Presentation
A review of the literature on medullary lesions presenting with intractable hiccups is provided. [ncbi.nlm.nih.gov]
Entire Body System
- Fever
A 51-year-old female, with a past medical history of resection of cerebellar hemangioblastoma, presented with an atrial tumor detected by sonography after undergoing a series of examinations for fever of unknown origin and subsequent symptoms of heart [ncbi.nlm.nih.gov]
Eyes
- Diplopia
The patient’s left eye was unable to fully deviate laterally, with worsening diplopia on left lateral end gaze. These findings were consistent with left sixth cranial nerve palsy. [consultant360.com]
Most patients present with diplopia and eventually develop complete paralysis of their eyes. Antibodies to the ganglioside GQ1b are associated with Miller Fisher syndrome. [dizziness-and-balance.com]
At 1 year postop she is stable, with resolution of her diplopia and dysconjugate gaze, and no progression of disease on subsequent imaging. She is scheduled to receive post radiation lomustine (CCNU) and cisplatin chemotherapy. [clinicalgate.com]
Diplopia is usually due to sixth nerve paralysis from increased intracranial pressure. [neurosurgery.me]
Neurologic
- Ataxia
His symptoms included episodes of loss of consciousness, cerebellar ataxia, and postural vertigo. The clinical diagnosis presented considerable difficulties. The simultaneous occurrence of both tumors in one patient has not been reported previously. [ncbi.nlm.nih.gov]
They are often called "ataxias". According to Musselman et al (2014), the prevalence of childhood ataxia is 26/100,000 children. Ataxia is rare compared to cerebral palsy (211/100,00) and autism (620/100,000). [dizziness-and-balance.com]
- Dysmetria
There may be nystagmus, ocular dysmetria and poor pursuit. Hemispheric cerebellar syndromes are characterized by incoordination of the limbs. There may be decomposition of movement, dysmetria, and rebound. [dizziness-and-balance.com]
These signs include ataxia (ie: wobbly gait), dysmetria (ie: uncoordinated movements of the limbs), and dysdiadochokinesia (ie: difficulty repeating rapid alternating movements). [virtualmedstudent.com]
Clinical signs of these deficits are detected by the presence of dysmetria in finger-to-nose or heel-to-shin testing, and dysdiadochokinesis demonstrated by testing of rapid alternating movements. [clinicalgate.com]
Clinical examination revealed left-sided cerebellar dysmetria with dysdiadochokinesia. He was normotensive and lacked any neurocutaneous marker. Routine laboratory investigations were within normal limits. [cancerjournal.net]
Ataxia in the extremities, dysmetria, and intention tremor are observed in cerebellar hemispheric lesions, whereas vermis lesions produce a broad-based gait and truncal ataxia. [neurosurgery.me]
- Cerebellar Ataxia
His symptoms included episodes of loss of consciousness, cerebellar ataxia, and postural vertigo. The clinical diagnosis presented considerable difficulties. The simultaneous occurrence of both tumors in one patient has not been reported previously. [ncbi.nlm.nih.gov]
According to Musselman et al (2014), the prevalence of childhood ataxia is 26/100,000 children. Ataxia is rare compared to cerebral palsy (211/100,00) and autism (620/100,000). Many cerebellar disorders are genetic in origin. [dizziness-and-balance.com]
View/Print Table Summary Table Condition Characteristics Ataxia-telangiectasia Telangiectasia of skin and conjunctiva, cerebellar ataxia Sturge-Weber syndrome Congenital facial angioma, glaucoma, choroidal hemangioma, leptomeningeal hemangioma Tuberous [aafp.org]
- Tremor
Intention tremors may be present on an attempt to touch an object. A kinetic tremor may be present in motion. The finger-to-nose and heel-to-knee tests are classic tests of hemispheric cerebellar dysfunction. [dizziness-and-balance.com]
The cardinal symptoms are episodic headaches, excessive sweating, palpitation, nervousness, and tremor. [neurosurgery.me]
Patients may also present with ataxic tremor, dysarthria, postural instability, or gait disturbances. [clinicalgate.com]
- Nystagmus
This patient was very unsteady and had strong positional nystagmus due to removal of her cerebellar nodulus. [dizziness-and-balance.com]
At the time of visit to our hospital, she presented with right dominant gaze rotatory nystagmus and right cerebellar hemisphere symptoms. [surgicalneurologyint.com]
Nystagmus, especially with a vertical or rotatory component, signifies brain stem involvement. Occasionally in elderly patients, dementia may be the sole or major manifestation of the disease. [neurosurgery.me]
Workup
Such a workup should include imaging of the chest (via chest x-ray and/or CT scan of the chest) and also CT of the abdomen and pelvis. [clinicalgate.com]
Table-3 gives an outline of the diagnostic workup of a patient with suspected VHL complex TABLE-3 Diagnostic Workup of a Patient with Suspected VHL Complex 1. Elicitation of family history and clinical examination 2. [neurosurgery.me]
Treatment
Thorough coverage for each neurological disease clearly defines age at onset, course of illness, clinical features, and treatment options. Differential diagnosis tables and treatment algorithms expedite clinical decision making. [books.google.de]
Massive hemorrhage from hemangioblastoma is rare but needs prompt treatment because of the relatively high mortality. [ncbi.nlm.nih.gov]
Top Treatment Definitive treatment is surgical resection. However, hemangioblastomas can be highly vascular, which means they tend to bleed like stink! [virtualmedstudent.com]
Prognosis
[…] is the leading international professional reference text that also serves as a bench book, describing all aspects of the pathology of brain tumours - genetics, molecular biology, epidemiology, morphology, immunohistochemistry, diagnostic criteria and prognosis [books.google.com]
However, cerebellar hemangioblastoma occurs in younger patients, is often multiple and recurrent, and has a poorer prognosis than sporadic cases. [jkns.or.kr]
These tumors generally have a good prognosis after surgical resection with an 85% to 100% 5-year survival rate. 1, 9, 10 The favorable prognosis in these patients has been attributed to the low-grade nature of cerebellar pilocytic astrocytomas and the [clinicalgate.com]
Prognosis [ edit ] The outcome for hemangioblastoma is very good, if surgical extraction of the tumor can be achieved; excision is possible in most cases and permanent neurologic deficit is uncommon and can be avoided altogether if the tumor is diagnosed [en.wikipedia.org]
In general, the patient with suspected VHL had a prognosis of vision resembling that of the non-VHL patients. [iovs.arvojournals.org]
Etiology
Although the precise etiology of the cystic component within hemangioblastoma tumor nodules is not known, it probably results from tumor degeneration. [ncbi.nlm.nih.gov]
Abstract quote Central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. [thedoctorsdoctor.com]
Etiology of the hemangioblastoma is obscure, but its presence in various clinical syndromes may suggest an underlying genetic abnormality. [emedicine.medscape.com]
Epidemiology
This is the leading international professional reference text that also serves as a bench book, describing all aspects of the pathology of brain tumours - genetics, molecular biology, epidemiology, morphology, immunohistochemistry, diagnostic criteria [books.google.com]
Hemangioblastomas in the elderly: epidemiology and clinical characteristics. J Clin Neurosci. 2014 Jul. 21 (7):1205-8. [Medline]. Yoon JY, Gao A, Das S, Munoz DG. [emedicine.medscape.com]
Clin Neuroradiol 20:5–24 Google Scholar Laakso A, Hernesniemi J (2012) Arteriovenous malformations: epidemiology and clinical presentation. [link.springer.com]
Pathophysiology
Various theories have been proposed to explain the rapid neurologic deterioration of hemangioblastoma patients during pregnancy; however, the pathophysiologic behavior and histogenesis of this vascular tumor are still not well understood. [ncbi.nlm.nih.gov]
Springer Science & Business Media, ٠٦/١٢/٢٠١٢ - 246 من الصفحات Because of the topographic and pathophysiologic information obtained with contemporary neuroimaging techniques, CT and MR scanning now constitute the most important investigation in clinical [books.google.com]
Pathophysiology VHL results from a germline mutation in the VHL gene which is a tumour suppressor gene located on the short arm of chromosome 3 (3p25–26). [eyewiki.aao.org]
Prevention
[…] wall enhancement demonstrates a correlation between enhancement on magnetic resonance imaging and presence of neoplasia within the cyst wall, as well as the importance of considering complete resection of these areas, including the cyst wall, for the prevention [ncbi.nlm.nih.gov]
However, XRT does not prevent regrowth following surgery. Outcome Hemangioblastomas are potentially curable lesions, although they are the cause of death in 82 percent of patients with von Hippel-Lindau syndrome. [neurosurgery.ucla.edu]
[…] kidney disease Treatment Medical management surveillance e.g., ophthalmologic exams for retinal hemangioblastomas Surgical management e.g., argon laser photocoagulation, partial nephrectomy, and neurosurgical excision in non-stable lesions Prognosis, Prevention [medbullets.com]
[…] while a complete surgical resection usually provides a definitive cure in sporadic hemangioblastomas. 3,4 Due to the autosomal dominant nature of VHL disease, the patient’s relatives should be screened to detect the manifestations at an early stage and prevent [consultant360.com]
Therefore, interventions such as early detection of dissemination, surgical removal of the local tumor, and stereotactic radiation may prevent widespread dissemination. [surgicalneurologyint.com]