Idiopathic pure sudomotor failure: anhidrosis due to deficits in cholinergic transmission.

BACKGROUND: Acquired idiopathic generalized anhidrosis (AIGA) represents a heterogeneous clinical syndrome including sudomotor neuropathy and failure of the sweat glands. However, most AIGA cases comprise idiopathic pure sudomotor failure (IPSF), a distinct subgroup without sudomotor neuropathy or sweat gland failure. METHODS: Eight patients with IPSF (mean +/- SD age 20 +/- 5 years) were assessed by thermoregulatory and pilocarpine-induced sweating tests, as well as emotional sweating using sudorometer (4 cases), microneurography of skin sympathetic nerve activity (2 cases), and skin biopsies from the forearm or axilla (3 cases). RESULTS: Clinical features of IPSF comprise early onset; acute or sudden onset; concomitant sharp pain or cholinergic urticaria over the entire body; lack of autonomic dysfunction other than generalized anhidrosis; elevated serum IgE levels; and marked response to steroid. Sudomotor function testing revealed complete absence of thermoregulatory sweating, but well-preserved emotional sweating; pilocarpine did not induce sweating, and microneurography revealed that bursts of skin sympathetic nerve activity were not decreased; and skin biopsy displayed no morphologic abnormalities in sweat glands. The first two findings suggest lesions on the postsynaptic side of the nerve-sweat gland junction. CONCLUSION: The lesions in IPSF may be in the muscarinic cholinergic receptors of sweat glands. Allergic mechanisms are probably involved in its pathophysiology.

[A case of a persistent primitive proatlantal intersegmental artery with a ruptured basilar bifurcation aneurysm].

A case of persistent primitive proatlantal intersegmental artery (PPPIA) associated with a ruptured basilar bifurcation aneurysm was reported. A 44-year-old male with sudden headache was admitted to our hospital. CT scan revealed subarachnoid hemorrhage. Cerebral angiography revealed anomalous anastomosis between the internal carotid artery and the vertebral artery at the proatlantal region. This anastomosis branched off from the left internal carotid artery at the C4 level and joined the horizontal portion of the left vertebral artery. It was thought to be PPPIA. Angiography also revealed an aneurysm of the basilar bifurcation which was responsible for the patient's subarachnoid hemorrhage. The aneurysm was successfully treated by endovascular embolization with Guglielmi detachable coils in an acute stage, and resulted in good outcome. PPPIA with basilar bifurcation aneurysm has not been presented or reported in the literature to date. To our knowledge, this is the first report of such an association of vascular anomalies. The frequency of PPPIA combined with the intracranial aneurysm is relatively high, whereas the occurrence of PPPIA is extremely rare. Therefore, it was suggested that some congenital and/or hemodynamic factors changed by PPPIA may affect the pathogenesis of intracranial aneurysms.

Carrier-mediated transport systems for glucose in mucosal cells of the human oral cavity.

The in vitro uptake study was performed using the isolated cells of human oral mucosa, buccal and the dorsum of the tongue, to investigate the mechanisms of glucose uptake. The uptake of D-glucose was much larger in cells of the dorsum of the tongue than in buccal cells and was inhibited more extensively by 2-deoxy-D-glucose, a substrate of facilitative glucose transporters, than by alpha-methyl-D-glucoside, a specific substrate of SGLT1, suggesting the larger contribution of a facilitative transporter than Na(+)/glucose cotransporter. Furthermore, from the results of inhibition studies by several sugar analogues including maltose and D-mannose, GLUT1 and/or GLUT3 were suggested to take part in the glucose uptake by oral mucosa. Therefore, we have attempted to confirm the expression of glucose transporters on the oral mucosa by employing Western blotting. As a result, it was suggested that SGLT1, GLUT1, GLUT2, and GLUT3 are expressed in the epithelial cells of human oral mucosa.

Primary intracranial squamous cell carcinoma--case report.

A 50-year-old female presented with primary intracranial squamous cell carcinoma (SCC) at the right cerebellopontine angle manifesting as right facial nerve paresis. She had undergone gross total removal of a right cerebellopontine angle epidermoid cyst 10 years before and had done well until recently. Magnetic resonance imaging showed a heterogeneous tumor with markedly enhanced irregular margin. Subtotal removal of the tumor was achieved. Histological examination showed moderately differentiated SCC. After surgery, she underwent chemotherapy and gamma radiosurgery. She is now well 5 years after the diagnosis of SCC.

Significance of acute cerebral swelling in patients with sylvian hematoma due to ruptured middle cerebral artery aneurysm, and its management.

A retrospective study of 75 patients treated surgically for ruptured middle cerebral artery (MCA) aneurysm within 48 hours evaluated clinical grade at admission, secondary development and management of cerebral swelling associated with space-occupying hematoma, cerebral infarction caused by vasospasm, development of hydrocephalus, and clinical outcome. Clinical grade at admission was significantly better in patients without than in those with hematoma (p < 0.01). Twenty-seven patients with sylvian hematoma caused by ruptured MCA aneurysm often developed ipsilateral cerebral swelling in the early period after subarachnoid hemorrhage. Seventeen of these patients developed serious cerebral swelling and received barbiturate therapy. Nine of these 17 patients had good outcome, but six patients died of cerebral swelling. The incidence of hydrocephalus was significantly higher in patients with than in those without hematoma (p < 0.01). The incidence of infarction was more pronounced in patients with sylvian hematoma. Clinical outcome was significantly better in patients without than in those with sylvian hematoma (p < 0.01). Development of cerebral swelling in patients with sylvian hematoma due to ruptured MCA aneurysm has a significant effect on outcome, and improvements in management are required.

Clinical analysis of recurrent subarachnoid hemorrhage after neck clipping surgery.

The clinical features of recurrent subarachnoid hemorrhage (SAH) after neck clipping surgery were investigated in a series of 1,436 consecutive patients treated between 1980 and 1994, and seven patients treated prior to 1980. Recurrent SAH occurred within 1 month in seven patients and between 1.5 and 20 years in 20 patients (mean interval 9.2 years) from the first surgery. The patients were aged from 31 to 76 years (mean 49.8 years) at the first SAH. There were 19 females and eight males. Recurrent SAH occurred at the same site as the prior aneurysms in 12 cases, at an infundibular dilatation in three cases, de novo aneurysms in nine cases, untreated multiple aneurysms in two cases, and unknown in one case. The main causes for early recurrent SAH were incomplete clipping or untreated multiple aneurysms, whereas late recurrent SAH was due to de novo aneurysms, untreated multiple aneurysms, or regrowth aneurysm at the prior site. The outcomes of late recurrent SAH were good in eight cases, moderate disability in two, severe disability in three, and dead in seven, whereas most cases of early recurrent SAH resulted in poor outcome. Immediate postoperative angiography is desirable in cases with incomplete clipping, because early recurrent SAH resulted in poor outcomes. De novo or regrowth aneurysms caused late recurrent SAH, so follow-up angiography is strongly recommended for young patients, even if complete clipping was achieved.

Correlation between blood parameters and symptomatic vasospasm in subarachnoid hemorrhage patients.

Serial changes in platelet and white blood cell (WBC) counts and other blood parameters were analyzed in 103 patients with aneurysmal subarachnoid hemorrhage (SAH). The WBC counts during days 3-5, 6-8, 9-11, and 12-14 after the onset of SAH were significantly higher in patients with than in patients without symptomatic vasospasm. Platelet counts during days 0-2, 3-5, 6-8, 9-11, 12-14, 15-17, 18-21, and 22-28 after SAH were significantly higher in patients with than in patients without symptomatic vasospasm. Monitoring of platelet and WBC counts may provide an indicator of the occurrence of symptomatic vasospasm.

[Treatment of facial arteriovenous malformation].

Arteriovenous malformations (AVMs) of the face are notoriously difficult lesions to manage because of the abundant vascular network in this region. We report three cases in which AVMs of the face were treated with endovascular embolization. Two cases with small AVM of the face and auricle were treated with direct puncture of the nidus and embolization with a small amount of n-butyl-cyanoacrylate (NBCA). In the third patient with a large AVM of the cheek, the external carotid artery (ECA) of which had been previously ligated surgically, percutaneous direct puncture of the ECA was performed. Superselective catheterization of each of the feeding arteries and embolization with a small amount of NBCA was then achieved. All cases demonstrated clinical and angiographical improvement. In two cases with a small AVM of the face and auricle, complete obstruction of the nidus was achieved. Direct puncture of the nidus and embolization with a small amount of NBCA was a safe and effective technique for the treatment of facial AVM.

[Vertebral artery injury following mild neck trauma: report of two cases].

Two cases of vertebral artery injury following mild neck trauma are reported. A 52-year-old man was hospitalized with gait disturbance 7 days after mild traffic accident. Right vertebral angiogram revealed complete occlusion of the rt. vertebral artery (VA) and MR images revealed infarction in the rt. cerebellar hemisphere and rt. dorsolateral part of the medulla oblongata and revealed the thrombus in the rt. VA. He underwent anticoagulation and became asymptomatic. Angiogram 6 months later revealed the vessel to be normal. A 23-year-old man who has a habit of self-manipulation of his neck was hospitalized on the day when he experienced dysesthesia in the left part of his face and left upper and lower extremities and unsteady gait. MR images revealed multiple infarction in bilateral cerebellar hemispheres and thrombus in bilateral VAs. Bilateral vertebral angiogram revealed severe stenoses of bilateral VAs. He underwent anticoagulation and wore soft collar. Angiogram 20 days after onset revealed improvement of bilateral VA stenoses. He was discharged with no neurological deficit. It is said that vertebral artery injuries in association with head and neck trauma are relatively rare, but this condition is possible to be more common than realized, considering that the case of unilateral VA occlusion or the case with well developed collateral circulation is sometimes well tolerated for ischemia and that this condition can occur even after mild head and neck injury. The diagnosis must be established by vertebral angiogram, but MRI and MRA are very useful as ancillary methods. The therapeutic point is to prevent propagation of the thrombus and distal embolism, accordingly wearing a collar and anticoagulation are important.

[Cavernous angioma in the cerebellopontine angle: a case report].

A rare case of cavernous angioma (CA) in the cerebellopontine angle (CPA) is reported. A 60-year-old female suffered from a right progressive sensorineural hearing loss and a successive right facial paresis over 2 years. A small mass was detected in her right CPA on CT scans. Both T1- and T2-weighted MR images demonstrated an intracanalicular lesion protruding into the CPA as being hyperintense. A small red colored lobulated tumor involving the 7th and 8th cranial nerves was found in the CPA through the suboccipital approach. The tumor contained multiple small hematomas in various stages. Biopsy with evacuation of these hematomas was selected to avoid damaging the cranial nerves. Histological examination of the specimen disclosed it as CA. Postoperatively her facial paresis improved slightly, but her hearing loss remained unchanged. Discussions were carried out concerning clinical and neuroradiological features of CA in the internal auditory canal and CPA. The present case and a previously reported 17 cases were the subjects under discussion.

Clinical analysis of a series of vertebral aneurysm cases.

We reviewed 38 cases of aneurysms of the vertebral artery treated over the last 10 years: 26 (68%) located at the junction of the vertebral and posterior inferior cerebellar arteries, 10 (26%) at the vertebral artery, and 2 (5%) at the vertebrobasilar union. There were three distinct forms of aneurysms: 20 saccular (53%), 10 fusiform (26%), and 8 dissecting (21%). Among these 38 aneurysms, 33 (87%) had ruptured: 18 of the saccular aneurysms (90%), all 10 of the fusiform aneurysms (100%), and 5 of the dissecting aneurysms (63%). Computed tomography of the 28 ruptured aneurysms revealed diffuse subarachnoid hemorrhage in the basal cistern combined with intraventricular hemorrhage in 24 cases (86%). Magnetic resonance imaging was useful for differentiating between fusiform and dissecting aneurysms. Abnormalities such as a double lumen of the vertebral artery were demonstrated in four of the dissecting aneurysms. The overall surgical results were good for 22 of the 27 surgically treated cases (81%). New bleeding was observed in 8 (24%) of the 33 ruptured aneurysms. The rate of new bleeding was high (60%) in the patients with dissecting aneurysms, and occurred mostly in the acute stage. The incidence of vasospasm was 27%, and only two patients suffered permanent neurological deficits. These findings indicate that the rate of new bleeding tends to be high in patients with saccular and dissecting aneurysms, and thus, they should be treated as early as possible. A preoperative balloon occlusion test should be conducted if proximal occlusion of the vertebral artery is necessary, since proximal occlusion is not always safe, despite angiographic evidence of sufficient contralateral arterial flow.

[Peripheral aneurysms of the posterior inferior cerebellar artery; analysis of 15 cases].

Peripheral aneurysms of the posterior inferior cerebellar artery (PICA) are rare. The authors report 15 cases of peripheral PICA aneurysms. Twelve of the aneurysms were discovered after their rupture and two were discovered after rupture of concomitant AVM, and one was detected incidentally. As to the location of aneurysms, more than half of the aneurysms arose at the telovelotonsillar segment. One patient had 2 aneurysms on the same peripheral PICA, which were not obvious on preoperative angiography because the ruptured one was partially thrombosed. Another rare case with aneurysm located at the internal auditory meatus is also reported. The findings of CT were characteristic, namely hemorrhage in the fourth ventricle without obvious hemorrhage in the basal cisterns, or only within the cerebellar vermian cistern. This phenomenon strongly suggested the possibility of rupture of peripheral PICA aneurysms. As to the surgical results, 10 patients (71%) of the 14 patients had a good recovery, and 1 patient (7%) with nonrupture aneurysm had a fair result due to pre-existing pontine hemorrhage. Three patients (21%) died. Rebleeding occurred in 3 patients, but cerebral vasospasm occurred in only 1 patient. Consequently, as peripheral PICA aneurysms are likely to be missed, and rebleeding is an unfavorable factor, one should try to demonstrate aneurysms with angiography, by using various methods such as subtraction or magnification.

Brain tumors manifesting as intracranial hemorrhage.

The clinical course and computed tomographic (CT) findings of 23 patients with brain tumors manifesting as tumoral hemorrhage were reviewed. The most common symptoms were headache and clouding of consciousness. A CT finding of a lesion located next to a solid or irregular clot indicated intratumoral hemorrhage. Precontrast CT demonstrating an indent on the hematoma surface was a valuable indicator of tumoral hemorrhage. A CT finding of accumulated levels of blood/fluid or a hyperdense mass containing small hematoma indicated intratumoral hemorrhage, and obscure hyperdensity indicated intratumoral hemorrhagic infarction. Such findings were often difficult to distinguish from spontaneous intracerebral hemorrhage due to other factors. The incidence of rebleeding from residual tumors was high, carrying a very poor prognosis, so radical removal of brain tumors with hemorrhage is very important.

[Familial cavernous angioma of the central nervous system--report of a family and review of literature].

In this paper we reported a familially occurred cavernous angiomas (CA) of the central nervous system (CNS). Case 1 was a 14-year-old female with an initial symptom of transient motor and sensory disorders of the right upper extremity. On examination she had a cutaneous angioma and no neurological deficit. CT and MRI revealed two lesions in the brain. These lesions were removed surgically and were diagnosed histologically as CA. Case 2 was a 45-year-old female, the mother of case 1, with a long standing headache. She had a cutaneous angioma and no neurological deficit. CT and MRI revealed a CA in the brain and a one in the cervical cord. Case 3 was an 11-year-old female, the cousin of case 1, with an initial symptom of left hemiparesis. On examination, she had a cutaneous angioma and left hemiparesis. CT revealed two lesions in the brain. These lesions were removed surgically and were diagnosed histologically as CA. Familial CA of the CNS is relatively rare and the reported cases were 24 families consisted of 70 cases. We analyzed the reported cases to clarify the specificities of the familial cases. In the familial cases, CAs were apt to be multiple and were located more frequently in the posterior fossa than in the non-familial cases. In some familial cases, the vascular anomalies of the CNS other than CA or CA of the skin or retina were also revealed. Because of high incidence (89%) of the hemorrhage from the histologically verified CA, operation should be carried out even in the cases of CA with mild or no symptom, if the lesion is accessible surgically.

MRI demonstration of Kernohan's notch: case report.

We report a left acute subdural haematoma caused by rupture of a left anterior cerebral artery aneurysm, which progressed to left hemiparesis. Coronal MRI was superior to CT for visualizing Kernohan's notch.

[Pulmonary embolism complicated with stroke: analysis of 5 cases].

Though pulmonary embolism (PE) has been thought to be rare, the incidence seems to be increasing recently. During the past 10 years the authors have encountered 5 cases of PE among stroke patients. There were 2 males and 3 females, aged 51 to 71 years (mean age; 63 years). The mean time between admission and onset of PE was 23 days. As to the primary disease to be treated, 5 patients had subarachnoid hemorrhage and one had intracerebral hemorrhage. Generally, PE tends to be overlooked or misdiagnosed because of the fact that stroke patients are often in a state of unconsciousness. In our series, only one patient complained of dyspnea and the other 4 patients due to unexplained sudden tachycardia, tachypnea and hypoxemia were suspected to have PE. Deep venous thrombosis known as the risk factor leading to PE was presented in 3 patients. Especially in one patient, femoral venous catheterization was considered as a risk factor possibly leading to deep venous thrombosis. Regarding the diagnosis of PE, the roles of electrocardiogram and of chest x-ray film were small. In 3 patients, the elevation of the diaphragm was the only abnormal finding on chest X-ray. On the other hand, the lung scintigram with 99mTc-MAA was a useful method for definitive diagnosis of PE. In 3 patients, filling defects were demonstrated on the lung perfusion scintigrams. Consequently, we emphasize that PE must be kept in mind when tachycardia, tachypnea and hypoxemia appear suddenly. Prompt diagnosis and treatment are required.(ABSTRACT TRUNCATED AT 250 WORDS)

[Fusiform and dissecting aneurysm of the vertebrobasilar systems: differential diagnosis].

Fusiform aneurysms (FA) and dissecting aneurysms (DA) of the vertebro-basilar systems have been thought to be rare, but recently reports of these aneurysms have increased. The differential diagnosis between FA and DA is requisite for deciding therapy or for prognosis, but it is often difficult to distinguish these aneurysms even if angiographies are conducted. The authors have treated 6 cases with FA and 5 cases with DA during the past 17 years. As the initial symptom, subarachnoid hemorrhage (SAH) was noted in all 6 cases with FA. On the other hand, 2 cases of SAH, 2 cases of brain stem infarction, and 1 case of ischemic attack were noted in DA cases. The aneurysmal locations of FA were at the vertebral artery (VA) in all 6 cases, and those of DA were at the VA in 3 cases and at the basilar artery (BA) in 2 cases. As angiographic findings of DA, pearl & string signs were demonstrated in 3 cases, and retention of contrast medium was noted in 3 cases. Diagnosis of FA is comparatively easy on angiography but when angiospasm exists, it is difficult to differentiate DA from FA. Consequently, repeated angiography is recommended. MR imaging findings of 2 cases with FA were compared with those of 3 cases with DA. No abnormal findings, excepting a dilatation of the signal-void area corresponding to the arterial blood flow were shown in FA, but various abnormalities were detected in all of the 3 cases with DA. Namely, an intimal flap and a double lumen in 1 case, intra-mural hematoma in 1 case, and a hematoma adjacent to the parent artery in 2 cases were demonstrated. Thus, MR imaging was thought to be a useful means for distinguishing between FA and DA.

Primary intracranial arachnoid cyst in the elderly: a survey on 39 cases.

Thirty-nine cases of primary intracranial arachnoid cyst in the elderly, including one case of our own, were analyzed. Clinical characteristics in these patients were as follows. (1) The number of patients decreased with age, but there were no differences according to sex. (2) Clinical manifestations were similar to those of chronic subdural haematoma or normal pressure hydrocephalus including dementia, urinary incontinence, and hemiparesis. General symptoms such as headache and seizures were also present. (3) Surgery was performed in most patients with generally good outcome regardless of operative procedures (capsular resection vs. shunt). (4) In some cases of advanced age, disease manifestation may have been due to slight head injuries.