Primary Sjögren′s syndrome manifesting as multiple cranial neuropathies: MRI findings

Ashraf, VV; Bhasi, Ramesh; Kumar, R Praveen; Girija, A S

doi:10.4103/0972-2327.53083

Cited by 21 publications

(22 citation statements)

References 9 publications

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“…Anterior pituitary enlargement and palsy of cranial nerve VI in this patient initially raised the possibility of an inflammatory disorder of the pituitary or a pituitary adenoma. Indeed, hypophysitis, particularly granulomatous hypophysitis, can present as a sellar mass associated with cranial nerve palsies due to involvement of the adjacent cavernous sinus [ 29 ] as can occur with other inflammatory disorders of the pituitary including Sjögren's syndrome [ 30 , 31 ], sarcoidosis [ 32 ], Wegener's granulomatosis [ 33 , 34 ], tuberculosis [ 35 ], syphilis [ 36 , 37 ], and fungal infections [ 38 ]. Langerhans histiocytosis, germinoma, and lymphoma may also be associated with similar findings but often associated with central diabetes insipidus [ 39 – 41 ].…”

Section: Discussionmentioning

confidence: 99%

Bilateral Carotid-Cavernous Fistulas: An Uncommon Cause of Pituitary Enlargement and Hypopituitarism

Liberatore

Lechan

2016

Case Reports in Endocrinology

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Carotid-cavernous fistulas (CCFs) are rare, pathologic communications of the carotid artery and the venous plexus of the cavernous sinus. They can develop spontaneously in certain at risk individuals or following traumatic head injury. Typical clinical manifestations include headache, proptosis, orbital pain, and diplopia. We report a case of bilateral carotid-cavernous fistulas associated with these symptoms and also with pituitary enlargement and hypopituitarism, which improved following surgical intervention. Arterialization of the cavernous sinus and elevated portal pressure may interfere with normal venous drainage and the conveyance of inhibiting and releasing hormones from the hypothalamus, resulting in pituitary enlargement and hypopituitarism. This condition should be considered in the differential diagnosis of hypopituitarism associated with anterior pituitary enlargement.

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Section: Discussionmentioning

confidence: 99%

Bilateral Carotid-Cavernous Fistulas: An Uncommon Cause of Pituitary Enlargement and Hypopituitarism

Liberatore

Lechan

2016

Case Reports in Endocrinology

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“…in peripheral neuropathy and ganglionopathy in trigeminal or ataxic neuropathies have been reported as the main pathogenic etiology. The rapid and almost complete recovery from nerve palsy after therapy with corticosteroids and azathioprine suggests that lymphocytic infiltrate, rather than a vasculitic process, was the cause of cranial neuropathy in SS [33][34][35][36][37][38][39][40].…”

Section: Laryngological and Otological Manifestations Of Ssmentioning

confidence: 99%

Laryngological and Dental Manifestations of Sjögren’s Syndrome

Kamiński¹,

Błochowiak²

2019

Chronic Autoimmune Epithelitis - Sjogren's Syndrome and Other Autoimmune Diseases of the Exocrine Glands

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Sjögren's syndrome (SS) affects numerous different areas, and many specialists may be involved in the diagnosis and treatment of SS. Otolaryngological and dental manifestations, neurological impairment, and hearing loss may be the initial symptoms of SS. This chapter describes the most common otolaryngological and oral manifestations of SS, its pathomechanism and possible etiology. Dryness accompanying SS is associated with many clinical implications. The rate of dry mouth in SS ranged from 41% at initial diagnosis to 84% 10 years after diagnosis. An unstimulated salivary flow rate of 0.1 ml/min in sialometry gives a score of 1 to the weighted sum of 5 items according to the current EULAR/ACR criteria. The presence of mononuclear cell aggregates around the ducts and acini of salivary glands results in functional and structural alterations at the level of these glands and impairs their secretory function. The most common oral signs and symptoms are dental caries, tooth decay, fungal infections, traumatic oral lesions, dysphagia, dysgeusia, and inflammation of the salivary glands. Saliva in SS is characterized by the increased concentration of lactoferrin, potassium and cystatin C and the decreased concentration of amylase, carbonic anhydrase, mucins, histatines, IgA, statherins, proline-rich proteins, and the loss of salivary buffer properties. The lack of these physiological defense mechanisms increases the risk of opportunistic infections, mainly fungal infections by Candida albicans. Candidiasis accompanies angular cheilitis, simple cheilitis, and exfoliative cheilitis. The salivary glands of SS patients are characterized by chronic inflammation with the presence of lymphocytic infiltrates located around the striated ducts. These periductal foci may lead to the development of organized ectopic lymphoid structures resembling secondary lymphoid organs with segregated T-and B-cell areas, and high endothelial venules. These structures become an active center of immune response. The presence of foci in labial salivary glands is a hallmark of SS, and their histopathologic analysis is an important item in the diagnosis and classification. A biopsy can be taken from either the labial or the parotid salivary gland, but currently according to the diagnostic criteria, only labial salivary gland biopsy (LSGB) is recommended to confirm the diagnosis of SS. The authors present their own experience and recommendations in taking labial salivary gland biopsy, the main surgical approaches, and the main limitations for this diagnostic method and describe the possibilities and principles of histopathological examination in SS. The authors present the main ultrasonic signs of SS major salivary glands and perspectives of the usage of salivary gland ultrasonic examination in the diagnosis and monitoring of SS. The presented chapter also includes the most common laryngological manifestations associated with SS: nose dryness, crusting, Chronic Autoimmune Epithelitis-Sjogren's Syndrome and Other Autoimmune Diseases... 2 or atrophy of th...

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“…Also the vagus and the hypoglossal nerves were rarely mentioned in the literature and invariably associated with the IX cranial nerve [48, 51, 60, 76, 91]. All cases seem to be related to transient, often recurrent episodes of multineuritis, generally responsive to treatment.…”

Section: Review Of the Literaturementioning

confidence: 99%

Neurologic Complications Associated with Sjögren’s Disease: Case Reports and Modern Pathogenic Dilemma

Colaci

Cassone

Manfredi

et al. 2014

Case Reports in Neurological Medicine

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Objectives. Sjögren's syndrome (SS) may be complicated by some neurological manifestations, generally sensory polyneuropathy. Furthermore, involvement of cranial nerves was described as rare complications of SS. Methods. We reported 2 cases: the first one was a 40-year-old woman who developed neuritis of the left optic nerve as presenting symptom few years before the diagnosis of SS; the second was a 54-year-old woman who presented a paralysis of the right phrenic nerve 7 years after the SS onset. An exhaustive review of the literature on patients with cranial or phrenic nerve involvements was also carried out. Results. To the best of our knowledge, our second case represents the first observation of SS-associated phrenic nerve mononeuritis, while optic neuritis represents the most frequent cranial nerve involvement detectable in this connective tissue disease. Trigeminal neuropathy is also frequently reported, whereas neuritis involving the other cranial nerves is quite rare. Conclusions. Cranial nerve injury is a harmful complication of SS, even if less commonly recorded compared to peripheral neuropathy. Neurological manifestations may precede the clinical onset of SS; therefore, in patients with apparently isolated cranial nerve involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable.

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Primary Sjögren′s syndrome manifesting as multiple cranial neuropathies: MRI findings

Cited by 21 publications

References 9 publications

Bilateral Carotid-Cavernous Fistulas: An Uncommon Cause of Pituitary Enlargement and Hypopituitarism

Bilateral Carotid-Cavernous Fistulas: An Uncommon Cause of Pituitary Enlargement and Hypopituitarism

Laryngological and Dental Manifestations of Sjögren’s Syndrome

Neurologic Complications Associated with Sjögren’s Disease: Case Reports and Modern Pathogenic Dilemma

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