Presentation
A review of the literature on medullary lesions presenting with intractable hiccups is provided. [ncbi.nlm.nih.gov]
Eyes
- Retinal Lesion
The visual prognosis for patients with retinal capillary hemangioma is guarded, especially with large, untreated lesions. [aafp.org]
Frequently there is retinal separation, massive gliosis and retinal edema. On occasion, a macular star or Papilledema develops. Recurrent bleeding from the lesion is not infrequent. [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]
- Dizziness
A 69-year-old man presented with dizziness and gait disturbance. He had a 4-year history of evacuation of cerebellar hemorrhage. [ncbi.nlm.nih.gov]
Signs and symptoms of hemangioblastoma include headaches, nausea/vomiting, difficulty with balance and dizziness/vertigo. Diagnosis The mass itself is found on either computed tomography (CT) or magnetic resonance imaging (MRI) scan. [neurosurgery.ucla.edu]
This case illustrates why it is is occasionally critical that patients with dizziness of unknown cause see a neurologist. [dizziness-and-balance.com]
Additionally, she noted the onset of dizziness, face numbness, and intermittent ataxia a few weeks prior to presentation. She had no family history of von Hippel-Lindau (VHL) disease. Physical examination. [consultant360.com]
[…] clinical manifestations, although these groups often correlate with certain types of mutations present in the VHL gene. [5] Signs and symptoms [ edit ] Signs and symptoms associated with VHL disease include headaches, problems with balance and walking, dizziness [en.wikipedia.org]
- 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]