1/43. Arachnoid cyst of the middle fossa with paradoxical changes of the bony structures.Two patients with an arachnoid cyst of the middle fossa showed paradoxical changes of the adjoining bony structures of the skull. There was a diminution of the middle fossa and hyperplasia of the sphenoid sinus (pneumosinus dilatans) as well as a marked bulging of the squamous part of the temporal bone. In one case in which scinticisternography was performed, communication between the cyst and the subarachnoidal space was proven as well as an extremely slow cerebrospinal fluid circulation in the cyst. The pathogenesis of the cyst is discussed, based upon the structural changes of the skull, the angiographic findings and the locally disturbed cerebrospinal fluid circulation. The primary disturbance seems to be a temporal lobe agenesis.- - - - - - - - - - ranking = 1keywords = subarachnoid (Clic here for more details about this article) |
2/43. Superficial siderosis of the CNS associated with multiple cavernous malformations.Superficial siderosis of the CNS due to chronic, recurrent subarachnoid hemorrhage is an uncommon and potentially debilitating disorder. The classic clinical manifestation is progressive bilateral sensorineural hearing loss (SNHL), although ataxia and pyramidal signs also are observed frequently. Cavernous malformations rarely present with subarachnoid hemorrhage. We describe an unusual case of a young patient who presented with progressive, bilateral SNHL who was found to have superficial CNS siderosis associated with multiple cavernous malformations.- - - - - - - - - - ranking = 2keywords = subarachnoid (Clic here for more details about this article) |
3/43. Transthyretin amyloidosis and superficial siderosis of the CNS.OBJECTIVE: To describe a previously unreported clinical and radiologic presentation of hereditary transthyretin (TTR)-related amyloidosis. BACKGROUND: Unexplained cerebellar ataxia, pyramidal syndrome, and hearing loss are observed in some patients with TTR-related amyloidoses. methods: We performed clinical, radiologic, and pathologic examinations of three family members with TTR-related (Ala36Pro) amyloidosis. RESULTS: The patient was a 69-year-old woman with vitreal amyloid deposits, progressive sensorineural deafness, cerebellar ataxia, pyramidal syndrome, and recurrent transient neurologic symptoms. Cranial MRI showed symmetric thin rims of low signal intensity in T2- and T2*-weighted images in the cortex of the sylvian fissures, of the cerebellar hemispheres and vermis, and in the quadrigeminal plate consistent with superficial siderosis of the CNS. Her older daughter had vitreal amyloid deposits, acute brown-sequard syndrome at C4, acute sensorineural deafness, and recurrent transient neurologic symptoms. Cranial MRI at age 48 revealed a rim of low signal intensity in T2- and T2*-weighted images in the superior vermis folia and the right sylvian cortex. In addition, two small hemosiderin deposits were seen in the left parietal cortex. Lumbar puncture yielded colorless CSF with increased ferritin content and was followed by fourth ventricle hemorrhage. Cranial MRI 11 months later showed progression of brain hemosiderin deposits. The younger daughter had vitreal deposits, sensorimotor polyneuropathy, and acute sensorineural hearing but no evidence of siderosis on cranial MRI. She died at age 43 years of posterior fossa subarachnoid hemorrhage, and the neuropathologic examination showed amyloid deposition in the leptomeningeal spaces and vessels. CONCLUSION: Transthyretin-related amyloidosis may cause superficial siderosis of the CNS through subarachnoid bleeding related to meningovascular amyloid deposition.- - - - - - - - - - ranking = 2keywords = subarachnoid (Clic here for more details about this article) |
4/43. Primary angiitis of CNS : neuropathological study of three autopsied cases with brief review of literature.Primary angiitis of CNS(PACNS) or granulomatous angiitis of CNS is a rare inflammatory disease of small blood vessels mostly confined to the CNS. The clinical and pathological features of 3 autopsied cases are described. Clinically all the three PACNS patients were young males, age ranging from 19 to 31 years. All presented with varied neurological manifestations. There was no evidence of systemic disease in any of the cases. The ESR was normal and CSF analysis showed chronic meningitic pattern. The cerebral angiogram in one case was normal and the CT scan done in another case showed multiple intracerebral haematoma due to vasculitis. Brain biopsy was not done. diagnosis was made at post-mortem examination. histology showed characteristic but variable degree of granulomatous and non-granulomatous angiitis of small vessels. Venulitis with parenchymal haemorrhages was the predominant feature and in one case phlebitis with thrombosis was noted. Since the disease responds to steroids and immunosuppressive therapy, establishing antemortem diagnosis is important. In view of the association of angiitis of CNS with bacteria and viral infections, their role in the evolution of the disease needs to be investigated.- - - - - - - - - - ranking = 0.10307152086539keywords = haemorrhage (Clic here for more details about this article) |
5/43. Severe lightning pain after subarachnoid block in a patient with neuropathic pain of central origin: which drug is best to treat the pain?OBJECTIVE: There have been many reports that spinal anesthesia induces severe lightning pain in the lower limbs of patients with phantom limb pain, tabes dorsalis, or causalgia. We report on a patient with neuropathic pain of central origin who showed newly developed severe lightning pain after therapeutic subarachnoid block (SAB). We performed SAB 16 times in this patient, and he complained of severe pain each time. We investigated which drug was best for treating such induced pain by administering various drugs to the patient. SETTING: The patient was hospitalized for treatment and observation. PATIENT: The patient was a 48-year-old man with neuropathic pain secondary to an incomplete spinal cord injury at the cervical segment. INTERVENTIONS: Various drugs were administered for relieving the newly developed severe pain, and the effectiveness of these agents was compared. RESULTS AND CONCLUSIONS: Intravenous thiopental, fentanyl, butorphanol, ketamine, midazolam, droperidol, and sevoflurane-oxygen anesthesia were quite effective. Intramuscular butorphanol was not effective. Intravenous physiologic saline and atropine sulfate as a placebo, intrathecal morphine hydrochloride, intravenous mexiletine, and lidocaine were ineffective. Intravenous thiopental (approximately 1 mg/kg) was thought to obtain the best pain relief because it stopped the pain quickly, the dose needed was subanesthetic, and there was no adverse effect.- - - - - - - - - - ranking = 5keywords = subarachnoid (Clic here for more details about this article) |
6/43. Subarachnoid cyst and ascent to high altitude--a problem?A 31-year-old man suffered diplopia and ataxia on two occasions when he ascended from sea level to 4,000 m. Evaluation revealed a moderate-sized subarachnoid cyst in the left frontal region, which did not communicate with the cerebral ventricles. The cyst might have acted as a space-occupying lesion, and caused symptoms on ascent due to hypoxic brain swelling, brain compression against the cyst, and elevated intracranial pressure. Subarachnoid cysts are common, and they should be considered in the differential diagnosis of neurological problems at high altitude.- - - - - - - - - - ranking = 1keywords = subarachnoid (Clic here for more details about this article) |
7/43. central nervous system superficial siderosis, headache, and epilepsy.Almost 95 cases of superficial siderosis of the central nervous system have been reported in the literature. These patients showed a clinical syndrome characterized by ataxia, deafness, pyramidal system involvement, and mental deterioration with xanthochromic cerebrospinal fluid and neuroradiological findings of hemosiderin deposits. About 30% of the patients had headache as an accompanying symptom. In the present case report, we describe a 33-year-old man with the typical clinical features of superficial siderosis, who complained, since aged 8, of a severe recurrent frontal headache often associated with loss of consciousness occurring after at least 2 hours of pain. The MRI and CSF findings were consistent with subarachnoid bleeding. In our patient, headache due to meningeal irritation by subarachnoid blood induced seizures as a probable reflex of extreme pain. carbamazepine and nimodipine prophylaxis dramatically reduced the frequency of headaches and seizures.- - - - - - - - - - ranking = 2keywords = subarachnoid (Clic here for more details about this article) |
8/43. Eales' disease with neurological involvement. Part 2. pathology and pathogenesis.Detailed neuropathologic examination was carried out on 1 case of Eales' disease with CNS involvement, in the form of retinal vasculopathy, followed first by signs of brain stem and cerebellar disease and then by a myelopathy, with death 4 years later from retinal infection. There was mild chronic inflammation in the retina, and sub-total demyelination of one optic nerve. The brain stem and cerebellum showed extensive vasculopathy, with various stages of venous change extending from proliferation and dilatation to haemorrhage, or to thickening with hyalinisation. The perivenular brain tissue, particularly of the cerebellum, often showed demyelination, with relative axon preservation, but no inflammation. Similar, but less pronounced venopathy was seen in the dorsal cord. There was ascending degeneration of Goll's columns and descending degeneration of the lateral columns.- - - - - - - - - - ranking = 0.10307152086539keywords = haemorrhage (Clic here for more details about this article) |
9/43. Superficial siderosis of the central nervous system many years after neurosurgical procedures.Recurrent haemorrhage into the subarachnoid space causes superficial siderosis, which clinically manifests as cerebellar ataxia, sensorineural hearing loss, and myelopathy. Two patients developed clinical, radiological, and biochemical evidence of superficial siderosis many years after surgery. One had two posterior fossa operations, a left temporal craniectomy, and radiotherapy for a presumed brain tumour before developing clinical evidence of superficial sidersosis 37 years later. The other had small bilateral subdural collections from recurrent shunt revisions following posterior fossa surgery for a Chiari malformation, and then developed deafness and ataxia. The first patient currently has the longest recorded delay between presumed subarachnoid bleeding and clinical manifestations of superficial siderosis. Both patients provide further evidence that superficial siderosis of the central nervous system, a progressive neurodegenerative vascular condition, may be a delayed complication of neurosurgical procedures.- - - - - - - - - - ranking = 2.1030715208654keywords = subarachnoid, haemorrhage (Clic here for more details about this article) |
10/43. Superficial siderosis of the central nervous system: report of three cases and review of the literature.We present 3 cases and a review of the literature to demonstrate the current state of clinical diagnosis and therapy of superficial siderosis of the central nervous system. Typical symptoms were progressive cerebellar ataxia, spasticity and hearing loss. Repeated subarachnoid hemorrhage was indicated by persistent xanthochromia of the cerebrospinal fluid and confirmed by the presence of erythrophages, siderophages and iron-containing pigments. Deposition of free iron and hemosiderin in pial and subpial structures leads to intoxication of the central nervous system and represents the pathophysiological mechanism of superficial siderosis. Hypointensity of the marginal zones of the central nervous system on T2 weighted MR images indicates an iron-induced susceptibility effect and seems pathognomonic for superficial siderosis. In 39 of the 43 previously described cases superficial siderosis was verified by biopsy or autopsy. Today magnetic resonance imaging enables diagnosis at an early stage of the disease. Therapeutic management requires the elimination of any potential source of bleeding. In patients with unknown etiology no proofed therapy is yet available.- - - - - - - - - - ranking = 1keywords = subarachnoid (Clic here for more details about this article) |
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