Self-rated anosognosia score may be a sensitive and predictive indicator for progressive brain atrophy in amyotrophic lateral sclerosis: an X-ray computed tomographic study.

We investigated whether a self-rated anosognosia score can be an indicator for progression of brain atrophy in patients with amyotrophic lateral sclerosis (ALS). Scores for 16 patients were compared with the ventricular areas of the bilateral anterior and inferior horns measured on x-ray computed tomography. Longitudinal enlargement was expressed as a monthly increase in size: (ventricular size at the initial scan - ventricular size at the follow-up scan)/scan interval (months). The anosognosia scores ranged from -4 to 3 and 3-18 in patients with and without frontotemporal lobar degeneration (FTLD), respectively (p = 0.0011). Anosognosia scores were significantly correlated with sizes of anterior (r = 0.704, p = 0.0016) and inferior (r = 0.898, p < 0.0001) horns. In non-demented patients for whom follow-up CT scans were available (n = 7), the scores were significantly correlated with the longitudinal increase in inferior horn size (r = 0.754, p = 0.0496), but not with that of anterior horn size (r = -0.166, p = 0.7111). In conclusion, anosognosia in ALS is associated with greater anterior and inferior horn sizes, reflecting frontotemporal lobar atrophy. Moreover, mild anosognosia in ALS patients without FTLD may predict impending inferior horn enlargement, reflecting medial temporal atrophy.

Writing error may be a predictive sign for impending brain atrophy progression in amyotrophic lateral sclerosis: a preliminary study using X-ray computed tomography.

AIM: To investigate whether writing errors are predictive of longitudinal brain atrophy progression in patients with amyotrophic lateral sclerosis (ALS). METHODS: The frequency of writing errors in 6 ALS patients without dementia was compared with longitudinal changes in lateral ventricular areas of the bilateral anterior and inferior horns on X-ray computed tomography scans. The increase in area per month for the anterior and inferior horns was used as a measure of longitudinal brain atrophy progression, and was calculated as: (area on the initial scan - area on the follow-up scan)/scan interval (month). RESULTS: The longitudinal rate of increase in the area of the anterior horns showed significant associations with the rates of total writing errors (r = 0.886, p = 0.0152), kana errors (r = 0.887, p = 0.0148) and kana omission (r = 0.856, p = 0.0268), whereas that for the inferior horns size showed no significant association with any writing errors. CONCLUSION: The increased area of the anterior horns indicates frontal-lobar atrophy, and writing errors may be a predictive sign for impending brain atrophy progression in the frontal lobes, which reflects the development of anterior-type dementia.

[Tuberculous cranial pachymeningitis presenting with long-standing diffuse brain dysfunction].

We report a 59-year-old immunocompetent man presenting with slowly progressive gait unsteadiness, dysarthria, and clumsiness in writing over 6 months. There were bilateral pyramidal signs, pseudobulbar palsy, and attention deficits. Cerebrospinal fluid examination showed mild mononuclear pleocytosis, and magnetic resonance imaging revealed pachymeningeal pattern of contrast enhancement beneath the calvarium and the posterior cranial fossa. Interferon-gamma release assay in whole blood after stimulation by specific tuberculosis antigens was positive and repeat polymerase chain reaction assay detected Mycobacterium tuberculosis genome in the cerebrospinal fluid. After combination therapy with anti-tuberculous agents and corticosteroids, the patient's pachymeningitis regressed. Tuberculous cranial pachymeningitis may present with chronic diffuse brain dysfunction without headache, fever, or cranial nerve dysfunction.

Kana versus kanji in amyotrophic lateral sclerosis: a clinicoradiological study of writing errors.

The frequency of writing errors in samples from 14 patients with amyotrophic lateral sclerosis without manifest aphasia were compared with clinical background and indices from X-ray computed tomography, including the Evans' index (EI) and the cella media index (CMI). The inferior horn index (IHI) was measured as the maximal width of the short axis of the bilateral inferior horn of the lateral ventricles/the maximum transverse distance between the two internal laminae. Overt dementia and disinhibitive behavioral changes were significantly associated with frequency of total errors (p = 0.0280) and kanji errors (p = 0.0025). Significant associations were found for the EI with kana errors (p = 0.0481) and for the IHI with kanji errors (p = 0.0052). Preferential involvement of kana and kanji may reflect involvement of language-related areas in the frontotemporal lobes with frontal lobe or temporal lobe predominance.

Recurrent abducens nerve palsy associated with neurovascular compression.

We report a 50-year-old man who suffered from 5 transient diplopia episodes in 16 months. His diplopia lasted between 2 weeks and 3 months and examination revealed isolated left abducens palsy during the attacks of diplopia. Magnetic resonance (MR) angiography and MR imaging with constructive interference in the steady state sequence showed neurovascular compression of the left abducens nerve at the point of exit from the brain stem. Together with a lack of the preceding headache or febrile illness, we propose that neurovascular compression is a possible etiology of recurrent, isolated abducens nerve palsy.

[Is hyperdynamic therapy possible for elderly patients with vasospasm following subarachnoid hemorrhage?].

BACKGROUND: Recently we have an increased number of elderly patients with subarachnoid hemorrhage (SAH) to be treated. Elderly patients are expected to have a worse cardiac function than that of younger patients. A question arises whether management for elderly patients in the vasospasm period can be performed as safely as it is for younger patients. The aim of this study is to examine the cardiac function of the elderly patients (> or =75 y.o.) with SAH correlated with various complications in the vasospasm period. MATERIALS: We retrospectively analyzed consecutive 356 patients with SAH encountered in our institute since 2000 to 2006. Seventy-three patients (20.5% of all) are 75 or more than 75-year-old. Their mean age is 80.4 +/- 4.43 (16 male, 57 female). Cardiac function was examined by trans-thoracic echocardiography (CTE) in 40 patients (54.8%). RESULT: Average value of their ejection fraction (EF) and rates of perioperative complications were not so different from those of the younger patients. But among patients of > or =75 y.o., certain patients in whom EF was under 0.6 significantly have experienced cardiopulmonary complications and longer hospitalization. In a multiple logistic analyses, only EF is significantly related with cardiopulmonary complications (P = 0.013). CONCLUSIONS: Among elderly SAH patients > or =75 year of age, some have experienced more cardiopulmonary complications than younger patients and have needed longer hospitalization. For such patients hyperdynamic therapy must be carefully carried out. TTE is effective to predispose and help eliminate their cardiopulmonary complications in the pre- and postoperative period.