Polyarteritis nodosa in association with subarachnoid hemorrhage.

We report a 65-year-old man with classic polyarteritis nodosa (PAN) who developed subarachnoid hemorrhage. Polyarteritis nodosa was strongly suspected, however, the biopsy specimens of kidney and sural nerve showed no findings of vasculitis and the serum titer of antimyeloperoxidase-antineutrophil cytoplasmic autoantibody (MPO-ANCA) was negative. Cranial magnetic resonance angiography showed no findings of aneurysms. He developed subarachnoid hemorrhage (SAH) during the course and died. Autopsy confirmed fibrinoid necrosis in the medium-sized artery of multiple organs. To our knowledge, this is the first report of a case of classic PAN accompanied by SAH in which MPO-ANCA proved negative.

[Headache and neck pain as only manifestations of spontaneous of the dissecting aneurysm vertebral artery--a case report].

A 57-year-old woman had noticed occipital headache and neck pain bilaterally 6 weeks before admission. The headache and neck pain persisted for 3 weeks, then disappeared. Cranial magnetic resonance (MR) image obtained 4 weeks after the onset demonstrated intramural hematoma adjacent to intracranial left vertebral artery. MR image did not show any abnormal lesions in the brain parenchyma. Although cranial MR angiography did not show any definite aneurysms, three-dimensional CT angiography (3D-CTA) obtained 6 weeks after the onset disclosed an aneurysm of the left vertebral artery. The vessel distal to the aneurysm was occluded. Because the left posterior inferior cerebellar artery originated from the aneurysm, we did not perform embolization using coils. Spontaneous dissecting aneurysm of the left vertebral artery was diagnosed based on the clinical, MR image, and 3D-CTA findings. The systolic blood pressure was maintained under 130 mmHg using antihypertensive agents. Thereafter, 3D-CTA obtained 6 months after the onset showed recanalization of the occluded vessel distal to the aneurysm. The size of the aneurysm was unchanged. During these 6 months, there were no ischemic or hemorrhagic stroke events. In the present patient, headache and neck pain were the only manifestations of spontaneous dissection of the vertebral artery.

[A case of primary HIV infection presenting as meningoencephalitis].

A 23-year-old man presented with disturbance of consciousness and convulsion. Two weeks prior to his admission, he had general arthralgia, retro-orbital pain, and body temperature increase to 40 degrees C. These symptoms persisted for two weeks. He was admitted to the hospital because of general convulsion, followed by disturbance of consciousness. On admission, he was deeply lethargic. Cerebrospinal fluid obtained on admission showed pleocytosis (247/mm3) and an increased level of protein. Electroencephalogram obtained on admission disclosed diffuse slow waves. Enhanced cranial MR image did not show any abnormal lesions in the cerebral parenchyma. The patient was diagnosed as having meningoencephalitis and the treatment consisting of aciclovir, CTRX, and methylprednisolone was undertaken. A test for human immunodeficiency virus (HIV) antibody was found to be positive on the second day of hospitalization. A Western blot was positive with bands at gp160 and P24 confirming HIV infection. Antibody titers of paired acute and convalescent sera including HSV, EBV, CMV, mumps, measles, and Japanese encephalitis did not show any significant increase. The patient became alert on the 3rd day of hospitalization. Electroencephalogram obtained on the 10th day of hospitalization was normal. Western blot, which was obtained 4 months later, was positive with bands at gp120, p68, p55, p52, gp41, p40, p34, p18, including gp160 and P24. He was diagnosed as having primary HIV infection. Meningoencephalitis was attributed to acute primary HIV infection. Acute HIV infection should be considered as an etiology of meningoencephalitis.

[A case of anterior cerebral artery dissection causing enlargement of infarction].

A 46-year-old woman presented with weakness in the right arm and leg. She had developed difficulty in moving the right arm and leg while exercising and had noticed headache simultaneously. On admission, she had hemiparesis of the right side. Angiography on day 1 disclosed irregularity of the left anterior cerebral artery (ACA). We started argatroban and aspirin to prevent exacerbation of ischemia. Diffusion-weighted MR image on day 2 disclosed acute infarction in the territory of the left ACA. When she became able to move her leg against gravity (MMT 3/5), we discontinued antithrobotic therapy on day 3. MRI on day 10 disclosed enlargement of the infarcted area. Angiography on day 16 disclosed stenosis and dilation corresponding to pearl and string sign involving the left ACA. Spontaneous ACA dissection was established based on clinical features and angiographic findings. Antithrombotic therapy in patients with intracranial artery dissection remains controversial because subarachnoidal hemorrhage can complicate the course due to rupture of dissecting aneurysm, for there is no external elastic lamina in intracranial arteries. However, some cases of ACA dissection have showen progression of ischemic symptoms and enlargement of the infarcted area during the acute or subacute phase.

[Prolonged headache six weeks before ischemic symptoms due to vertebral artery dissection--a case report].

A 43-year-old woman presented with weakness in her left arm and leg. She had noticed headache persisting for 2 weeks in her right occipital area 6 weeks prior to the onset of the weakness. Cranial diffusion-weighted MR image demonstrated acute infarction in the right medial part of medulla oblongata. Cerebral angiography revealed pearl and string sign in the right intracranial vertebral artery. Magnetic resonance T1-weighted image obtained when the patient complained of left-sided weakness showed a high intensity area in right vertebral artery corresponding to intramural hematoma. The diagnosis of spontaneous vertebral artery dissection was established based on clinical features, laboratory findings, MR images and angiographic findings. Generally, in patients with cervicocephalic artery dissection with ischemic onset, the occurrence of headache is either simultaneous with the ischemic symptoms, or several days prior to the ischemic symptoms. The present case showed that headache due to arterial dissection can occur several weeks prior the ischemic symptoms. Precise history of headache should be taken in patients with cervicocephalic artery dissection.

[A case of anterior cerebral artery dissection causing hemorrhagic infarction].

A 56-year-old man presented with weakness in his right arm and leg. Throbbing headache occurred several hours prior to his weakness. Brain CT obtained on day 3 demonstrated low density areas in the medial part of the left frontal lobe. Cerebral angiography on day 14 demonstrated dilatation and narrowing of the left anterior cerebral artery (ACA) corresponding to "pearl and string sign". The diagnosis of spontaneous ACA dissection was established with clinical features, laboratory findings, and angiographic findings. Antiplatelet therapy was undertaken for the prevention of ischemic events. Serial Brain CT demonstrated hemorrhagic change in the area of infarction. However, there was no definite clinical deterioration. Antiplatelet therapy was withdrawn after hemorrhagic change was noted. Cerebral angiography on day 35 showed improvement of both dilatation and narrowing. Possible reperfusion injury caused by absorption of intramural hematoma seems to be responsible for hemorrhagic change. In patients with cerebral infarction due to dissection of intracranial arteries, antithrombotic therapy is controversial as hemorrhagic complications including hemorrhagic infarction as well as subarachnoidal hemorrhage can occur. Further accumulation of cases is required.

Reversible cardiomyopathy as the autonomic involvement of neuroleptic malignant syndrome.

Autonomic disturbance develops commonly in neuroleptic malignant syndrome (NMS). However, the association of cardiomyopathy is rare. Takotsubo-shaped cardiomyopathy is a unique reversible heart syndrome involving autonomic disturbance and mimicking myocardial infarction. We report a 63-year-old woman who developed Takotsubo-shaped cardiomyopathy during the course of NMS. Our case suggests the occurrence of reversible cardiomyopathy as the autonomic involvement of NMS.

Marked diffuse dilations of the biliary tree associated with intrahepatic calculi, biliary sludges and a mucinous cyst of the pancreatic head in a 99-year-old woman.

A 99-year-old woman was admitted to Shizuoka Shimizu Municipal Hospital because of fever and anasarca. Imaging and laboratory tests showed pneumonia, urinary tract infection, and cardiac failure. The patient died 20 days after admission. An autopsy revealed marked diffuse dilations of the biliary tree ranging from the lower common bile duct to intrahepatic bile ducts. Intrahepatic calcium bilirubinate stones and biliary sludges were recognized within the dilated bile ducts. A unilocular cyst (2 cm in diameter) was present in the pancreatic head adjacent to the lower common bile duct, and it appeared to compress the common bile duct. Histologically, the walls of the dilated biliary tree showed proliferation of peribiliary glands, fibrosis, and infiltration of lymphocytes and neutrophils (cholangitis). The lumens of the dilated biliary ducts contained neutral and acidic mucins, fibrinous materials, bacteria, neutrophils, and Aspergillus fungi, in addition to the calculi and sludges. The background liver showed atrophy (400 g). The pancreatic unilocular cyst was composed of mucous columnar cells with a few infoldings, and the pancreas also showed foci of mucinous duct hyperplasia and ectasia; the pathological diagnosis of the cyst was cystic dilations of a pancreatic duct branch (mucinous ductal ectasia or mucinous cyst). Other lesions included aspiration pneumonia, emaciation, atrophy of systemic organs, gastric leiomyoma, serous cystadenoma of the right ovary, and arteriosclerotic nephrosclerosis. The present case suggests that a mucinous cyst of the pancreas may compress the biliary tree and lead to marked diffuse dilations of the biliary tree. Alternatively, the dilations of the bile ducts may be associated with aging or may be of congenital origin. The dilated bile ducts may, in turn, give rise to bacterial and fungal cholangitis and formation of biliary sludges and intrahepatic calcium bilirubinate stones.