A Novel Presentation of the Acute Airway: Anti-IgLON5 Disease.

Anti-IgLON5 disease is a newly discovered novel sleep disorder at the crossroads of neurology and immunology. In addition to the underlying sleep disorder, anti-IgLON5 manifests with progressive aerodigestive symptoms such as dysphagia, stridor, and vocal cord paresis in 90% cases and may present to the otolaryngologist. Herein we present a case of a patient with anti-IgLON5 disease who presented to the hospital with an acute airway including marked stridor and respiratory failure requiring intubation and subsequently a tracheostomy. Laryngoscope, 131:E724-E726, 2021.

Indicaciones no tradicionales de amigdalectomía: [revisión] / Nontraditional indications for tonsillectomy: [review]

La amigdalectomía es la intervención quirúrgica más frecuente en otorrinolaringología y sus indicaciones son ampliamente conocidas en la especialidad. El avance en el conocimiento de sus funciones inmunológicas ha determinado su uso en el tratamiento de otras enfermedades tales como la nefropatía por IgA, síndromes neuropsiquiátricos, el síndrome de fiebre recurrente, algunas entidades dermatológicas, entre otras. Pretendemos así, revisar la literatura para evaluar la evidencia disponible que sustente lo que denominamos indicaciones no tradicionales. Al parecer, la evidencia a favor del uso de amigdalectomía en el tratamiento de algunas de estas enfermedades es sólido, mientras que para otras aún no supera la suposición teórica, pues sólo se cuenta con casos aislados. Consideramos necesario estudios más extensos, con mayor calidad metodológica para definir mejor la indicación de amigdalectomía. Esto, más la evaluación multidisciplinaria de cada caso nos debiera conducirá la mejor decisión.

Tonsillectomy is the most common surgical procedure in otorhinolaryngology and their indications are well known in the field. The advance in knowledge of their immune function has determined its use in the treatment of other diseases such as IgA nephropathy neuropsychiatric syndromes, periodic fever syndrome, some dermatologic entities, and others. We review the literature to evaluate the available evidence that supports what we cali nontraditional indications. Apparently the evidence for the use of tonsillectomy in the treatment of some diseases is solid, while others still not overcome the theoretical assumption, since there are only isolated cases. Larger studies are needed to consider, with higher methodological quality, to better define the indication for tonsillectomy. This and the multidisciplinary assessment of each case should lead us to the best decision.

A case of PANDAS treated with tetrabenazine and tonsillectomy.

PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) is a rare clinical syndrome characterized by the presence of tics, Tourette syndrome, obsessive-compulsive disorder, or chorea in the context of an immediately precedent streptococcal infection. In this report, we describe the case of an 11-year-old boy who developed PANDAS with severe choreic movements. The criteria for PANDAS diagnosis were met. Moreover, serum antibrain antibodies were present. The patient was initially treated with tetrabenazine 12.5 mg twice daily with remission of the neurological symptoms. Subsequently, the patient underwent tonsillectomy and has been asymptomatic since, with antistreptolysin O titer levels in range.

Gamma knife surgery for lymphocytic hypophysitis.

Lymphocytic hypophysitis is a relatively uncommon autoimmune inflammatory disorder affecting the pituitary gland. It most frequently occurs in women of child-bearing age. The authors report on their experience with a patient who presented with diplopia and marked enlargement of the pituitary gland. She underwent transsphenoidal surgery, and histopathological analysis confirmed the diagnosis of lymphocytic hypophysitis. The disease proved refractory to resection, and any attempt at withdrawal of corticosteroid therapy resulted in a return of the patient's symptoms and enlargement of the sellar contents. The patient underwent Gamma Knife surgery (GKS) to the sella and both cavernous sinuses. After GKS, the patient was able to discontinue steroid therapy without return of her symptoms. Follow-up MR images demonstrated no evidence of recurrence of lymphocytic hypophysitis. For persistent lymphocytic hypophysitis, GKS is a reasonable treatment option.

Rasmussen's syndrome: progressive autoimmune multi-focal encephalopathy.

Rasmussen's encephalitis, originally thought to be a chronic form of viral encephalitis, is now thought to be an autoimmune disease of the brain and is more properly termed Rasmussen's syndrome. Starting in one area of one side of the brain, the disease appears to gradually and progressively involve that side of the brain causing progressive and intractable focal seizures, a hemiparesis, and expressive aphasia when the left hemisphere is involved. Immune therapy with steroids, immunoglobulins, or plasmaphoresis provide only temporary relief from seizures. Neither antibodies to Glu-R3 nor cortical biopsy are helpful in the diagnosis. Hemispherectomy of one form or another is the only curative therapy, and there is no evidence that one form of hemispherectomy is preferable to another. Immuno-ablative therapy may be a therapy of the future.

PANDAS: pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections--an uncommon, but important indication for tonsillectomy.

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, also know as "PANDAS," is well described in the neurologic and psychiatric literature. PANDAS is associated with obsessive compulsive disorders (OCD) and tic disorders. The streptococcal infections may trigger an autoimmune reaction that exacerbates these conditions. Recurrent streptococcal tonsillitis is one of the recurrent infections associated with PANDAS. This paper reviews the case reports of two brothers, one with OCD and the other with a tic disorder, both of whom improved significantly after undergoing adenotonsillectomy for treatment of their recurrent tonsillitis. A review of the pathophysiology and current understanding of PANDAS is presented.

Cochlear implants in systemic autoimmune vasculitis syndromes.

The concept that autoimmunity may damage the inner ear was introduced by McCabe in 1979. Audiovestibular symptoms may occur in isolation or may be mediated by vasculitis in patients affected by systemic autoimmune disorders. Sensorineural hearing loss (SNHL) is typical in Cogan's syndrome but occurs less frequently in Beçhet's syndrome and in systemic necrotizing vasculitides. Patients affected by immune-mediated profound SNHL represent ideal candidates for cochlear implantation as these patients become deaf after years of hearing. The disease itself and the medication taken may, however, influence the prognosis of cochlear implantation in these patients. We retrospectively evaluated the pre- and intraoperative findings as well as the postoperative course and performance of a group of five patients affected by a systemic vasculitis syndrome who received a cochlear implant. Implantation was successful in all patients, no complications occurred and excellent postoperative speech perception was achieved. We conclude that cochlear implantation in patients affected by immune-mediated inner ear disorders is effective although the long-term results remain to be evaluated.