BACKGROUND: Wernekinck commissure syndrome (WCS) is an extremely rare midbrain syndrome, which selectively destroys the decussation of the superior cerebellar peduncle and the central tegmental tract, which commonly presents with bilateral cerebellar ataxia, dysarthria, and internuclear ophthalmoplegia. Palatal myoclonus in Wernekinck commissure syndrome is uncommon and often occurs as a late phenomenon due to hypertrophic degeneration of bilateral inferior olivary nuclei.
METHODS: A patient with WCS, admitted to our hospital from December 2023, was chosen for this study, and the syndrome\'s clinical manifestations, imaging features, and etiology were retrospectively analyzed based on the literature. A 68-year-old right-handed East Asian man presented with dizziness, slurred speech, difficulty with swallowing and walking, and rhythmic contractions of the soft palate. He had several risk factors for ischemic cerebrovascular diseases (age, sex, dyslipidemia, hypertension and smoking history). Brain magnetic resonance imaging showed hyperintensity of DWI and hypointensity of ADC at the caudal midbrain which was around the paramedian mesencephalic tegmentum anterior to the aqueduct of midbrain.
RESULTS: He was diagnosed with Wernekinck commissure syndrome (WCS) secondary to caudal paramedian midbrain infarction. He was started on dual antiplatelet therapy (aspirin and clopidogrel) and intensive statin therapy. Blood pressure and glucose were also adjusted. His symptoms improved rapidly, and he walked steadily and speak clearly after 7 days of treatment.
CONCLUSIONS: Palatal myoclonus is known to occur as a late phenomenon due to hypertrophic degeneration of bilateral inferior olivary nuclei. However, Our case suggests that palatal myoclonus can occur in the early stages in WCS.

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.

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.

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.
METHODS: 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.
CONCLUSIONS: 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.

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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.
METHODS: 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.
CONCLUSIONS: 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.

暂无摘要。

OBJECTIVE: To present cases of atypical palatal tremor (PT) and showcase the variable phenomenology of this condition.
METHODS: 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.
CONCLUSIONS: 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.

暂无摘要。

UNASSIGNED: 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.
UNASSIGNED: 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].
UNASSIGNED: An awareness of this complication is of importance to all clinical neuroscience to prevent misdiagnosis with the occurrence of new symptoms.