Hypertrophic olivary degeneration secondary to a Guillain Mollaret triangle cavernoma: Two case report.

Hypertrophic olivary degeneration (HOD) is a rare form of transsynaptic degeneration. It is caused by a damage at the Guillain-Mollaret triangle (GMT), which is defined by three anatomical structures: the dentate nucleus, the red nucleus, and the inferior olivary nucleus (ION). Clinically, it may be revealed by palatal myoclonus. On MRI, it appears as a unilateral or bilateral enlargement of the inferior olivary nucleus which shows a high signal intensity on T2-weighted images, with sometimes a cerebellar atrophy. Here we report 2 cases of healthy patients which present hemorrhagic brainstem cavernomas, complicated later by the development of palatal myoclonus and cerebellar ataxia, with MRI features corresponding to an (HOD) secondary to a (GMT) cavernoma. The purpose is to explain the mechanism of (HOD) subsequent to lesion in (GMT), and to describe magnetic resonance imaging features.

Essential Palatal Myoclonus and Clicking Tinnitus in a Nine-Year-Old Boy-A Case Report.

The work describes a case of palatal myoclonus with distressing tinnitus in a 9-year-old boy and its successful treatment with injections of botulinum toxin. This case report discusses common questions about myoclonic-induced clicking tinnitus and provides answers. Laryngoscope, 134:397-399, 2024.

Palatal myoclonus and hypertrophic olivary degeneration following wernekinck commissure syndrome: a case report.

BACKGROUND: Hypertrophic olivary degeneration (HOD), a rare form of transsynaptic degeneration, is secondary to dentato-rubro-olivary pathway injuries in some cases. We describe a unique case of an HOD patient who presented with palatal myoclonus secondary to Wernekinck commissure syndrome caused by a rare bilateral "heart-shaped" infarct lesion in the midbrain. CASE PRESENTATION: A 49-year-old man presented with progressive gait instability in the past 7 months. The patient had a history of posterior circulation ischemic stroke presenting with diplopia, slurred speech, and difficulty in swallowing and walking 3 years prior to admission. The symptoms improved after treatment. The feeling of imbalance appeared and was aggravated gradually in the past 7 months. Neurological examination demonstrated dysarthria, horizontal nystagmus, bilateral cerebellar ataxia, and 2-3 Hz rhythmic contractions of the soft palate and upper larynx. Magnetic resonance imaging (MRI) of the brain performed 3 years prior to this admission showed an acute midline lesion in the midbrain exhibiting a remarkable "heart appearance" on diffusion weighted imaging. MRI after this admission revealed T2 and FLAIR hyperintensity with hypertrophy of the bilateral inferior olivary nucleus. We considered a diagnosis of HOD resulting from a midbrain heart-shaped infarction, which caused Wernekinck commissure syndrome 3 years prior to admission and later HOD. Adamantanamine and B vitamins were administered for neurotrophic treatment. Rehabilitation training was also performed. One year later, the symptoms of this patient were neither improved nor aggravated. CONCLUSION: This case report suggests that patients with a history of midbrain injury, especially Wernekinck commissure injury, should be alert to the possibility of delayed bilateral HOD when new symptoms occur or original symptoms are aggravated.

Hypertrophic olivary degeneration and palatal myoclonus from a Streptococcus intermedius infection of the brain: illustrative case.

BACKGROUND: Hypertrophic olivary degeneration (HOD) is a rare condition that can occur after disruption of the Guillain-Mollaret triangle. Clinically, HOD can present with palatal myoclonus with or without oculopalatal tremor, which sometimes results in symptomatic dysphagia and/or speech abnormalities. This condition is commonly associated with vascular lesions, with only three prior reported cases of HOD resulting from intracranial abscess. OBSERVATIONS: An otherwise healthy patient developed multiple intracranial abscesses. Biopsy showed gram-positive cocci; however, culture findings were negative. Polymerase chain reaction (PCR) identified Streptococcus intermedius. The patient demonstrated palatal myoclonus and vertical nystagmus, which resulted in persistent mild dysphagia and altered speech intonation. After appropriate antimicrobial therapy with resolution of the enhancing lesions, symptoms persisted. Follow-up imaging demonstrated progressive hypertrophy of the right olive with persistent disruption of the right-sided rubro-olivo fiber pathways. LESSONS: Although HOD classically occurs after vascular insult, it can also be seen as a postinfectious sequela. Despite eradication of the infection, palatal myoclonus and oculopalatal tremor may have a persistent impact on quality of life due to impaired speech and swallowing. This case emphasizes the utility of universal PCR in detecting fastidious organisms as well as diffusion tensor imaging for characterization of disrupted fiber pathways.

Laryngeal, Pharyngeal and Respiratory Involvement in Palatal Tremor.

OBJECTIVE: To present cases of atypical palatal tremor (PT) and showcase the variable phenomenology of this condition. STUDY DESIGN: Retrospective case series. RESULTS: PT, or palatal myoclonus, is a movement disorder characterized by brief, involuntary rhythmic muscular contractions of the soft palate. Variants of PT have been described and include synchronous tremors in other branchial arch derivatives including the larynx, pharynx, neck, face, jaw, ocular and also respiratory and trunk muscles. We present 3 cases, including clinical videos, of atypical PT with extra-palatal manifestations, in addition to a brief discussion of the pathophysiology and management of this condition. CONCLUSION: Variations of PT are of interest to the practicing otolaryngologist as the clinical spectrum of this condition is wide and can present with laryngeal, pharyngeal, respiratory and other head and neck manifestations.

Mollaret's triangle: An important neuroanatomical territory for all clinicians.

BACKGROUND: Hypertrophic olivary degeneration is a rare condition caused by damage within the triangle of Guillain and Mollaret. We discuss the anatomical, radiological, and clinical history of this rare condition. CASE DESCRIPTION: A 32-year-old lady presented with sub-acute headache, photophobia, and dizziness. She also described facial tingling and itching over her nose, and a thirty-minute episode of slurred speech. Magnetic resonance imaging revealed a 12.1 × 11 × 7.3 mm lesion arising from the floor of the fourth ventricle [Figure 1]. Postoperative imaging confirmed complete resection of the tumor, but changes consistent with hypertrophic olivary degeneration [Figure 2a and b]. CONCLUSION: An awareness of this complication is of importance to all clinical neuroscience to prevent misdiagnosis with the occurrence of new symptoms.

Essential Palatal Tremor Following an Upper Respiratory Tract Infection: A Case Report.

Bilateral objective tinnitus is a rare accompanying manifestation of an underlying palatal tremor (PT). PT can either be secondary to a lesion in the triangle of Guillain and Mollaret, or it can present without a causal organic lesion. In this report, we present an unusual case of a young female with objective tinnitus revealing a PT.

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.

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

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.

La mioclonía palatina esencial es una entidad otoneurológica 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 resonancia 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.

Isolated Ear Clicks with Partial Voluntary Control.

BACKGROUND: Ear click is a rare type of objective tinnitus, classically described with associated palatal tremor/myoclonus (PT). CASE REPORT: A 15-year-old boy reported a constant bilateral ear clicking for 4 years, that could be stopped at will for a few seconds. Clinically, the ear clicks were audible without visible eardrum or palatal movement, and could be entrained by the examiner. Brain MRI was normal. DISCUSSION: We propose to classify this as isolated ear clicks with partial voluntary control, putting it into context with other subcategories of "essential" or "isolated" PT.

Objective bilateral tinnitus from palatal nystagmus. Audio and video features of a rare case of palatal myoclonus.

Tinnitus is one of the most represented otological symptom, affecting 15% of adults, worldwide. Literature describes subjective tinnitus when it's perceived by the patient only, and objective tinnitus when it's heard both, by patient and examiner. An objective tinnitus can be caused by a large variety of anomalies and diseases; one of them is Palatal Myoclonus, characterized by rhytmic movements of soft palatal muscles and, only occasionally, involving other near districts. Case presentation. We observed a rare case of essential palatal myoclonus in a 54 y.o. female, suffering from chronic objective bilateral tinnitus, since 35 years, who underwent a wide number of clinical evaluations over the years, without receiving any conclusive diagnosis. In this video, we illustrate all the districts involved in clonic movements: soft palate, larynx and nasal wings. At the same time, we report the spectrographic analysis of tinnitus, recorded in esternal ear canal, taken together with the muscle movements. Palatal Myoclonus has to be considered in the etiological diagnosis of each objective tinnitus and should always be investigated properly.

Electrophysiological Evidence for Functional (Psychogenic) Essential Palatal Tremor.

Background: There is little published work describing the electrophysiological characteristics of essential palatal tremor, a condition now believed by many to be a functional (psychogenic) movement disorder. Case Report: Here we combine electroencephalography and electromyography with time-locked video recordings to document two cases of essential palatal tremor in which a definitive diagnosis is achieved using these electrophysiological tools. Discussion: We believe that sharing how these objective tools can be used to diagnose a functional movement disorder, as well as providing more published evidence to support the functional origin of essential palatal myoclonus, will help to diagnose this condition in the future.

Palato-pharyngo-laryngeal myoclonus with recurrent retrograde feeding tube migration after cerebellar hemorrhagic stroke: a case report and review of hypertrophic olivary degeneration.

BACKGROUND: Palato-pharyngo-laryngeal myoclonus, a variant of palatal myoclonus, is characterized by involuntary rhythmic movements of palatal, pharyngeal, and laryngeal muscles. Symptomatic palatal myoclonus is classically associated with hypertrophic olivary degeneration on MRI imaging due to a lesion in the triangle of Guillain-Mollaret. CASE PRESENTATION: We report a case of palato-pharyngo-laryngeal myoclonus in a patient post-cerebellar hemorrhagic stroke who presented with recurrent retrograde migration of his gastrojejunostomy feeding tubes. Treatment with either divalproex sodium or gabapentin resulted in a significant decrease in his gastrointestinal symptoms and no further episodes of gastrojejunostomy tube migration. CONCLUSIONS: This case study indicates that the movement disorder associated with hypertrophic olivary degeneration may involve the gastrointestinal system. Anticonvulsants, such as gabapentin and divalproex sodium, may reduce the severity of gastrointestinal symptoms in cases associated with hypertrophic olivary degeneration. The anatomy of the Guillain-Mollaret triangle and the pathophysiology of hypertrophic olivary degeneration are reviewed.