Yoshito Uchihashi

Germinoma of the Basal Ganglia

We describe a case of germinoma of the left basal ganglia. An 11-year-old boy, who demonstrated calcification of the left basal ganglia on CT scan following a head injury at the age of 3 years, presented with a weakness of the right upper extremity for 2 months. MRI demonstrated high intensity in the left basal ganglia on a T1-weighted image without enhancement as well as high intensity on a T2-weighted image. Ipsilateral hemiatrophy of the hemisphere and midbrain was also noted. In addition, high intensity in the left internal capsule and cerebral peduncle was demonstrated on T2-weighted image. Surgical specimens obtained by stereotactic biopsy showed germinoma with a two-cell pattern. The patient had remained asymptomatic for 8 years after abnormal calcification was initially detected on CT scan. Ipsilateral hemiatrophy of the hemisphere and midbrain was demonstrated before the onset of weakness.

Significance of blood aspiration in carotid artery stenting with Angioguard XP

BACKGROUND AND PURPOSE In some patients, angiographic flow impairment is observed during carotid artery stenting (CAS) using Angioguard XP (AGXP), resulting in neurological symptoms. CAS was thus modified to improve clinical outcome. METHODS Ninety-seven patients were treated with CAS using AGXP from January 2008 to October 2009. In period I (January-December 2008; n = 53), blood aspirations were performed only in no-flow cases. In period II (January-October 2009; n = 44), blood aspirations were performed in no-flow and slow-flow cases. Clinical outcome, detection of microembolic lesions on diffusion-weighted imaging (DWI) and flow impairment during CAS were examined between these two periods before and after modifying the CAS procedure. RESULTS Periprocedural transient ischemic attacks occurred in 10 patients (18.9%) and one patient (2.27%) in periods I and II, respectively (P = .018). Minor and major strokes were observed in two patients in each period (P = .849). New ipsilateral DWI lesions were detected in 25 patients (47.2%) and 11 patients (25.0%) in periods I and II, respectively (P = .024). Among 18 slow-flow cases, new DWI lesions were detected in one patient (9.09%) and five patients (71.4%) with (n = 11) and without (n = 7) blood aspirations, respectively (P = .013). Neurological symptoms were observed only in three of seven patients (42.9%) without aspirations, compared to one of 11 patients (9.1%) with aspirations (P = .043). CONCLUSION Postoperative symptomatic stroke and new DWI lesions are significantly associated with blood flow impairment during CAS using AGXP. When flow impairment occurs, blood aspiration should be performed.

Severe inhibitor-negative acquired factor XIII/13 deficiency with aggressive subdural haemorrhage.

Acquired factor XIII (FXIII) deficiency is a common disease and seldom causes bleeding. However, severe FXIII deficiency may result in life-threatening bleeding. Although the inhibitor against FXIII has recently been focused as the cause of haemorrhagic acquired FXIII deficiency, the pathophysiology of inhibitor-negative cases could also be involved. We report a case of an 85-year-old Japanese man with serious subdural haemorrhage showing a remarkable decreased level of FXIII activity. He also manifested complications of compensated disseminated intravascular coagulation (DIC) with chronic renal failure, abdominal aortic aneurysm (AAA) and right renal carcinoma. Despite the successful evacuation of the haemorrhage, acute subdural haemorrhage subsequently developed that necessitated further craniotomies. Plasma cross-mixing studies and dot blot assay revealed no inhibitors against FXIII. We speculated that the decreased FXIII activity could be mainly due to hyperconsumption by DIC and surgery. Because plasma-derived FXIII concentrates are available to stop bleeding, clinicians should be aware of severe acquired inhibitor-negative FXIII deficiency in cases of unexplained excessive bleeding.

A 40‐Year‐Old Female with Leptomeningeal Lesions

A 40-year-old right-handed female first noticed 6 months ago headache and dizziness. One month later, she complained of repeated vomiting and frequent headache. She was hospitalized at another medical facility. MRI examinations revealed multiple contrast enhanced lesions over the surface of cerebral hemispheres (Figure 1a), cerebellum, brainstem (Figure 1b) and cervical spinal cord (Figure 1c). Laboratory examinations showed no abnormality in the white blood cell (WBC) count, C-reactive protein (CRP), or the tumor markers such as CEA, CA19-9 and SCC. Lumbar punctures revealed an increased opening pressure of 280 mm H2O. Analysis of the cerebrospinal fluid (CSF) disclosed mildly elevated cell counts (22 cells/mm; 86% mononuclear, 14% polymorphonuclear), a severely decreased concentration of glucose (4 mg/dl) and elevated level of protein (446 mg/dl). A polymerase chain reaction (PCR) test for tuberculosis DNA in the CSF was reported to be normal, and negative result of tuberculosis cultures. No clinical improvement was observed despite a trial of steroid therapy. Because these studies did not reveal a diagnosis and she became tetraparetic, an open biopsy was performed through a left temporal craniotomy 4 months after the onset of initial symptom. At surgery, milky opacification of the leptomeninges was observed at the convexity of the brain and they were biopsied with adjacent brain tissue. Five months after the onset of the disease, she was transferred to our hospital. At this time, she showed tetraparesis (Manual Mascle Test (MMT): Upper extremity = 3/5, Lower extremity = 1/5), dysphagia, right hearing disturbance, left oculomotor paralysis, sensory disorders of the bilateral lower extremities, and bladder and rectal disturbances. The Karnofsky Performance Scale (KPS) was 40.