Mioclonía palatina esencial. Reporte de un caso pediátrico / Essential palatal myoclonus. A pediatric case report

La mioclonía palatina esencial es una entidad otoneurológi-ca rara. Se caracteriza por movimientos involuntarios de los músculos del paladar blando que causan un tinnitus objetivo.La mioclonía del paladar se clasifica en dos tipos: secundaria y primaria (mioclonía palatina esencial); esta última es más frecuente en pediatría. La tomografía computada y la reso-nancia magnética de cerebro normal orientan al diagnóstico. La mioclonía palatina esencial, generalmente, se resuelve en forma espontánea.Se presenta a una paciente de 8 años de edad con un "clic" rápido en forma rítmica en su boca que cedía en forma espontánea

Essential palatal myoclonus is a rare neurological disorder characterized by involuntary movements of the soft palate musculature causing objective-clicking tinnitus. The palatal myoclonus is classified in two forms, secondary and essential palatal myoclonus. Primary (essential) palatal myoclonus is the most common type in childhood. Normal computed tomography and magnetic resonance guide the diagnosis. Spontaneous resolution usually occurs in the essential type of palatal myoclonus.In this report, we present an 8-year-old child making rhythmic, rapid clicking noises from her throat with spontaneous resolution.

Effectiveness of Combining Behavioral Treatment With Valproic Acid for Dysphagia Caused by Palatal Myoclonus in Patients With Stroke: Two Case Reports

Palatal myoclonus (PM) is a rare disease that may induce dysphagia. Since dysphagia related to PM is unique and is characterized by myoclonic movements of the involved muscles, specific treatments are needed for rehabilitation. However, no study has investigated the treatment effectiveness for this condition. Therefore, the aim of this case report was to describe the benefit of combining behavioral treatment with valproic acid administration in patients with dysphagia triggered by PM. The two cases were treated with combined treatment. The outcomes evaluated by videofluoroscopic swallowing studies before and after the treatment showed significant decreases in myoclonic movements and improved swallowing function. We conclude that the combined treatment was effective against dysphagia related to PM.

A Child of Palatal Myoclonus Tinnitus Treated with Intraoral Injection of Botulinum Toxin / 대한이비인후과학회지

Palatal myoclonus (palatal tremor) was reported to be presented with a sole symptom of objective pulsating tinnitus and could be controlled by botulinum toxin injection alone. However, there were 3 cases reported in Korea of palatal myoclonus tinnitus controlled with botulinum toxin injection through the dual administration route of the mouth and nasal cavity. We present an 11-year-old boy of essential palatal myoclonus tinnitus, which was controlled by intraoral botulinum toxin injection to the tensor veli palatini muscle alone. Intraoral injection of botulinum toxin to the anatomical location of tensor veli palatini muscle with the guidance of electromyography was effective and safe for the child of objective tinnitus caused by palatal myoclonus.

Palatal Myoclonus Associated with Orofacial Buccal Dystonia

Palatal myoclonus is a rare condition in which there are rhythmic jerky movements of the soft palate and sometimes of the other muscles innervated by the brainstem A particularly annoying symptom is a rhythmic clicking sound in the ear due to the opening and closing of the Eustachian tube. Orofacial buccal dystonia is a focal dystonia with sustained spasms of the masticatory, facial or lingual muscles. The frequent symptoms of this disease have mainly been reported to be involuntary and possibly painful jaw opening, closing, deflecting and retruding, or a combination of the above. However, the subtle and unnoticeable involuntary movement of multiple facial muscles, which might be an infrequent symptom of orofacial buccal dystonia, makes this disease hard to diagnose. Understanding the functional orofacial anatomy that is responsible for the clinical signs and symptoms is necessary for making a proper diagnosis. Here we report on a rare case of palatal myoclonus that was associated with orofacial buccal dystonia, and such a case has not been previously reported. We describe the diagnostic approach and excellent treatment results after Botulinum toxin A (Dysport) injection and proper counseling.

One Case of Objective Tinnitus due to Palatal and Middle Ear Myoclonus / 대한이비인후과학회지

Tinnitus is very frequent and internal noise that impaires the quality of life. Objective tinnitus, which may be caused by palatal and middle ear myoclonus, is an extremely rare disorder. Such tinnitus may present very rapid, irregular and involuntary vibratory sound. Because it is a very rare disorder and patients have difficulty expressing their symptoms of tinnitus, the examiner may miss the diagnosis unless a careful history taking and physical examination is done. We have experienced a patient with objective tinnitus caused by both palatal myoclonus and middle ear myoclonus, who responded poorly to medical therapy and injection of botulinum toxin. However, this patient is treated with pterygoid hamulus fracture and sectioning of the tensor tympani and stapedial tendons. We report a review of the literature and present a case of a 26-year old man who did not respond to medical treatment and had to be relieved of the symptoms with surgical intervention.

Hemiplegia and Palatal Myoclonus after Hypertrophic Olivary Degeneration: A case report

Hypertrophic olivary degeneration (HOD) is caused by the damage of dentator rubroolivary pathway which triangular area is consisted of red nucleus, dentate nucleus, inferior olivary nucleus and the connection fibers. It usually resulted from vascular damage in pons or cerebellum, and also fromencephalitis, degenerative disease, multiple sclerosis, tumor and trauma. We experienced this case of a 56-year-old man with characteristic clinical findings of HOD such as palatal myoclonus, ipsilesional facial palsy, crossed hemiplegia and truncal ataxia after right pons and midbrainhemorrhage. Therefore we report this case with observation of olivary nucleus size in brain MRI followed up after 5 years.

A Case of Palatal Myoclonus Tinnitus Treated with Botolinum Toxin Injection / 대한이비인후과학회지

Palatal myoclonus, a kind of objective tinntius, is an uncommon disorder which shows rhythmic involuntary movement of the muscles of soft palate. Because of inadequate response of the muscles involved in palatal myoclonus to medical therapy, injection of botulinum toxin has been considered. We report a case of palatal myoclonus which had not responded to medical therapy. Injection of botulinum toxin into the muscle under the electromyographic guidance showed complete recovery of tinnitus and no more rhythmic movements of the muscles. Electromyography findings before and after botulinum toxin injection were also demonstrated.

MR demonstration of cryptic vascular malformation producing a palatal myoclonus: a case report

A 47-year-old man had suffered oscillopsia associated with palatal myoclonus for 10 years. High-field magnetic resonance imaging (MRI) revealed a cryptic vascular malformation within the "Guillain-Mollaret triangle" which was thought to be the responsible lesion.