Makoto Tadano

Increased brachial-ankle pulse wave velocity is independently associated with white matter hyperintensities.

Background: White matter hyperintensities (WMHs) are a risk factor for stroke. Their etiology is considered to be cerebral microvascular abnormality. However, the association between WMHs and arteriosclerosis is not yet clear. The aim of this hospital-based cohort study was to identify the arteriosclerotic characteristics associated with WMHs. Methods: We cross-sectionally included 240 consecutive patients with no history of stroke. We measured the brachial-ankle pulse wave velocity (baPWV), ankle brachial pressure index, and intima-media thickness of the common carotid artery, and we performed magnetic resonance brain imaging. WMHs were defined as periventricular hyperintensity (Fazekas grade ≧3) and/or separate deep white matter hyperintense signals (Fazekas grade ≧2). We determined the prevalence of WMHs, silent brain infarction (SBI), hypertension, hypercholesterolemia, diabetes mellitus, ischemic heart disease, and smoking. We compared 2 groups of patients, defined by the presence or absence of WMHs, using multiple logistic regression analyses. Results: In multivariable analysis, SBI (OR 3.38; 95% CI 1.52–7.72), hypertension (OR 2.23; 95% CI 1.03–5.15), female sex (OR 1.95; 95% CI 1.03–3.76), baPWV (OR 1.12; 95% CI 1.02–1.23), and age (OR 1.09; 95% CI 1.04–1.14) were independently associated with WMHs. Conclusions: An increased baPWV is associated with WMHs. Management of increased baPWV may help to prevent the progression of WMHs and stroke.

Elderly case of Dieulafoy's lesion in the jejunum presenting with repeated hemorrhagic shocks

A 72-year-old man with hypertension was admitted to our hospital because of temporary stupor. On admission, neurological examination and brain magnetic resonance imaging showed no remarkable findings. Hemoglobin concentration of the blood was 14.4 g/dL. He developed shock following melena (day 1). Hematological examination after the shock revealed hemoglobin of 7.6 g/dL. Blood transfusion was performed. Initial gastrointestinal endoscopy and colonoscopy performed 4 h after the shock revealed blood in the colon, but did not reveal mucosal changes and the bleeding source. A second gastrointestinal endoscopy performed after a second shock revealed faint backward flow of the intestinal bleeding from the jejunum and did not show bleeding source in the esophagus, stomach and duodenum (day 2). Computed tomography (CT) and angiography performed on day 2 revealed the bleeding source (Fig. 1a– d). Although radiologists performed transcatheter arterial embolization (Fig. 1e), the patient developed a third shock following melena (day 4). A third gastrointestinal endoscopy showed a Dieulafoy’s lesion (DL) in the jejunum (Fig. 1f,g). We applied two clips and an endoscopic local injection of hypertonic saline– epinephrine solution. The hemostasis was successful. We could not perform gastrointestinal endoscopy after the treatment because of disappearance of melena and patient refusal. As in a similar previous report, we speculate that the DL in our case healed and disappeared. However, this could not be proven. Dieulafoy’s lesion is a vascular malformation and a rare but potentially life-threatening disease that is responsible for 7% of upper gastrointestinal hemorrhage. DL in the small intestine is extremely rare, and the diagnosis is difficult. In addition, elderly patients with DL can have several complications such as cardiovascular disease, chronic renal disease and dementia. Repeated hemorrhagic shocks can cause anemia and malnutrition, and worsen the general condition of elderly patients. Malnutrition among elderly patients leads to poor outcomes such as functional decline, frailty, the decline in quality of life and higher mortality. Nevertheless, DL located in the jejunum is beyond conventional gastrointestinal endoscopy. Angiography is useful for defining the lesion. Therefore, early detection of the bleeding source by repeated endoscopy, CT and angiography is necessary, and appropriate treatment is more important in elderly than young patients. Aging may be a risk factor for DL. The findings of DL in elderly patients suggest that atherosclerosis is associated with DL. DL also may be caused by an aneurysm in one of the vessels, perhaps in combination with atherosclerosis. Meanwhile, congenital or acquired vascular malformation might be an underlying cause in young patients. Although surgery had been performed in patients presenting with DL in the jejunum, endoscopic therapy is effective and safe in most cases. Endoscopic hemostasis is less invasive than surgery, especially for elderly patients. Although balloon-assisted enteroscopy recently has become practicable, this method was unavailable in our case. Transoral endoscopy accomplished by colonoscopy could be an alternative in emergency cases. This case highlights the unusual gastrointestinal hemorrhage that can induce repeated hemorrhagic shocks and malnutrition. The possibility of underlying causes in the small intestine should be kept in mind in patients with unusual gastrointestinal hemorrhage. Careful evaluation of the clinical and laboratory findings, and early treatment is important, especially in elderly patients. Geriatr Gerontol Int 2010; 10: 267–268

Late-onset CMT2 associated with a novel missense mutation in the cytoplasmic domain of the MPZ gene

Phenotypic variations have been reported in Charcot-Marie-Tooth disease type 2 (CMT2) including age-at-onset, disease progression and severity. Sporadic cases with CMT2 have also been demonstrated by genetic test. We here report a patient with late-onset CMT2 without family history, who developed gait disturbance at the age of 68. Sequence analysis revealed a novel heterozygous Arg198Gly mutation in the cytoplasmic domain of the major peripheral myelin protein zero (MPZ). The mutation is located in the protein kinase C (PKC) alpha substrate motif (RSTK) of MPZ, presumably leading to the loss of PKC-mediated phosphorylation in adhesion. Routine genetic test for CMT is not recommended for every patient with late-onset peripheral neuropathy without known causes, however, the genetic test may be taken into consideration if the patient shows a clinical phenotype similar to that of CMT, and the possibility of a de novo mutation cannot be excluded.

Elderly case of prolonged hypoglycemic coma presenting with reversible magnetic resonance imaging changes

The aging population is leading to an increase in the prevalence of diabetes mellitus (DM). Safer treatments for DM are required in elderly patients, because this age group suffers from a higher prevalence of geriatric syndrome and increased risk of functional dependency, frailty and hypoglycemia compared with younger patients. In addition to DM, recurrent severe hypoglycemia is associated with cognitive impairments, and prolonged hypoglycemia can induce irreversible coma. Thus, preventing hypoglycemia is very important, particularly for elderly patients with DM. A 75-year-old woman developed nausea and anorexia. She had DM, dyslipidemia, atrial fibrillation, lumbar spinal canal stenosis and a history of cerebral infarction in a subcortical parietal lobe. Although she was functionally independent, she had slight cognitive and gait balance impairments. A history of stroke and peripheral diabetic neuropathy was speculated to be the causes of these impairments. She had been treated well for the DM with glimepiride and metformin hydrochloride, but had not yet started insulin therapy. Her family physician diagnosed acute enterogastritis with a cold and prescribed an antiphlogistic therapy. She had eaten very little for several days and had twice suffered disturbances in her consciousness within 3 days of the onset of the cold. Each attack revealed hypoglycemia, which was urgently restored by a glucose injection at her doctor’s clinic. Although the doctor recommended hospitalization, she refused and went home. Against the doctor’s recommendations, she continued to take oral blood glucose-lowering agents and went to bed. The next afternoon, she was found in a sustained coma lasting at least 18 h, and was admitted to Hyogo Brain and Heart Center at Himeji, Himeji, Japan (day 1). On admission, her state of consciousness was Glasgow Coma Scale (GCS) 3; E1V1M1. Her blood pressure was 141/84 mmHg, her heart rate was 106 b.p.m. and her body temperature was 38.8°C. Neurological examination revealed ocular conjugate deviation to the right side. Her muscle tone was flaccid, deep tendon reflexes were absent and bilateral plantar responses were extensor. No meningeal signs were noted. Laboratory examination on admission showed a white blood cell count of 12 000 ¥ 10 /ml (polynuclear cells dominant) and a C-reactive protein level of 0.7 mg/dL. Her blood glucose level was 21 mg/ dL, which was urgently restored to 284 mg/dL by a glucose injection. This procedure did not improve her state of consciousness. Her HbA1c level was 5.9%. A urinalysis showed proteinuria and glucosuria, but was negative for bacteriuria. Cerebrospinal fluid (CSF) examination revealed pleocytosis, with a cell count of 180 cells/mL (164 polynuclear cells/mL), a protein level of 33 mg/dL and a glucose level of 177 mg/dL (day 2). This CSF analysis also proved negative for inflammatory markers (myelin basic protein and oligoclonal bands) and bacterial culture on day 5. An electroencephalogram (EEG) showed diffuse theta background activity without epileptoid discharges (day 2). We made a diagnosis of sustained hypoglycemia that resulted in hypoglycemic encephalopathy (HE) with the possibility of complicated acute encephalitis, most likely parainfectious or postinfectious acute disseminated encephalomyelitis (ADEM). She was treated with antibiotics, glycerin and methylprednisolone pulse therapy (mPSL-pulse). Her consciousness level improved a few days later (GCS 6; E4V1M1), but she remained akinetic and mute. A repeat CSF analysis on day 10 gave normal results. After 6 months, she was still in an akinetic state. Geriatr Gerontol Int 2010; 10: 331–333

Isolated trochlear nerve palsy due to a contusion at the trochlear nerve exit zone.

A 52-year-old man struck his occiput. A few hours later, he became aware of vertical diplopia. Brain CT disclosed no intracranial hemorrhage; however, MRI demonstrated a lesion at the right side of the surface of the midbrain tegmentum ( fig. 1 a–c). Isolated right trochlear nerve palsy was revealed by Hess screen test ( fig. 1 d). His symptom disappeared during a 1-month follow-up. Trochlear nerve palsy following head trauma is not rare, but detection of contusional lesion by neuroimaging has been possible in a few cases. It is speculated that traumatic force caused the trochlear nerve exit zone to impact on the tentorium in this patient.