Tremor Caused by Dandy-Walker Syndrome Concomitant with Syringomyelia: Case Report and Review of the Literature Review

Wang, Yiqi; Guo, Shunyuan; Xu, Liang'e; Yang, Geng; Shi, Zongjie; Lei, Bing; Ma, Yuyuan; Wang, Meiping

doi:10.1016/j.wneu.2020.01.045

Cited by 4 publications

(4 citation statements)

References 11 publications

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“…[10] Adult patients with late onset of symptoms related to the malformation have also been reported; the symptoms may include neural dysfunction manifesting as tremor, sensorimotor disorders, and in cases of Dandy-Walker variants, neurocognitive disorders such as psychosis and bipolar disorder. [11][12][13] The reason for the delayed diagnosis of DWM is not clear. Some suggest that a good communication between posterior fossa cyst membrane and the surrounding basal cisterns and distal pathways may explain the prolonged asymptomatic condition; the onset of symptoms in such cases may be attributed to age-related degeneration of the communicating channels.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Lateral ventricle pleomorphic xanthoastrocytoma concurrent with Dandy-Walker complex: A case report

Luo

Wang

et al. 2022

Medicine

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Introduction: Dandy-Walker complex and pleomorphic xanthoastrocytomas are both rare disease entities that typically manifest early in life and are associated with congenital etiological factors. Dandy-Walker complex is a cerebellar malformation associated with a series of anatomical changes. The disease onset is usually at birth or during infancy. Late onset in adulthood is uncommon. Pleomorphic xanthoastrocytoma is a rare WHO grade II astrocytic tumor affecting mainly young adults. Concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma has not been previously reported.Patient concerns and diagnosis: A 30-year-old woman with a previous history of unconfirmed resected lateral ventricle meningioma presented with severe headache for 1 day. Imaging examination revealed a mass in the right lateral ventricle with heterogeneous signal patterns, changes in the posterior fossa corresponding to a Dandy-Walker variant, and mild hydrocephalus.Interventions and outcomes: Surgical complete resection of the mass was achieved. postoperative histopathological examination confirmed WHO grade II pleomorphic xanthoastrocytoma. Three years postsurgery, ventriculoperitoneal shunt was performed due to worsening of hydrocephalus. The patient has since remained symptom-free.Conclusion: This is the first report of concomitant occurrence of Dandy-Walker complex and pleomorphic xanthoastrocytoma. The association of neurological congenital malformation with intracranial neoplasms may be multifactorial, with underlying role of genetic mutations or chromosome alterations.

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Section: Discussion and Literature Reviewmentioning

confidence: 99%

Lateral ventricle pleomorphic xanthoastrocytoma concurrent with Dandy-Walker complex: A case report

Luo

Wang

et al. 2022

Medicine

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“…DWM is a rare congenital hypoplasia of the cerebellum often coexist with agenesis of corpus callosum, hydrocephalus and other intracranial malformation. [ 17 ] Hydrocephalus is common in DWM, and increased ICP lead to additional symptoms, which may manifest with intellectual disability and seizures. [ 14 ] The goal of continued treatment with regard to his DWM in the control of posterior fossa cyst and hydrocephalus required shunt placement.…”

Section: Discussionmentioning

confidence: 99%

“…Our case presented with obvious hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle before and after the surgery, which probably contributed to the disturbance of periphery and cerebellar feedback system. [ 17 ] Patients with pathological focal lesion and cortical dysplasia had lower seizure threshold. [ 18 ] Hydrocephalus can cause neuronal damage in the cortex by disturbing activity of the dopaminergic and noradrenergic neuronal system as well as to synaptogenesis, in advanced hydrocephalus, morphological deformation of cortical neurons are accompanied by variation in neurofilament immunoreactivity, since the neurofilament is the key component of the neuronal cytoskeleton which takes part in the maintenance of cytoplasmic morphology, functional and structural changes to the cytoskeleton can affect cortical neurons because of the procession of hydrocephalus, moreover, the axonal lesion in the periventricular white matter presented with the most incomplete recovery after shunt placement could attribute to the impaired cognitive function who have received shunt placement and have obviously restructured cerebral mantles.…”

Section: Discussionmentioning

confidence: 99%

Refractory psychiatric symptoms and seizure associated with Dandy-Walker syndrome: A case report and literature review

Chen¹,

Zhu²,

Zhang³

et al. 2022

Medicine

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Backgrounds: Dandy-Walker syndrome (DWS) is a group of brain malformations which occasionally accompanied by psychotic symptoms. The co-occurrence of DWS and epilepsy in children is quite rare. Case description: We reported a 14-year-old male who presented with a 8-month history of inconsistent upper limb tremor and accidental seizure. The MRI showed the typical alterations of DWS: cystic dilatation of the fourth ventricle, vermian hypoplasia, enlarged posterior fossa. He received the ventriculoperitoneal shunting (VPS) placement for hydrocephalus and had a symptom-free period for 8 days. Then he experienced a recurrence of involuntary upper limb tremor and behavior disturbance after decreasing the pressure of cerebrospinal fluid (CSF) from 150 to 130 mm Hg. After being treated with Olanzapine 10 mg/d, Clonazepam 3 mg/qn and Valproate acid (VPA) 500 mg/bid for nearly a month, his mental status and psychotic symptoms fluctuated. A search of Pub Med showed little report of hydrocephalus and DWS comorbidity with seizure and psychosis. Here we presented the whole process of a rare disease from the very beginning with all his symptoms, examinations and treatments. Conclusion: VPS placement surgery at an earlier stage may be an effective way to avoid inevitable brain damage so as to improve the clinical outcomes for patients with DWS. Continued treatment with regard to DWS condition may include shunt placement, but it mainly focus on developmental concerns, with occupational and physical therapy along with ongoing supportive psychotherapy to improve the coping skills and quality of life.

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“…Various movement disorders such as dystonia and tremor have been studied in spinal cord injuries such as syringomyelia and intramedullary masses 5,6 . To date, various electrophysiological studies have been conducted in dystonia.…”

Section: Introductionmentioning

confidence: 99%

Peripheral Silent Period In Cervical and Generalized Dystonia

Tütüncü

2021

Dicle Tıp Dergisi

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Objective: Dystonia is a movement disorder described by continuous or alternating muscle contractions causing unusual postures or insistent motions. Abnormal sensorimotor integration and abnormalities in inhibitory pathways are thought to have a role in pathogenesis. This paper aims to research the state of inhibitory pathways in the spinal cord in dystonia through documenting a silent period (SP) through cutaneous stimulation (CuSP) or mixed nerve stimulation (MnSP). Methods: 23 patients with dystonia were included in the study. Also, 19 healthy subjects were included in the study as a control group. Ages were similar between two groups. (p=0.092). CuSP and MnSP are recorded through abductor pollicis brevis and median nerve at the wrist respectively. Results: Onset latency, time, and suppression index of CuSP were equivalent between two groups. Moreover, the initiation and terminate latencies of MnSP, also duration, were similar between these two groups. I2 suppression index was lower in generalized dystonia patients compared to patients with cervical dystonia (100.0 vs 86.7±21.4%; F=4.252, 0.035). Conclusions: The most striking result to emerge from the data is that there was lower suppression during CuSP in generalized dystonia patients. Our results suggest that spinal inhibitory circuits are less active if only there is clinical involvement of the relevant segment.

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Tremor Caused by Dandy-Walker Syndrome Concomitant with Syringomyelia: Case Report and Review of the Literature Review

Cited by 4 publications

References 11 publications

Lateral ventricle pleomorphic xanthoastrocytoma concurrent with Dandy-Walker complex: A case report

Lateral ventricle pleomorphic xanthoastrocytoma concurrent with Dandy-Walker complex: A case report

Refractory psychiatric symptoms and seizure associated with Dandy-Walker syndrome: A case report and literature review

Peripheral Silent Period In Cervical and Generalized Dystonia

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