Manual of neurologic therapeutics - part 3 ppsx

1. Poor prognostic factors: age younger than 2 years, incomplete resection, supratentorial location, duration of symptoms less than 1 month, and anaplastic histology. 2. The 5-year survival after complete resection and radiotherapy is 70% to 87% compared to 30% to 40% for partially resection; overall 10-year survival of 50%. 3. In children, fourth-ventricle tumors clinically more aggressive. 4. Anaplastic ependymoma has a 12% 5-year survival. 5. Subependymoma is indolent and often does not require treatment. 6. The prognosis for ependymoblastoma is poor with death within 1 year of surgery. DIAGNOSIS Location 1. Infratentorial in 60% of cases. 2. Most frequently in fourth ventricle (70%), lateral ventricles (20%), and cauda equina (10%). 3. In adults, commonly occurs in lumbosacral spinal cord and filum terminale (myxopapillary ependymoma). 4. May spread via CSF and seed other locations (12%). 5. Ependymoblastoma usually in cerebrum with frequent craniospinal metastasis. Clinical Presentation 1. Intracranial tumors produce symptoms due to obstruction of CSF flow (headaches, nausea, vomiting, visual disturbance), ataxia, dizziness, hemiparesis, and brainstem symptoms. 2. Spinal cord tumors present as a chronic, progressive myelopathy or cauda equina syndrome (see section on Spinal Cord Tumor). Diagnostic Tests 1. MRI shows a well-demarcated, heterogenous, enhancing intraventricular mass, with frequent calcifications. Obstructive hydrocephalus and hemorrhage may be present. 2. Spinal MRI should be done to rule out neuraxis dissemination. Pathology 1. Grossly well circumscribed, tan, and soft tissue. 2. Microscopically densely cellular with ependymal rosettes, blepharoplasts, and perivascular pseudorosettes. 3. In cauda equina, the myxopapillary form common. 4. Anaplastic ependymomas have malignant features such as mitotic activity, pleomorphism, and necrosis. 5. Ependymoblastoma has ependymoblastic rosettes in fields of undifferentiated cells. 6. Subependymoma is a benign lesion located within ventricles. Has both ependymal and astrocytic features. Differential Diagnosis Subependymoma, anaplastic ependymoma, ependymoblastoma, astrocytomas, medulloblastoma. TREATMENT 1. Surgical resection is treatment of choice but many tumors recur regardless of completeness of resection. P.133 2. For ependymoma and anaplastic ependymoma, postoperative local radiation (4,500 – 6,000 cGy) improves survival. 3. Craniospinal radiation reserved for tumors with CSF spread. 4. Chemotherapy is used in children younger than 3 years to delay onset of RT. 5. Results of chemotherapy are generally poor. CHOROID PLEXUS TUMORS BACKGROUND 1. Choroid plexus tumors are derived from the choroid plexus epithelium. 2. Peak incidence in first two decades of life. It is the most common intracranial tumor in the first year of life. 3. Accounts for less than 1% of all intracranial tumors. PATHOPHYSIOLOGY 1. Possible role for simian virus 40 (SV40) in pathogenesis. 2. Choroid plexus papilloma (CPP) (WHO grade I) histologically resembles normal choroid plexus and probably represents local hamartomatous overgrowths. 3. Choroid plexus carcinoma (CPC) (WHO grades III–IV) account for 10% of choroid plexus tumors. They are aggressive tumors with dense cellularity, mitoses, nuclear pleomorphism, focal necrosis, loss of papillary architecture, and invasion of neural tissue. They frequently seed CSF pathways. Usually occurs in children younger than 8 years. PROGNOSIS 1. Good with CPP. With complete resection, 80% 5-year survival; 4.3% recurrence rate overall. 2. Poor with CPC. DIAGNOSIS Location 1. In adults, common in fourth ventricle, lateral ventricle, and third ventricle. 2. In children, most common in lateral ventricles and cerebellopontine angle (CPA). Clinical Presentation Present with symptoms secondary to CSF obstruction or CSF overproduction, headaches, nausea, vomiting, ataxia. Diagnostic Tests MRI shows homogenous, enhancing mass with prominent flow voids due to rich vascularization, frequent calcification. Differential Diagnosis Ependymoma, astrocytoma, metastases. TREATMENT 1. Surgical resection. 2. Postoperative RT for CPC; RT at recurrence for CPP. P.134 NEURONAL AND MIXED NEURONAL – GLIAL TUMORS BACKGROUND 1. Initially thought to be hamartomas, but these are ganglion cell tumors that form a continuum between those with mixed ganglion and glial cell components (gangliogliomas) and some that are relatively pure ganglion cell tumor. 2. Include ganglioglioma, gangliocytoma, DNT, neurocytoma, and dysplastic gangliocytoma of the cerebellum (Lhermitte – Duclos disease). Epidemiology 1. Occur in children and young adults in first three decades of life. 2. Account for less than 1% of glial neoplasms. 3. Neurocytomas occur in patients aged 20 to 40. PATHOPHYSIOLOGY 1. Uncertain. 2. Gain of chromosome 7 in neurocytomas. 3. Gangliogliomas associated with Down syndrome, callosal dysgenesis, and neuronal migration disorders. 4. Lhermitte–Duclos disease may occur as part of Cowden disease (mucosal neuromas and breast cancer), an autosomal dominant disorder caused by germline mutation of PTEN gene. PROGNOSIS 1. Ganglioglioma: Indolent, cured with surgery. If subtotal resection, 41% progress. Rare malignant transformation from glial component; 89% 5-year and 84% 10-year survival. 2. Neurocytoma: Good with resection, recurrence and CSF spread are rare. 3. DNTs are indolent. 4. Lhermitte – Duclos disease: Good with resection. DIAGNOSIS Location 1. Gangliogliomas have a predilection for temporal lobe but also occur in the basal ganglia, optic pathway, brainstem, pineal gland, cerebellum and spinal cord. 2. Neurocytomas are intraventricular, usually in body of lateral ventricle, attached to septum pellucidum. Rarely in pons, cerebellum, spinal cord, or brain parenchyma. 3. DNTs involve predominantly the cerebral cortex, especially temporal lobes. 4. Lhermitte – Duclos disease occurs in cerebellum. Clinical Presentation 1. Gangliogliomas usually present with seizures and, less often, headaches and focal deficits. 2. Neurocytomas present with symptoms of hydrocephalus. 3. DNTs usually have chronic complex partial seizures. 4. Lhermitte – Duclos disease presents with ataxia and hydrocephalus. Diagnostic Tests 1. Ganglioglioma: MRI is nonspecific and shows a well-demarcated, superficial, nonenhancing mass with increased T 2 and FLAIR signal. Can have cysts or calcification. P.135 2. Neurocytoma: MRI shows a heterogenous mass with multiple cysts, calcification, occasional hemorrhage, variable enhancement; some have a “ honeycomb ” appearance on T 1 -weighted images. 3. DNT: MRI shows a multicystic mass with gyrus-like configurations, cortical dysplasia. 4. Lhermitte – Duclos disease: MRI shows increased T 2 and FLAIR abnormality in cerebellum. Pathology 1. Gangliogliomas (WHO grades I – II) have neuronal and astrocytic neoplastic cells, granular bodies, Rosenthal fibers, large irregular ganglion cells, and perivascular infiltrates. 2. Neurocytomas (WHO grade I) have small uniform, well-differentiated neuronal cells, frequently misdiagnosed as oligodendrogliomas. 3. DNTs (WHO grade I) have a glioneuronal element, nodular component, and cortical dysplasia. 4. Gangliocytomas (WHO grade I) are well-differentiated neoplastic cells with neuronal characteristics, no malignant transformation. 5. Lhermitte – Duclos (WHO grade I) disease has a dysplastic gangliocytoma confined to cerebellum, Purkinje cell layer is absent. TREATMENT 1. Surgical resection; complete resection is curative for all these conditions. 2. RT may have limited role for recurrent gangliogliomas. 3. Anaplastic gangliogliomas may respond to chemotherapy with temozolomide or PCV. PINEAL PARENCHYMAL TUMOR BACKGROUND 1. Rare tumors that account for fewer than 1% of all intracranial tumors; 14% to 30% of pineal region tumors. 2. Pineocytoma most common between 25 and 35 years; pineoblastoma most common in first two decades. PATHOPHYSIOLOGY Arise from pinocyte in pineal gland. PROGNOSIS 1. Pineocytoma is slow growing and has favorable prognosis following resection; 86% 5-year survival. 2. Pineoblastoma has poorer prognosis; less than 50% 5-year survival. 3. Pineal parenchymal tumors of intermediate differentiation (PPTIDs) have an intermediate prognosis. DIAGNOSIS Location Pineal gland; pineoblastoma has relatively frequent leptomeningeal metastases. P.136 Clinical Presentation 1. Most commonly presents with noncommunicating hydrocephalus from obstruction of aqueduct of Sylvius and Parinaud syndrome (paralysis of upgaze, convergence – retraction nystagmus, light – near dissociation) due to compression of midbrain tectum. Ophthalmoplegia, ataxia, weakness, numbness, and memory loss may also occur. 2. Hypothalamic dysfunction (diabetes insipidus, precocious puberty) when tumors encroach anteriorly; sleep disturbance due to abnormal melatonin regulation. Diagnostic Tests 1. MRI shows a variably enhancing pineal region mass with or without leptomeningeal enhancement. 2. Serum and CSF alpha fetoprotein (AFP) (yolk sac tumors) and β-human chorionic gonadotropin (β-hCG) (choriocarcinoma) are negative and help to exclude germ cell tumors. 3. Check CSF cytology and contrast-enhanced MRI of spine to rule out leptomeningeal metastases if not contraindicated. Pathology 1. Grossly displaces surrounding structures; does not invade; can seed leptomeninges. 2. Pineocytoma: Well-differentiated with small, uniform, mature cells resembling pinocytes. 3. PPTID, as name implies, has intermediate histologic appearance. 4. Pineoblastoma is high grade and histologically identical to PNETs. Composed of highly cellular sheets of small cells with round/irregular nuclei and scant cytoplasm. Occasional Homer – Wright or Flexner – Wintersteiner rosettes. Differential Diagnosis Germ cell tumors [germinoma, teratoma, dermoid, choriocarcinoma, embryonal carcinoma, endodermal sinus (yolk sac) tumor], astrocytoma, ependymoma, choroid plexus papilloma, meningioma, metastases and nonneoplastic lesions including pineal cyst, arachnoid cyst, arteriovenous malformation, Vein of Galen aneurysm, and cavernous malformation. TREATMENT 1. Surgical exploration and complete resection. 2. Ventricular shunting for hydrocephalus. 3. Local irradiation for incompletely resected or recurrent pineocytoma. 4. Craniospinal RT for pineoblastoma and PPTID. 5. Role of chemotherapy unclear but usually given for pineoblastoma and often given for PPTID. 6. Chemotherapeutic agents include cisplatin, carboplatin, etoposide, cyclophosphamide, and vincristine. MEDULLOBLASTOMA BACKGROUND 1. Medulloblastomas are the most common (20%) malignant tumor of childhood. 2. Comprise more than one third of all pediatric posterior fossa tumors. 3. Incidence 0.5/100,000. 4. Male to female ratio 2 to 1. P.137 5. Occurs in first decade of life (ages 5 – 9 years), 70% diagnosed before age 20. Second peak in the 20 ′ s to 30 ′ s (30% of cases). PATHOPHYSIOLOGY Ninety percent are sporadic but can occur in Gorlin syndrome (basal cell carcinomas, congenital anomalies) caused by germline mutation of gene encoding the sonic hedgehog receptor PTCH. May also arise in Turcot syndrome caused by germline mutation of the adenomatous polyposis coli (APC) gene. Rarely, they occur in patients with ataxia–telangiectasia, xeroderma pigmentosum, or Li – Fraumeni syndrome. PROGNOSIS 1. Patients generally classified into poor-risk and standard-risk groups. 2. Poor-risk factors include residual disease greater than 1.5 cm 3 , metastases detected by contrast-enhanced MRI, and malignant cells in CSF obtained by LP. 3. The 5-year survival rate for standard-risk patients is approximately 70% to 80%. The 10-year survival rate is above 50%. 4. The 5-year survival rate for poor-risk patients is 40% to 50%. 5. Infants tend to have worse prognosis than older age groups. 6. Desmoplastic variant associated with better prognosis. 7. Tumors expressing neurotrophin-3 receptor, TrkC, have better prognosis; increased expression of neuroregulin receptors erbB2 and erbB4 and c-myc associated with worst prognosis. DIAGNOSIS Location 1. Midline cerebellum, inferior vermis (85%), and fourth ventricle. 2. Tends to infiltrate the cerebellar hemispheres and frequently (25% – 30% of cases) have leptomeningeal metastases ( “ drop metastases ” ). Systemic metastases rare (bone and lung). 3. Desmoplastic variant (15%) more lateral in cerebellar hemisphere. Clinical Presentation 1. Most tumors present with signs of increased ICP (headache, nausea, and vomiting) due to obstruction of CSF flow. Patients may also have ataxia and diplopia. 2. In older age groups, tumor more often occurs in cerebellar hemispheres, resulting in truncal ataxia and cerebellar dysfunction. Diagnostic Tests 1. MRI or CT shows a high-density, enhancing tumor, usually midline, often distorting or obliterating the fourth ventricle, and producing hydrocephalus. Calcification may be present. 2. High tendency to metastasize to other parts of the CNS; therefore, entire neuraxis should be imaged. 3. May also metastasize outside of CNS to bone; therefore, a bone scan and bone marrow aspirate should also be performed. Pathology 1. Grossly soft, pinkish-gray mass, granular with necrosis. 2. Microscopically highly cellular tumors with abundant dark staining round or oval nuclei and scant, undifferentiated cytoplasm typical of “ small round blue cell tumors. ” Mitoses and apoptotic cells are abundant. Homer – Wright rosettes (sheets P.138 of cells forming rosettes around a central area filled with neuritic processes) in up to 40% of cases. 3. Have both neuronal and glial differentiation and some with mesenchymal differentiation. 4. Desmoplastic variant has abundant reticulin and collagen. Differential Diagnosis Astrocytomas, ependymomas, ependymoblastoma, large cell PNET (aggressive course), medullomyoblastoma (contains immature muscle cells, malignant), melanotic PNET, and embryonal tumors (atypical teratoid or rhabdoid tumors, highly malignant and therapy-resistant). TREATMENT 1. Surgical resection needed to relieve mass effect and some may require a VP shunt for decompression. 2. Goal is maximal surgical resection because residual tumor greater than 1.5 cm is associated with increased risk of relapse. 3. Surgery occasionally complicated by “ cerebellar mutism ” (mutism and emotional lability). 4. Craniospinal RT indicated in all patients after surgery. 5. RT comprising 5,000 to 5,500 cGy usually administered to the posterior fossa and 3,600 cGy applied to the remainder of the cranium and the spine of all high-risk patients. 6. Craniospinal RT of 2,400 cGy for standard-risk patients, especially those younger than 5 years. 7. Craniospinal RT frequently produces neurocognitive complications in children. 8. Current studies are evaluating lower doses of craniospinal RT in conjunction with chemotherapy in children to reduce long-term complications of RT. 9. SRS boost often administered to any residual nodules of tumor. 10. Sensitive to chemotherapy: adjuvant therapy with agents such as cisplatin and etoposide, and cyclophosphamide and vincristine. Other active agents include lomustine, procarbazine, and carboplatin. Adjuvant chemotherapy improves survival in patients with high-risk disease and possibly also for patients with standard-risk disease. 11. Controversy regarding use of chemotherapy before or after RT. No evidence that preradiation chemotherapy is more effective. 12. In infants and young children, chemotherapy is sometimes used alone and RT deferred until they are 3 years old. Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition CRANIAL AND SPINAL NERVE TUMORS Part of "6 - Neurooncology" SCHWANNOMA BACKGROUND 1. Schwannomas are benign tumors that originate from the Schwann cell at the glial – Schwann cell junction (Obersteiner – Redlich zone) of the peripheral nerves. 2. Vestibular schwannomas (acoustic neuroma) arise from the vestibular portion of the eighth nerve. 3. In periphery, arise from paraspinal dorsal nerve roots and cutaneous nerves. P.139 Epidemiology 1. Incidence 1/100,000, female-to-male ratio (1.5:1). 2. Occurs in middle adult life and rare in childhood. 3. Most commonly arises from vestibular nerve (usually solitary; frequently bilateral in NF2). 4. Vestibular schwannomas account for 8% of all intracranial tumors and 80% of CPA tumors in adults. PATHOPHYSIOLOGY 1. Increased incidence in NF2. Patients often have bilateral acoustic schwannomas and multiple cranial and spinal schwannomas, meningiomas, and gliomas. 2. Inactivating mutations of NF2 gene also frequent in spontaneous schwannomas. PROGNOSIS 1. Slow-growing tumors usually cured by surgery. 2. Malignant degeneration rare in the CNS but more common in the PNS. DIAGNOSIS Location Most common CN VIII in the CPA but may occur wherever Schwann cells are present (other CNs, spinal nerves, and peripheral nerve trunks). Clinical Presentation 1. Most common include unilateral hearing loss, tinnitus, and unsteadiness from acoustic nerve dysfunction evolving over months to years. 2. Dysfunction of other CNs and brainstem occurs if it becomes large enough [trigeminal dysfunction (loss of corneal reflex, facial numbness), facial weakness, ataxia, vertigo]. 3. Isolated vertigo uncommon as initial symptom. Diagnostic Tests 1. Audiometry is helpful for detecting unilateral sensorineural hearing loss. 2. Brainstem auditory evoked potentials abnormal in more than 90% of patients (prolongation of waves I – III and I – V latency). 3. MRI with gadolinium is the most sensitive imaging modality and shows intradural, extraaxial, enhancing mass. 4. In the spine, tumor may extend through the intervertebral foramen, resulting in an hourglass appearance. 5. CT scan useful to delineate the anatomy of the bones involved. Pathology 1. Two types of distinct histology: Antoni A (compact, elongated cells with occasional nuclear palisading) and Antoni B (loose, reticulated tissue). 2. Arise at the periphery of nerve; usually encapsulated and compress but do not invade adjacent neural tissue. Differential Diagnosis 1. Most common CPA tumor. Differential includes meningioma, cholesteatoma, epidermoid, metastatic disease, glioma. P.140 2. Schwannomas arising from spinal roots may resemble meningiomas and neurofibromas. TREATMENT 1. Small asymptomatic lesions can often be observed and treated only if they increase in size. 2. Surgical resection can be complete for tumors smaller than 2 cm and can preserve hearing in 75% of patients. 3. Surgical morbidity is related to size of tumor (lower than 5% for tumors smaller than 2 cm, 20% for tumors larger than 4 cm) and includes facial paralysis, hearing loss, CSF leak, imbalance, and headache. 4. If hearing is good, then one should also consider early treatment as delay may result in hearing impairment. 5. SRS probably equally effective, especially in older patients and those at high risk for surgery. Fractionated SRT associated with less morbidity. NEUROFIBROMA BACKGROUND 1. Arise from cells with features of Schwann cells, fibroblasts, and perineural cells and are usually benign. 2. Almost always associated with NF1 and usually multiple. 3. Malignant peripheral nerve tumors (MPNTs) occur in 1/10,000 and arise de novo or from sarcomatous degeneration of a preexisting plexiform neurofibroma. PATHOPHYSIOLOGY Associated with NF1. PROGNOSIS Additional lesions tend to arise and in NF1, malignant degeneration may occur. DIAGNOSIS Location Most involve dorsal spinal nerve roots, major nerve trunks, or peripheral nerves. CN involvement very rare. Clinical Presentation Cutaneous neurofibromas present as small painless masses. Nerve root neurofibromas may present with pain and sensorimotor disturbance. Diagnostic Tests MRI shows widening of the neural foramina with pedicle erosion in neurofibromas arising from spinal roots. Pathology 1. Hyperplasia of Schwann cells and fibrous elements of the nerve. Elongated wavy interlacing hyperchromatic cells with spindle-shaped nuclei in a disorderly loose mucoid background with collagen fibrils. Nerve fibers are intertwined in the tumor. P.141 2. Plexiform neurofibroma associated with NF1, which has an increased incidence of malignant transformation. 3. Malignant peripheral nerve sheath tumors (MPNSTs) are highly malignant sarcomas, many occur in NF1 with preexisting plexiform neurofibroma. Differential Diagnosis Perineuriomas arise from pericytes. TREATMENT 1. Palliative surgical decompression as needed. 2. RT occasionally useful in malignant tumors. Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition MENINGEAL TUMORS Part of "6 - Neurooncology" MENINGIOMA BACKGROUND 1. Arise from cells that form the outer layer of the arachnoid granulations of the brain (arachnoid cap cells). 2. Meningioma is the most common benign tumor and the second most common PBT in adults. 3. Represents approximately 20% of all intracranial neoplasms and 25% of intraspinal tumors. 4. Rare in first two decades and increases progressively thereafter. 5. Peak incidence in fourth to fifth decades, strong female predominance (3:2). 6. Higher incidence in patients with breast cancer. 7. Pregnancy may be associated with tumor progression (strong hormonal influence). PATHOPHYSIOLOGY 1. Proven risk factors are female gender, increasing age, NF2, and history of cranial irradiation. 2. Meningiomas have partial or complete deletions of chromosome 22. 3. Patients with NF2 may have multiple meningiomas. 4. Progesterone receptors are present in 70% of tumors and play a role in tumor growth. 5. PDGF, EGFR, vascular endothelial growth factor (VEGF), and their receptors are expressed in meningiomas. PROGNOSIS 1. Excellent for most patients. Median survival more than 10 years. 2. Most are slow-growing lesions that remain stable for many years. 3. Of meningiomas, 4.7% to 7.2% have atypical features and 1% to 2.8% have anaplastic features and much worse prognosis. Median survival for malignant meningiomas is less than 2 years. 4. Recurrence is related to completeness of the resection and location. 5. Poor prognostic factors include papillary histologic characteristics, large number of mitotic figures, necrosis, and invasion of cortical tissue by tumor cells. P.142 DIAGNOSIS Location 1. Mostly extraaxial and intracranial. 2. Ninety percent are supratentorial involving the cerebral convexities (50%, parasagittal, falx, or lateral convexity), skull base (40%, sphenoid wing, olfactory groove, or suprasellar), posterior fossa, foramen magnum, periorbital region, temporal fossa, and ventricular system. 3. Intraspinal tumors account for 25% of primary spinal tumors and are usually in thoracic segment. Clinical Presentation 1. Present with seizures, headaches, and focal deficits. 2. Twenty percent are asymptomatic and are an incidental finding. 3. Spinal meningiomas present with pain, weakness, numbness, and gait unsteadiness. Diagnostic Tests 1. MRI or CT with contrast shows a well-defined, homogenously enhancing extraaxial mass that may be calcified. If edema present usually indicates a higher grade tumor or a secretory meningioma. 2. On T 1 - and T 2 -weighted sequences, meningiomas can be easily missed as they are isointense to slightly hypointense [...]... Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition GERM CELL TUMORS Part of "6 - Neurooncology" BACKGROUND 1 Most common tumor of pineal gland (60%) and most are malignant 2 Peak incidence second decade, predominantly males (3: 1); 95% occur before age 33 3 Germinomas account for 60% of germ cell tumors Teratoma and mixed germ cell tumors (20% 30 %) Embryonal carcinoma, endodermal... observed 2 Surgical excision is treatment of choice Tumors often very vascular 3 RT and SRS may be of benefit for recurrent or inoperable tumors 4 Clinical trials using inhibitors of VEGF under way Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA Part of "6 - Neurooncology" BACKGROUND 1 Primary central... Postoperative radiation can delay recurrence or progression of symptoms Patients usually receive 3, 500 to 4,500 cGy 7 Chemotherapy has a very limited role for high-grade gliomas and recurrent tumors Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition NEUROLOGIC COMPLICATIONS OF SYSTEMIC CANCER Part of "6 - Neurooncology" BRAIN METASTASES BACKGROUND 1 Brain... Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition PARANEOPLASTIC SYNDROMES OF THE BRAIN AND CEREBELLUM Part of "6 - Neurooncology" P.165 CEREBELLAR DEGENERATION 1 Subacute cerebellar syndrome with truncal and appendicular ataxia progressing over weeks or months Dysarthria, diplopia, vertigo, nystagmus (Tables 6-2 and 6 -3 ) TABLE 6-2 PARANEOPLASTIC SYNDROMES AFFECTING... SCLC (anti-Hu, anti-CV2, anti-CRMP 5), testicular cancer (anti-Ma2), and a variety of tumors (anti-Ma1) 7 Some patients respond to treatment of cancer Rare responses to immunosuppression OPSOCLONUS – MYOCLONUS 1 Subacute opsoclonus, myoclonus, ataxia 2 Usually associated with neuroblastoma in children; breast cancer, SCLC in adults 3 Anti-Ri (breast and gynecologic cancer), anti-Hu, anti-Yo, Anti-Ma2 4... Anti-Ma2 4 May respond to treatment of tumor, steroids, and IVIG Prognosis better in children Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A Manual of Neurologic Therapeutics, 7th Edition PARANEOPLASTIC SYNDROMES OF THE EYE Part of "6 - Neurooncology" P.167 CARCINOMA-ASSOCIATED RETINOPATHY 1 Carcinoma-associated retinopathy (CAR) is subacute onset of episodic visual obscuration, photosensitivity,... tumor at an early stage 3 Usually produce significant neurologic disability 4 Often mistaken for metastatic disease 5 Provide information concerning autoimmune disorders and tumor immunology PROGNOSIS 1 Most patients develop profound neurologic disability Response of neurologic deficits to treatment is often poor 2 Course of underlying neoplasm often indolent, possibly because of antitumor immunologic... spread via communication with Batson venous plexus 3 Compression of spinal cord from tumor and peritumoral edema PROGNOSIS 1 Depends on the primary tumor, extent of systemic disease, and severity of symptoms at presentation 2 Ninety percent to 100% of patients who are ambulatory at onset of treatment remain so at the end of treatment 3 Only 13% to 30 % of patients who are nonambulatory at presentation... frequency of side effects For most patients, 10-mg bolus of dexamethasone and then 16-mg/d maintenance in divided doses is adequate A 100-mg bolus and 96-mg/d maintenance may be reasonable for patients who are paraplegic Patients receiving high doses of dexamethasone should receive H 2 -blockers or proton pump inhibitors 2 Nonsteroidal antiinflammatory medications and opiates for pain control 3 Stool softeners... Paraneoplastic neurologic syndromes account for a high percentage of certain disorders (70% of LEMS, 50% of subacute cerebellar degeneration, 50% of opsoclonus–myoclonus in children, 20% of opsoclonus–myoclonus in adults, 20% of subacute sensory neuronopathy) 2 Frequently occur before diagnosis of the underlying cancer Recognition of these syndromes syndrome may lead to the diagnosis of the underlying . are 3 years old. Copyright ©2004 Lippincott Williams & Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition CRANIAL AND SPINAL NERVE TUMORS Part of "6 - Neurooncology". Martin A. Manual of Neurologic Therapeutics, 7th Edition MENINGEAL TUMORS Part of "6 - Neurooncology" MENINGIOMA BACKGROUND 1. Arise from cells that form the outer layer of the arachnoid. Wilkins Samuels, Martin A. Manual of Neurologic Therapeutics, 7th Edition GERM CELL TUMORS Part of "6 - Neurooncology" BACKGROUND 1. Most common tumor of pineal gland (60%) and most