Cerebellar haemorrhage as a cause of neurogenic pulmonary edema-case report

The neurogenic pulmonary edema is a rare clinical situation caused by an imbalance characterized by an excessive sympathetic outflow. It is observed mostly in young patients, is associated with brain or spinal cord haemorrhage, trauma, tumours or infections and is usually fatal. A case of neurogenic pulmonary edema in a 27-year-old woman is presented, caused by a cerebellar haemorrhage due to a vermian and paravermian arteriovenous malformation rupture. The vermian and hemispheric haemorrhage injuring the sub-lobule IX-b of the uvula induced a disruption of both carotid baroreceptor and chemoreceptor reflexes control mechanisms. Medical treatment with controlled ventilation, PEEP, diuretics and morphine reverted the pulmonary edema. After surgical treatment of the haemorrhage and cerebellar AVM the patient recovered to an almost normal social and professional life. The cerebellar lesion induced a temporary vermian sub lobule IX-b dysfunction that was responsible for the sympathetic storm that evoked the neurogenic pulmonary edema.     

Essential thrombocythemia: Rare cause of chorea

Essential thrombocythemia (ET) is a clonal myeloproliferative disorder (MPD), characterized predominantly by a markedly elevated platelet count without known cause. It is rare hematological disorder. In ET clinical picture is dominated by a predisposition to vascular occlusive events and hemorrhages. Headache, transient ischemic attack, stroke, visual disturbances and light headedness are some of the neurological manifestations of ET. Here, we describe a 55 year-old female who presented to us with generalized chorea. On evaluation, she was found to have thrombocytosis. After ruling out the secondary causes of thrombocytosis and other MPD we confirmed diagnosis of ET in her by bone marrow studies. Polycythemia vera (PV) another MPD closely related to ET may be present with generalized chorea. There are few case reports of PV presenting as chorea in the literature, but none with ET. We report the first case of ET presenting as generalized chorea.     

Straight sinus stenosis as a proposed cause of perimesencephalic non-aneurysmal haemorrhage.

Perimesencephalic nonaneurysmal haemorrhage (PMNH), a term first coined in 1985, is a diagnosis of exclusion. It is considered a benign form of subarachnoid haemorrhage with a good prognosis and a low risk of rebleeding. Its precise aetiology has not yet been determined. Haemorrhage of venous origin has been proposed as a cause, and has been demonstrated to be associated with venous abnormalities of the basal vein of Rosenthal. We present a patient with PMNH in whom an 80% stenosis at the union of the vein of Galen with the straight sinus is demonstrated. We thus propose hypertensive venous rupture secondary to venous sinus stenosis as an alternative cause for PMNH.     

Senile chorea: a multicenter prospective study

Senile chorea (SC) is characterized by the presence of late onset, generalized chorea with no family history and no dementia. It is unclear whether it is a distinct clinical entity or represents late onset Huntington's disease (HD) with an undetected family history. In order to clarify this issue, we carried out a prospective, multicenter study of suspected cases of SC. Since 1994 we identified six cases that met clinical criteria for SC. Their study included routine lab tests, cerebral MRI, neuropsychological assessment, and lastly gene IT15 analysis. An abnormal expansion of the (CAG)n repeat was found in three patients. Although there were no criteria for dementia, most neuropsychological tests revealed mild to moderate deficits, particularly in visuospatial and prefrontal tasks, in all six patients, those that were finally diagnosed as having late onset "sporadic" HD, but also in patients that finally had SC. This study provides further evidence on the existence of SC; however, the distinction from late onset "sporadic" HD seems not to be possible on clinical grounds unless a genetic study is carried out. Some cases of suspected "SC" have late onset "sporadic" HD.     

Non-ketotic hyperglycaemic chorea: a SPECT study.

OBJECTIVE: To study regional blood flow of the striatum in non-ketotic hyperglycaemic choreic patients. METHODS: Brain SPECT was performed with intravenous injection of 20 mCi 99mTc hexamethylpropylene amineoxime in six non-ketotic hyperglycaemic choreic patients and 10 age matched patients with a similar level of hyperglycaemia without chorea as a control. The focal perfusion defects were analysed by visual interpretation and semiquantitative determination with reference to homolateral occipital blood flow. RESULTS: The measured blood flow of striatum on the contralateral side of chorea was significantly decreased. CONCLUSIONS: Hypometabolism of the striatum is seen not only in Huntington's disease, but also in non-ketotic hyperglycaemic chorea. Hypofunction of the striatum is a possible common pathogenesis in the development of contralateral chorea in different diseases. Furthermore, the sensitivity and reliability of SPECT is as good as PET in assessing choreic patients.     

Histological verification of microaneurysms as a cause of cerebral haemorrhage in surgical specimens.

Surgical specimens taken from 14 patients with lobar intracerebral haemorrhage or cerebellar haemorrhage without vascular abnormalities on angiograms were examined histologically. In seven of the 14 patients, arteriovenous malformation or amyloid angiopathy were found by ordinary pathological examinations. Among the remaining seven patients, definite microaneurysms were verified in five and possible ones in two patients by using the technique of serial sectioning of the solid nodular tissues removed from the presumed bleeding site, where an arterial connection between the tissues and the surrounding brain was noted. Four of these seven patients had no history of hypertension and showed normal blood pressure before and after surgery. To verify microaneurysms in surgical specimens, it seems important to search the presumed bleeding site properly by a meticulous microsurgical technique and to section the tissues serially for the histological examination.     

Churg-Strauss, a rare cause of intracerebral haemorrhage

Intracerebral haemorrhage (ICH) is a common and devastating condition. We describe a 43-year-old man with the rare Churg-Strauss syndrome, which was diagnosed after he presented with an ICH. He was managed initially neurosurgically with clot evacuation and craniectomy. Post-investigation and diagnosis of Churg-Strauss, treatment was with high dose methylprednisolone (then oral prednsiolone) and intravenous cyclophosphamide. Clinicians should be aware that Churg-Strauss syndrome can, rarely, be a cause of ICH. Given its high morbidity and mortality, it is important that patients are diagnosed promptly and managed appropriately.     

An unusual cause of recurrent chorea.

Recurrent chorea is described in a 61-year-old woman who had had chorea gravidarum when she was younger. The recurrent chorea appeared to be induced by a topical vaginal cream that contained conjugated estrogen. This case is consistent with the existence of a recurrent syndrome of hormone-induced chorea. The effect of estrogen on the basal ganglia is complex and not fully understood.     

Subarachnoid haemorrhage of unknown cause: a long term follow-up.

Three hundred and sixteen patients who suffered a subarachnoid haemorrhage of unknown cause were followed up by questionnaire after 2 to 23 years, the average interval being 10 years. Two hundred and fifty one (79.5%) are known to be alive, 49 (15.5%) had died, and 16 (5%) patients were untraceable. The cumulative survival for the whole group after 22 years was 69%, the expected survival being 89%. The certified cause of death of 30 patients was from diseases of the circulatory system, mainly myocardial infarction and cerebrovascular disease, the expected number being 21.8. The results of this survey show a reduced life expectancy for both males and females and suggest that a more guarded view should be taken than is presently the case about the long term prognosis of these patients.     

Camel racing: a new cause of extradural haemorrhage in Australia

Camel racing is a relatively new sport in Australia. A 52 year old woman fell from her camel during a country race. Although she was wearing an approved equestrian helmet, she suffered a skull fracture and a life-threatening extradural haematoma. Her treatment highlights the key issues of management of head injuries in remote places. A paramount requirement is close collaboration between country medical practitioner, neurosurgeon and retrieval specialist.     

Huntington''s chorea: a prevalence study in the Florence area

A prevalence study of Huntington's chorea in Florence area in the period 1970-1979. Two clusters have been identified and the frequency of subjects heterozygote for Huntington's gene has been determined for planning Public Health intervention and the preparation of correct genetic counselling.     

非酮症性高血糖舞蹈症七例临床及神经影像学特点

目的 探讨非酮症性高血糖舞蹈症的临床及神经影像学特点.方法 对7例非酮症性高血糖舞蹈症患者进行临床及颅脑CT和MRI检查,分析其临床及影像学特征.结果 7例患者均有糖尿病病史,平素血糖控制不良,发病时血糖较高而酮体正常,表现为单侧肢体、双侧肢体或全身舞蹈样动作.颅脑CT和MRI可见单侧或双侧基底节区异常病灶.单纯药物控制舞蹈症效果不佳,降低血糖后舞蹈症状和神经影像改变可很快恢复,不留后遗症.结论 非酮症性高血糖舞蹈症多见于年龄较大的糖尿病患者,可能与大脑基底核在高血糖状况下脑细胞代谢出现异常有关.颅脑CT或MRI改变具有特征性.本病是可逆性的,对治疗反应较好,一般不留后遗症.