Ocular Myoclonus Research Articles

Ocular Flutter, Ataxia, and Myoclonus Associated With CSF Pleocytosis and GAD65 Antibodies Years After Curative Treatment of Follicular Thyroid Carcinoma: A Case Report and Literature Review.

Ocular Flutter, Ataxia, and Myoclonus Associated With CSF Pleocytosis and GAD65 Antibodies Years After Curative Treatment of Follicular Thyroid Carcinoma: A Case Report and Literature Review.

An interesting case of ocular flutter, myoclonus, and ataxia in the postpartum period

Abstract Opsoclonus–myoclonus–ataxia syndrome is a well-established neurologic syndrome with paraneoplastic, postinfectious, immune-mediated, or idiopathic etiology. Ocular flutter and opsoclonus are in the same continuum with a common pathophysiology; however, their phenomenology is distinct. While eye movements in ocular flutter are strictly confined to the horizontal plane, those in opsoclonus are multidirectional. There are few case reports describing the combination of ocular flutter, myoclonus, and ataxia. In this case report, we describe a woman with the abovementioned features in the postpartum period with dramatic response to immunotherapy.

395 - Serotonin syndrome with ocular myoclonus and inducible clonus: an uncommon clinical presentation

395 - Serotonin syndrome with ocular myoclonus and inducible clonus: an uncommon clinical presentation

A Case of Ocular Myoclonus Appearing After Pontine Hemorrhage

A Case of Ocular Myoclonus Appearing After Pontine Hemorrhage

Opsoclonus Myoclonus Syndrome and Supraventricular Tachycardia in a Pediatric Patient: A Case Report and Literature Review

Objective NA. Background Opsoclonus myoclonus syndrome (OMS) is a rare movement disorder in children often associated with an underlying neuroblastoma. In other cases, it is believed that the tumor is occult or there is another immune-stimulating precipitating event. Diagnosis can be difficult, requiring 3 of 4 criteria: opsoclonus or ocular flutter, myoclonus or ataxia, behavioral or sleep disturbances, and neuroblastoma. Prompt treatment of OMS is crucial to preventing permanent neurologic sequelae. In order to better characterize the clinical profile of this syndrome and its associated conditions, we present a case report of a 9 month old male with OMAS without an associated neuroblastoma and with new onset supraventricular tachycardia (SVT) and review the associated literature. Design/Methods NA. Results A 9-month-old male with a past medical history of macrocephaly, hypotonia, and developmental delay presented with abnormal eye and body movements starting one month prior. Due to concern for OMS, the patient underwent an extensive initial workup including CT neck, chest, abdomen, and pelvis and VHA/HMA levels, which were all within normal limits. The patient's spinal fluid revealed no evidence of paraneoplastic, autoimmune, or infectious processes. Per neurology recommendations, the patient was started on IVIG and dexamethasone. After his first IVIG infusion, the patient's abnormal movements worsened, and his heart rate increased into the 300s. The IVIG was discontinued. However, the patient continued to have recurring bouts of SVT, which was eventually controlled with digoxin. After resuming treatment with IVIG and dexamethasone, the patient's opsoclonus myoclonus symptoms began to improve. All additional metabolic labs resulted normal and the patient was discharged. Conclusions Because OMS can cause permanent developmental delay, prompt recognition and treatment of this syndrome is necessary. This is a unique case of OMAS without neuroblastoma, associated with recurrent bouts of SVT. Recognizing rare complications of OMS is crucial to improving medical management of its sequelae.

Successfull treatment of paroxysmal painful tonic spasms with carbamazepine in a patient with hypertrophic olivary degenaration

Hypertrophic olivary degeneration (HOD) is a rare phenomenon that can occur after different insults to the so-called Guillain-Mollaret triangle. Clinically, HOD presents as palatal tremor, dentatorubral tremor and/or ocular myoclonus.

Primary intracranial germ cell tumour originating from right brachium Pontis with hypertrophic Olivary degeneration: a case report

BackgroundPrimary right brachium pontis germinoma with hypertrophic olivary degeneration (HOD) is extremely rare. A preoperative diagnosis is challenging due to the absence of characterized clinical and neuroimaging features, and biopsy should be considered.Case presentationA 20-year-old male patient presented with a case of primary intracranial germinoma originating from right brachium pontis with HOD manifesting as ocular myoclonus, nystagmus in both eyes, ataxic gait and incoordination of the limbs. Magnetic resonance imaging (MRI) revealed an irregular patchy lesion with hyperintensity on T2-weighted images (T2WI) and T2 fluid-attenuated inversion recovery (FLAIR) without enhancement by gadolinium (Gd). Furthermore, a focal hyperintense nodule on T2WI in the left inferior olive nucleus (ION) of the medulla oblongata was considered hypertrophic olivary degeneration (HOD) based on the patient’s symptoms and neuroimaging findings. Due to suspected demyelinating disease and low-grade glioma (LGG), a biopsy was planned. The pathological diagnosis was germinoma. Subsequently, he received chemoradiation therapy, resulting in the improvement of neurological deficits and the disappearance of the lesion on MRI.ConclusionA case of “Primary right brachium pontis germinoma with HOD” is reported for the first time. A preoperative diagnosis is challenging due to the fact of absence of clinical signs and symptoms and neuroimaging characteristics. However, patients can have favourable prognoses with appropriate evaluation and treatment.

Bilateral Hypertrophic Olivary Degeneration Following Brainstem Insult: A Retrospective Review and Examination of Causative Pathology

Hypertrophic olivary degeneration is a rare condition caused by a lesion in the Guillain-Mollaret triangle which leads to trans-synaptic degeneration resulting in the degenerative hypertrophy of the inferior olivary nucleus. This condition presents clinically with palatal tremor but can also produce ocular myoclonus or cerebellar signs. While any lesion that occurs within the Guillian-Mollaret triangle and results in the deafferentation of the inferior olive can lead to hypertrophic olivary degeneration, the most common etiologies include ischemic and hemorrhagic stroke, vascular malformation, neoplasm, and iatrogenic injury related to surgery. We report a series of 7 patients who presented with this condition bilaterally on MRI imaging, including 1 case which represents the first report of toxoplasmosis leading to the development of bilateral hypertrophic olivary degeneration and only the third reported case, unilateral or bilateral, related to an infectious etiology.

Ocular flutter and myoclonus in hyperosmolar hyperglycemic state

Ocular flutter and myoclonus in hyperosmolar hyperglycemic state

Rhombencephalitis secondary to toxoplasma gondii infection: Case report and literature review

Immunocompetent (IC) individuals with primary toxoplasmosis are usually asymptomatic. However, in some IC, toxoplasmosis can present with brainstem encephalitis. We report an adult female patient with headache associated with worsening coordination. Physical examination showed bilateral, symmetrical, and nontender cervical lymphadenopathy. Toxoplasmosis immunoglobulin G (IgG) and IgM were high, and the IgG antibody avidity pattern was low. The cerebrospinal fluid analysis was requested that revealed a positive polymerase chain reaction results for toxoplasmosis. A brain magnetic resonance imaging (MRI) showed a T1-hypointense left pontine lesion. Pyrimethamine and sulfadiazine were started. After 1 month, the patient had full recovery. Four months after, the individual was admitted due to progressive abnormal eye movements and tremor. Her neurological examinations showed ocular myoclonus and Holmes tremor. A second brain MRI showed hypertrophy of the ipsilateral inferior olivary nucleus. In the follow-up, her symptoms decreased mildly. The tremor was managed with clonazepam and levodopa. Furthermore, immune suppression investigation was negative.

Hypertrophic olivary degeneration: A comprehensive review focusing on etiology

Hypertrophic olivary degeneration (HOD) is a rare form of trans-synaptic degeneration characterized by hypertrophy of the inferior olivary nucleus situated in the olivary body, part of the medulla oblongata, representing a major source of input to the cerebellum. HOD typically results from focal lesions interrupting connections from the inferior olive within the dentato-rubro-olivary pathway (DROP), a region also known as the Guillain-Mollaret triangle (GMT) (red nucleus, inferior olivary nucleus, and contralateral dentate nucleus). Clinically, HOD presents classically as palatal tremor and can include dentatorubral tremor and/or ocular myoclonus. Oppenheim first described the enlargement of the ION in his post-mortem study. Since then, a limited number of cases have been reported in the literatures. Thus, we intended to describe the definition, pathophysiology, clinical features, radiological features, diagnosis, and current management of HOD. We provide a comprehensive review of HOD focusing on etiology. The present review may lead to a better understanding of HOD clinical characteristic with the goal of contributing to existing knowledge of this rare disease.

Hypertrophic Olivary Degeneration: A Neurosurgical Point of View

Hypertrophic olivary degeneration (HOD) is a rare form of transsynaptic degeneration characterized by hypertrophy of the inferior olivary nucleus situated in the olivary body, part of the medulla oblongata, representing a major source of input to the cerebellum. HOD typically results from focal lesions interrupting connections from the inferior olive within the dentato-rubro-olivary pathway, a region also known as the triangle of Guillain-Mollaret (TGM) (red nucleus, inferior olivary nucleus, and contralateral dentate nucleus). Clinically, HOD presents classically as palatal tremor and can include dentatorubral tremor and/or ocular myoclonus. The pathologic changes associated with HOD feature radiologic changes with the inferior olivary nucleus appearing larger and increasing its T2-weighted signal intensity on magnetic resonance images. HOD is commonly managed with pharmacotherapy but may require surgical intervention in extreme cases. HOD has been found to develop as a consequence of any injury that disrupts the TGM pathways (e.g., pontine cavernoma).These findings highlight the critical importance of a thorough knowledge of TGM anatomy to avoid secondary HOD. We present a patient who developed HOD secondary to resection of a tectal plate cavernous malformation and review the literature with an emphasis on the current knowledge of this disorder.

Adult-onset opsoclonus-myoclonus-ataxia syndrome: clinical features and diagnostic findings

Background: Opsoclonus-myoclonus-ataxia (OMA) is a rare neurological disorder characterized by the presence of abnormal saccadic ocular movements, ataxia and myoclonus. OMA is an autoimmune disease probably caused by a dysfunction in Purkinje cells secondary to an altered humoral and cellular response. The most frequent etiologies in adults are paraneoplastic, idiopathic and parainfectious. Objective: to describe two cases of opsoclonus-mioclonus-ataxia. Patients and methods: We reviewed the clinical history and diagnostic tests of two patients from December 2012 to April 2015. Case one: 32 year/old woman who, a week after a gastroenteritis, started suddenly with opsoclonus, ataxia and generalized myoclonic jerks. Case two: 60 year/old man who, after flu-like syndrome, started suddenly with generalized myoclonus, ataxia and opsoclonus. Results: Both patients were studied in the same way. In table one we show the results of diagnostic tests performed.Tabled 1Case 1Case 2Brain MRINormalNormalEBV, VZV, HSV PCRNegativeNegativeHIV serologyNegativeNegativeAnti-Hu, anti-Yo, anti-Ri antibodiesNegativeNegativeRoutine laboratory test and CSF analysisNormalNormalWhole-Body FDG-PET scanRight cortical hypometabolismLeft cortical hypometabolism Open table in a new tab They were diagnosed as idiopathic OMA, received steroid therapy and had a good outcome. Conclusion: As in the patients we describe, an important number of idiopathic cases are preceded by infectious symptoms without confirmation of an infectious cause. Even though both patients presented typical features of idiopathic OMA, the result of both PET scans showed cortical unilateral cortical hypometabolism without cerebellar involvement, which is not usual in this syndrome.

Metronidazole‐induced encephalopathy and myoclonus: Case report and a review of the literature

AbstractWe describe a 36‐year‐old man with metronidazole‐induced encephalopathy in the course of alcoholic liver cirrhosis who developed periodic myoclonus on the head and fixed eye position with ocular myoclonus in the vertical direction. Involuntary movements are rare in patients with metronidazole‐induced encephalopathy, because neurological manifestations of metronidazole‐induced encephalopathy usually include ataxic gait, dysarthria and confusion. Involuntary movements including myoclonus should be considered as the clinical manifestation of metronidazole‐induced encephalopathy. In addition, we review the literature of metronidazole‐induced encephalopathy patients who developed myoclonus with regard to the clinical characteristics.

Gait and bilateral limb ataxia as isolated feature of a lower midbrain tegmental infarction

Sirs: The clinical picture of midbrain infarction is dominated by third-nerve palsy, conjugate or disconjugate gaze impairment, contralateral motor and sensory deficit and homolateral or contralateral limb ataxia [1–3]. Among various mesencephalic syndromes of vascular origin, those related to the lower midbrain lesion are rare [2, 3]. We describe a patient with an isolated lower tegmental midbrain infarction, with gait and bilateral limb ataxia as isolated features. A 71-year-old man complained of dizziness, imbalance, slight hypersomnolence, and bilateral clumsiness of arms and legs. There was a worsening of symptoms and 12 hours later at admission neurological examination revealed slight hypersomnolence, dysartric speech and pronounced gait and bilateral limb ataxia. The NIHSS score was four. The patient walked with a wide-based, staggering gait without tendency to deviate to one side. The stance was on a broad base with impossibility standing with their feet together or in tandem position. Romberg sign was absent. At the finger-to-nose and at the heel-to-shine tests important dysmetria was present. The movement path of the limbs was erratic with relevant hypermetria. Rapid alternating movements were irregular bilaterally. The rest of the neurological examination was normal. One year before he suffered from an anterior circulation infarction involving the frontal branches of the right middle cerebral artery, without any residual deficit. Regarding conventional vascular risk factors, the patient suffered from hypertension and non-valvular atrial fibrillation. He was taking oral anticoagulants, with INR value ranging between 2 and 3. Non contrast cranial CT showed only the previous right frontal infarction. Five days later brain MRI revealed a small ischemic lesion in the middle tegmental portion of the lower midbrain (Fig 1 A–C); multiple silent small lacunar infarctions were present in the white matter bilaterally. Extracranial duplex ultrasonography and MR-angiography of carotid, vertebral and basilar arteries were normal. Transthoracic and transoesophageal echocardiography failed in revealing intra-cardiac thrombi. Eight-months later gait and bilateral limb ataxia and dysartria were the isolated and persistent neurological deficits. Palatal myoclonus was absent and the modified Rankin Scale was 3. Our patient presented an infarction in the middle portion of the midbrain tegmentum, with gait and bilateral cerebellar limb ataxia as dominant and persistent symptoms. The vascularization of the midbrain regions is complex, and the region close to the midline is supplied by the paramedian branches from the basilar artery (Fig 1D). Generally, in midbrain infarction the cerebellar ataxia is overshadowed by upper-midbrain and thalamic-subthalamic signs [1–2]. A tetra-ataxia has been reported in patients with midbrain infarction [1–6] but in none of them it was the isolated feature. In these cases the bilateral ataxia was associated to other neurological deficits, such as hemiparesis, dysphagia, dysphonia, eye-movement disorders or palatal myoclonus. Moreover, a bilateral ataxia was the sole manifestation of a rostral pontine tegmental infarction [7] and in patients with heat stroke [8]. In our patient the clinical picture may be considered a partial form of the Wernekink commissure syndrome where bilateral ataxia is associated to ocular abnormalities and palatal myoclonus. Indeed oculomotor deficit and delayed palatal myoclonus were absent and cerebellar signs were isolated except for the transient hypersomnolence related to the involvement of the reticular formation located dorsally to the superior cerebellar peduncles Supported by Piedmont Region, Ricerea Scientifica Applicata 2004.

Inferior Olivary Hypertrophy Is Associated with a Lower Functional State after Pontine Hemorrhage

Background: Inferior olivary hypertrophy (IOH) may develop after pontine hemorrhage and may become a pacemaker for symptomatic palatal tremor (SPT). However, there is no information available that elucidates how IOH may affect the functional outcome. The purpose of this study was to investigate how frequently IOH was associated with clinical manifestations of involuntary movements, including ocular myoclonus (OM) and SPT, and whether IOH influenced the functional outcome after pontine hemorrhage. Methods: In 20 consecutive patients undergoing inpatient multidisciplinary rehabilitation after pontine hemorrhage, the location of lesions (tegmental vs. ventral) and the presence of IOH were examined by magnetic resonance imaging, and the functional outcome was assessed by means of Fugl-Meyer scale for neurological impairment and Functional Independence Measure for disability on admission and discharge. Results: In 10 patients, IOH was detected, and the tegmentum was involved in 7 of the 10 patients. OM or SPT was present in 7 of these 10 patients. In the remaining 10 patients, IOH was not detected, and the tegmentum was involved only in 2 of these 10 patients. None of them had OM or SPT. The presence of IOH was associated with a lower functional status on admission and discharge. Conclusion: After pontine hemorrhage, IOH may be associated with tegmental lesions with OM or SPT and may impose a detrimental effect on the functional outcome.

Glucose utilization in the inferior cerebellar vermis and ocular myoclonus

In a patient with symptomatic ocular myoclonus, the authors observed the regional cerebral metabolic rate of glucose use (rCMRGlu) before and after successful treatment with clonazepam. Even after the symptoms resolved, the rCMRGlu in the hypertrophic olive increased persistently, whereas that in the inferior cerebellar vermis contralateral to the hypertrophic olive decreased. The inferior cerebellar vermis, belonging to the vestibulocerebellar system, may be associated with the generation of symptomatic ocular myoclonus.

July 11 Highlight and Commentary

Yakushiji et al. studied FDG-PET in a patient with symptomatic ocular myoclonus before and after successful treatment. The glucose utilization in the inferior cerebellar vermis decreased with the clinical improvement. see page 131 ### Commentary by R. John Leigh, MD Since Guillain and Mollaret defined the syndrome of oculopalatal myoclonus or tremor in 1931, it has remained a neurologic mystery.1 Affected patients develop spontaneous oscillations that variably affect the eyes, palate, and other branchial muscles during …

Levetiracetam Effect in Demyelinating Ocular Flutter with Myoclonus

A 29-year-old male developed ocular flutter and subtle extremity myoclonus related to demyelination. Ocular flutter and myoclonus were symptomatically amenable to treatment with levetiracetam. Discontinuation of levetiracetam was associated with recurrent flutter and myoclonus over a two-year period of follow-up. Levetiracetam may be an effective agent for the symptomatic control of ocular flutter.

右橋被蓋出血後に出現したｏｃｕｌａｒ ｍｙｏｃｌｏｎｕｓの一例

We report here a fifty-one-year old male with ocular myoclonus due to hemorrhage in the right pontine tegment. The patient complained of a continual dizzy feeling after treatment for the acute stage of cerebral hemorrhage had been completed at an other hospital. Neurootological examination revealed saccadic smooth pursuit eye movement and slightly suppressed optokinetic nystagmus but no pathological spontaneous eye movement. However, one year after the onset, oscillopsia developed and an abnormal eye movement appeared. ENG study revealed pendular-like oscillatory eye movements in both vertical and horizontal directions. The vertical component was dominant and phase difference in both directions was observed. On follow-up studies over a 4-year period, the abnormal eye movement decreased and the high intensity noted at the right dorsal region of the pons in CT at the initial stage disappeared later. Pseudohypertrophy of the right inferior olivary nucleus was suspected based on CT findings at the later stage. The abnormal eye movement was diagnosed as ocular myoclonus due to a lesion in the Guillain-Mollaret's triangle.