case, all cases showed bilateral involvement of the brachial plexus.8 The pathophysiology of neural damage in cases of NA associated with HEV is unknown, hypothesizing both, a direct viral infection of the brachial plexus as well as the presence of HEV molecular mimicry as responsible for the initiation of the aberrant immune response, similar to the cases of GBS associated with HEV.8 Characteristically, type 3 is the viral genotype associated with the neurological symptoms, the dominant genotype in Europe. Acute HEV infection is confirmed by the presence of anti-HEV IgM, positive between 3 and 6 months after contact with the virus and viral RNA quantification by PCR which rapidly becomes negative in immunocompetent patients.10 The HEV must be taken into account as causal agent in cases of NA affecting young men with bilateral symptoms due to upper brachial plexus involvement accompanied by alteration of LFTs. References 1. Van Alfen N. Clinical and pathophysiological concepts of neuralgic amyotrophy. Nat Rev Neurol. 2011;7:315–22.
463
2. Kamar N, Bendall RP, Peron JM, Cintas P, Prudhomme L, Mansuy JM, et al. Hepatitis E virus and neurologic disorders. Emerg Infect Dis. 2011;17: 173–9. 3. Motte A, Franques J, Weitten T, Colson P. Hepatitis E-associated Parsonage–Turner syndrome, France. Clin Res Hepatol Gastroenterol. 2014;38:e11–4. 4. Fong F, Illahi M. Neuralgic amyotrophy associated with hepatitis E virus. Clin Neurol Neurosurg. 2009;111:193–5. 5. Rianthavorn P, Thongmee C, Limpaphayom N, Komolmit P, Theamboonlers A, Poovorawan Y. The entire genome sequence of hepatitis E virus genotype 3 isolated from a patient with neuralgic amyotrophy. Scand J Infect Dis. 2010;42:395–400. 6. Cheung MC, Maguire J, Carey I, Wendon J, Agarwal K. Hepatitis E – an unexpected problem at home. Scand J Gastroenterol. 2012;47:253. 7. Deroux A, Brion JP, Hyerle L, Belbezier A, Vaillant M, Mosnier E, et al. Association between hepatitis E and neurological disorders: two case studies and literature review. J Clin Virol. 2014;60:60–2. 8. Van Eijk JJ, Madden RG, van der Eijk AA, Hunter JG, Reimerink JH, Bendall RP, et al. Neuralgic amyotrophy and hepatitis E virus infection. Neurology. 2014;82:498–503. 9. Carli P, Landais C, Poisnel E, Cournac JM, Aletti M, Paris JF, et al. Shoulder pain in a 30-year-old man. Rev Med Interne. 2012;33:111–4. 10. Soriano V, Barreiro P. Hepatitis E: an emerging viral disease. Med Clin (Barc). 2014;143:22–4.
Laura Martínez Rodríguez a , Paula Carvajal b , Germán Morís a,∗ a Servicio de Neurología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain b Servicio de Neurofisiología Clínica, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain ∗ Corresponding author. E-mail address: [email protected] (G. Morís).