Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0004134 (ataxia)
 15,886 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A large endolymphatic sac papillary adenocarcinoma in a patient with Von Hippel-Lindau (vHL) disease is reported. A 31-year-old man was treated for a left eye retinal angioma 10 years previously and had been blind in that eye since. He was admitted with progressive tinnitus, lower cranial nerve paresis and ataxia. Investigations revealed a vascular and bone-eroding petrous tumour. The entirely extradural tumour involved a large part of the petrous bone and extended into the cerebellopontine angle. The vascular tumour was radically resected using a presigmoid approach after feeding vessel embolisation. The literature on this rare clinical entity is briefly discussed.
 ...
PMID:Endolymphatic sac carcinoma of the right petrous bone in Von Hippel-Lindau disease. 1667 27

Cavernous angiomas comprise 5-10% of all vascular malformations in the central nervous system, occurring most frequently in the supratentorial region, and 20% of them in the brain stem. According to literature, brain stem cavernous angiomas occur most frequently in the pons (60%), and equally in the mesencephalon (20%) and in medulla oblongata. In clinical evaluation the authors describe the successful removal of a mesencephalic cavernous angioma causing progressive neurological deficits and symptoms. The authors present a case of a 51 year old female, who had developed 1 year prior to her admittance: fatigue, weakness in the right upper limb and fingers, right lower limb ataxia. One month later, her lower right limb developed sensory deficits. The first neurological exploration indicated dysarthria, moderate facial and right hemiparesis, hemihypaesthesia and ataxia. CT and MR imaging indicated multilobulated cavernomas in the mesencephalon. After conservative treatment the patient became almost symptom free, and thus neurosurgical treatment was not discussed. Later on her symptoms fluctuated, but after 6 month she suddenly developed progressive right hemiparesis, right facial weakness, serious dysphasia, and emotional incontinence combined with continuous spastic sobbings. The control MRI showed enlargement of the cavernomas and new extravasation. Surgery was indicated for removing the cavernomas. The left infratentorial, supracerebellar approach revealed a blood engorged cavernoma in the center of the mesencephalon, almost dividing it. The cavernomas and accompanying haematoma was extirpated. The patient's neurological symptoms rapidly improved after surgery, her dysphasia as well as motor weakness have disappeared. Six days after surgery, we discharged a neurologically symptomless and self-supporting patient. The literature and the presented case indicates that the correct timing and proper surgery allows brain stem cavernomas to be safely removed, or significantly bated, which results in the massive regression of neurological symptoms.
 ...
PMID:[Successful surgical removal of a mesencephalic cavernous angioma, which was responsible for progressive neurological deficits]. 1876 80

We report the case of a 13-year-old boy who presented at the emergency department for nausea and vomiting with ataxia and dissymmetry. He had strabismus as a consequence of palsy of the VI cranial nerve when 9 months old that was attributed to an allergy to penicillin. He had no relevant family history. Urgent CT and posterior MRI examination showed multiple supratentorial and infratentorial cavernous angiomas, one of which was located in the brainstem and presented acute hemorrhage, causing the symptoms. Angiography performed later showed no evidence of vascular malformations. The patient was initially managed conservatively, but the cavernous angioma in the brainstem was surgically extirpated after the patient's condition progressively worsened.
 ...
PMID:[Intracranial cavernous angioma.]. 1928 34

A 15-year-old girl had no REM sleep presumably due to a pontine cavernous hemangioma was reported. Her brain MRI revealed a cavernous hemangioma extending from the dorsal pontine to the medulla. She manifested truncal ataxia, facial nerve palsy, and ocular motor apraxia. She could not sleep in the supine position due to the sleep apnea accompanied with loud snoring. Overnight polysomnography (PSG) was performed for detection of obstructive sleep apnea syndrome (OSAS). In addition to severe OSAS and Cheyne-Stokes-like respiration at wake after sleep onset, her 1st PSG study revealed no periods with rapid eye movement, EEG characteristic of REM sleep, atonia and variation on respiratory and heart rate. Even after effective therapy for OSAS with non-invasive positive airway pressure ventilation (NPPV), her 2nd PSG also failed to show stage REM. These findings suggest that this pontine cavernous hemangioma disturbed her REM-on system. This is the first report of an individual with long-term loss of REM sleep and a valuable case for the understanding of anatomical structures of the REM-on system and the role of REM in memory consolidation.
 ...
PMID:[Deficiency of REM sleep in a patient with pontine cavernous hemangioma]. 1976 54

Intracranial extra-axial cavernous angiomas are rare lesions. We report a rare case of extra-axial cavernous angioma in the cerebellopontine angle (CPA) in a 50-year-old male, who presented with lower cranial nerve palsy and gait ataxia. Computed tomography (CT) scan of the brain showed a hyperdense lesion in the left cerebellopontine angle. The lesion was totally excised by the retrosigmoid approach and a pathological examination confirmed the lesion to be a cavernous angioma. Following surgery, the lower cranial nerve palsy recovered significantly.
...
PMID:A rare case of an extra-axial cavernous angioma in the cerebellopontine angle. 2391 9

A 36-year-old man with progressive hearing impairment visited our hospital complaining of a severe headache. A neurological examination revealed bilateral sensorineural hearing impairment, mild ataxia, hyperreflexia and mild cognitive dysfunction. Brain MRI demonstrated hydrocephalus and typical hypointensity rimming the brain surface on T2(*)-weighted images. The patient was diagnosed as having superficial siderosis. Spinal MRI disclosed the presence of a lumbar intradural extramedullary mass. The surgically resected tumor was histologically found to be a cavernous angioma. Superficial siderosis is an important cause of hearing loss. With respect to the detection of disorders underlying this pathological condition, MRI examinations, including those of the brain and whole spinal cord, are recommended.
...
PMID:Spinal intradural extramedullary cavernous angioma presenting with superficial siderosis and hydrocephalus: a case report and review of the literature. 2513 Jan 2

The authors describe the case of a 3-year-old boy with a giant congenital vertex hemangioma who underwent presurgical embolization with Onyx (ethylene-vinyl alcohol copolymer dissolved in dimethyl sulfoxide) and Glubran ( N-butyl-2-cyanoacrylate). This vascular tumor had no intracranial vascular communication as assessed by pre-embolization MRI and catheter angiography. All embolizations were performed by direct percutaneous injection. One week following the last embolization procedure the child presented with a 24-hour history of ataxia and extrapyramidal tremor. He was diagnosed with a possible immune-mediated reaction to Onyx or Glubran, which was treated with an urgent surgical excision of the hemangioma followed by intravenous administration of immunoglobulin and steroids. To the authors' knowledge, this is the first case of possible immune-mediated toxicity secondary to either Onyx or Glubran administration. This case highlights the need for awareness of potential toxic reactions to these embolic agents in the treatment of hemangiomas in the pediatric patient.
 ...
PMID:Possible toxicity following embolization of congenital giant vertex hemangioma: case report. 2793 67

A 33-year-old male presented with sudden onset of dysarthria. MRI showed a single round lesion containing hematomas in varying stages combined with venous malformation in the superior midline portion of the midbrain, indicating a midbrain cavernous angioma. Serial follow-up MRI revealed enlargement of the angioma concomitant with worsening of the dysarthria, ataxia, and intention tremor. Preoperative MRI suggested that the angioma consisted of a cystic hemorrhagic lesion with an 18-mm diameter without hydrocephalus. Since the angioma was located just beneath the floor of the midline portion of the third ventricle, we chose an anterior interhemispheric transcallosal transforaminal approach. After callosotomy, the foramen of Monro was widened by dissecting the choroidal fissure, enabling entry into the third ventricle. The lower part of the massa intermedia was cut;the median floor of the third ventricle was dissected and the angioma was removed. After the surgery, only a transient complication of seesaw nystagmus was observed, caused by damage to the interstitial nucleus of Cajal. As the anterior interhemispheric transcallosal transforaminal approach does not damage both forces, this technique may be a safe and useful approach for superior medial midbrain lesions, located just beneath the floor of the third ventricle.
 ...
PMID:[Surgical Removal of a Superior Medial Midbrain Cavernous Angioma through the Anterior Interhemispheric Transcallosal Transforaminal Approach:A Case Report]. 3283 Jan 37


<< Previous 1 2 3