Anterior horn syndrome: A rare manifestation of primary Sjögren's syndrome.
The authors report an exceptional case of an anterior horn syndrome associated with Sjögren's syndrome in a 58-year-old patient with a flaccid tetraparesis revealed by asymmetric atrophy and diffuse fasciculations associated with xerostomia and xerophthalmia. The electroneuromyography objectified a diffuse anterior horn syndrome. The brain MRI and spinal cord were normal. Laboratory tests revealed positive anti-SSA and anti-SSB antibody. The salivary glands biopsy objectified lymphocytic sialadenitis grade 3 of Chisholm. The Schirmer's test was abnormally low. Diagnosis of anterior horn syndrome as part of Sjögren's syndrome was retained. The methylprednisolone bolus allowed partial clinical improvement after 12 months of evolution. Therefore, in patients with isolated anterior horn involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable.
Centre universitaire Mohamed VI, hôpital Errazi, service de neurologie, Marrakech, Morocco; Université Cadi Ayad, faculté de médecine et de pharmacie, département de neurologie, Sidi Abad BP 7010, Marrakech, Morocco. Electronic address: safaazahlane@gmail.com.
Safaa
S
Zahlane
Centre universitaire Mohamed VI, hôpital Errazi, service de neurologie, Marrakech, Morocco.
Nissrine
N
Louhab
Centre universitaire Mohamed VI, hôpital Errazi, service de neurologie, Marrakech, Morocco.
Meriem
M
El Mellakh
Centre universitaire Mohamed VI, hôpital Errazi, service de neurologie, Marrakech, Morocco.
Najib
N
Kissani
D004576:Electromyography / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D011239:Prednisolone / Q000008:administration & dosage*; D035583:Rare Diseases; D018570:Risk Assessment; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications* / Q000175:diagnosis; D013577:Syndrome; D016896:Treatment Outcome
D011239:Prednisolone; D008775:Methylprednisolone
Anterior horn syndrome; Peripheral neuropathy; Primary Sjögren's syndrome