Masashi Hamanaka

Characteristics of intracranial branch atheromatous disease and its association with progressive motor deficits

BACKGROUND Small deep brain infarcts are often caused by two different vascular pathologies: 1. atheromatous occlusion at the orifice of large caliber penetrating arteries termed branch atheromatous disease (BAD) and 2. lipohyalinotic degenerative changes termed lipohyalinotic degeneration (LD). We herein analyze and describe the characteristics of these 2 different pathologies. METHODS We studied 394 patients with penetrating artery territory infarcts in the territories of the lenticulostriate arteries and anterior pontine arteries. Radiologically defined BAD of the lenticulostriate arteries was defined as infarcts with size more than 10mm in diameter on axial slice and visible for 3 or more axial slices, and that of the anterior pontine arteries was defined as unilateral infarcts extending to the basal surface of the pons. Within each of the 2 territory groups, differences between BAD and LD were compared. RESULTS Ninety five patients in the lenticulostriate arteries group (36.1%) and 78 patients in anterior pontine arteries group (59.5%) were classified as BAD. Initial NIHSS, incidence of progressive motor deficits and poor functional outcome were significantly higher and incidence of concomitant silent lacunar infarcts tended to be lower in BAD than LD. In logistic regression analysis, BAD compared with LD was independently associated with PMD, in lenticulostriate arteries group (OR: 4.21, p=0.0001) and in anterior pontine arteries group (OR: 5.32, p=0.0018). CONCLUSIONS Radiologically defined BAD and LD had different characteristics. BAD was significantly associated with progressive motor deficits and considered as a major vascular mechanism of progressive motor deficits in penetrating artery infarcts.

A case of acute progressive myelopathy due to intravascular large B cell lymphoma diagnosed with only random skin biopsy

A 64-year old woman was admitted to our hospital with subacute onset paraparesis and sensory disturbance at a level below Th10. Spinal MRI showed a T2 weighted high-signal intensity lesion at a level from Th5 to Th12, and an abdominal CT showed a mass in the left kidney. Her paraparesis deteriorated rapidly, and administration of high dose methyl prednisolone followed by oral steroid therapy was started before obtaining of a definitive diagnosis. However her symptoms did not improve after the beginning of treatment. At the same time, a bone marrow puncture, and biopsies from kidney and spinal cord were performed. These biopsies demonstrated no clues, diagnostically. Therefore a random skin biopsy was performed at the five sites on the 17th day after the steroid dosage end. From this, pathological evidence of intravascular large B cell lymphoma (IVLBCL) was shown. For rapid diagnosis of acute myelopathy with mass lesion of another organ due to IVLBCL, a biopsy is taken not only from spinal cord or mass lesions, but is also taken of multiple sites in skin randomly. This must be performed without a delay before a sudden deterioration of neurologic symptoms can occur from ischemic events not responsive to steroid therapy.

[A case of motor dominant neuropathy and focal segmental glomerulosclerosis associated with Sjögren's syndrome].

A 49-year-old woman was admitted to our hospital with gradually progressive weakness of the limbs for about 20 days. She presented with weakness of the limbs, predominantly in the proximal portion, and slight dysesthesia of the limbs, predominantly in the distal portion. Repeated nerve conduction examination revealed axonopathy dominantly in the motor neurons. Therefore, we suspected her as having Guillain-Barré syndrome, and initiated intravenous administration of high-dose immunoglobulin. However, her symptoms progressed gradually and finally she found it difficult to walk. Her urine analysis simultaneously demonstrated albuminuria, and a kidney biopsy indicated focal segmental glomerulosclerosis. At that point, laboratory examination showed high levels of anti SS-A antibody and salivary gland biopsy revealed infiltration of a significant number of lymphocytes around the gland, which led to the diagnosis of Sjögrens syndrome. We considered the etiology of the neural and renal dysfunction as due to the inflammatory mechanism associated with Sjögrens syndrome. Therefore, we administered a second course of immunoglobulin therapy and steroid therapy, which included both pulse and oral administration. Her neurologic symptoms and albuminuria improved rapidly after steroid therapy. The present case indicates that both motor dominant neuropathy and focal segmental glomerulosclerosis can occur in patients with Sjögrens syndrome.

[Neurologist and emergency neuroendovascular revascularization -training programs for endovascular procedures-].

Emergency neuroendovascular revascularization is a reperfusion therapy for acute stroke. The operator for this therapy has to obtain a license as a specialist in endovascular procedures. For neurologists wishing to acquire this license, there are two kinds of training programs: full-time training and concurrent training. Full-time training was chosen by the first author of this review, while concurrent training will be performed by staff in the authors department. The advantage of full-time training is the acquisition of a lot of experience of various diseases that are treated with endovascular procedures and managed in the periprocedural period. However, full-time training has the disadvantages of a requirement to discontinue medical care of neurological diseases except for stroke and employment at a remote institution. The advantages and disadvantages of concurrent training are the reverse of those of full-time training. Neither training system can succeed without cooperation from Departments of Neurology in neighboring universities and the institutional Department of Neurosurgery. It is particularly important for each neurologist to establish a goal of becoming an operator for recanalization therapy alone or for all fields of endovascular procedures because training will differ for attainment of each operators goal.