Intramedullary hemorrhage caused by arteriovenous malformation: a case of mixed lateral and medial medullary syndrome.

A 48-year-old man with no known risk factor for cerebrovascular disease, other than cigarette smoking, experienced the sudden onset of a mixed lateral and medial medullary syndrome. Computed tomography scan failed to show any definite abnormality. Magnetic resonance imaging scans revealed hemorrhage restricted to the left dorsolateral medulla. Angiography showed abnormal arteries originating from the left vertebral artery with small niduses located on the surface of the medulla and contralateral cerebellum. Small brain-stem hemorrhages are a contraindication to thrombolytic or anticoagulant therapy, and therefore must be recognized in the acute stage.

Genetic analysis of a dentatorubral-pallidoluysian atrophy family: relevance to apparent sporadic cases.

Dentatorubral-pallidoluysian atrophy (DRPLA) is associated with an unstable CAG trinucleotide sequence. We describe a DRPLA family whose members have an allele containing an expanded CAG repeat, even in an elderly neurologically normal individual. The proband developed DRPLA at age 14. She was initially considered a sporadic case, but later her sister became symptomatic. Investigation of the number of CAG repeat units in her family revealed the 81-year-old father to have an expanded CAG repeat of 51 units. To our knowledge, such an advanced aged unaffected patient has not been previously documented. The present example may explain apparent sporadic cases.

[A case of Miller Fisher syndrome with gadolinium-enhancing lesions in the cranial nerves and the cauda equina on magnetic resonance imaging].

MRI findings for a patient with Miller Fisher syndrome (MFS) are reported. A 25-year-old woman presented with blephaloptosis, double vision, and gait disturbance after antecedent upper respiratory infection. Neurologic examination on admission revealed bilateral blephaloptosis and restriction of extraocular movement. She was unable to maintain an upright posture, nor able to walk because of severe truncal ataxia. Mild limb ataxia was also seen. The tendon reflexes were generally absent and she had hypesthesia in her shoulder and upper limbs. On the next day, her eyes became fixed in the central position. Based on these characteristic symptoms, she was diagnosed as having MFS and underwent plasmapheresis therapy six times. Although ataxia gradually reduced, ophthalmoplegia did not improve and severe symmetric facial nerve palsy appeared. Antiganglioside IgG antibodies (anti-GQ1b and GD1b) were detected in the sera on admission. Spinal fluid examination on day 18 revealed 5 cells/microliter and protein 171 mg/dl. T1-weighted images with Gd-DTPA on day 15 demonstrated enhancement of the posterior nerve roots of the cauda equina. MRI on day 32 revealed swelling and enhancement of the bilateral oculomotor nerves, as well as the facial nerves and the abducens nerves. Marked improvement of ophthalmoplegia followed high dose intravenous immunoglobulin therapy. Gd-enhanced MRI after recovery showed no enhancement of the cauda equina, nor the cranial nerves. These findings well correlated with her clinical features. Although abnormal Gd-enhancement of the cranial nerves or cauda equina in several neurological disorders has been documented, similar findings have been rarely reported in MFS. In our patient, IgG anti-GQ1b and GD1b antibodies were detected and the distribution of Gd-enhanced lesions was compatible with the distribution of the involved gangliosides. We suggest that contrast enhanced MRI is useful for identifying affected cranial nerves and cauda equina nerve roots. In addition, MRI may play a significant role in disclosing the pathophysiology of this disease.

Effects of bifemelane hydrochloride on cerebral circulation and metabolism in patients with aphasia.

The effects of bifemelane hydrochloride on aphasia and on cerebral circulation and metabolism were studied using positron emission tomography (PET) in 10 aphasic patients with cerebrovascular disease. After the first PET scan, bifemelane was administered orally three times in a daily dose of 150 mg. Aphasic features, such as fluency, auditory comprehension, object naming, and repetition, were evaluated by using the Western Aphasia Battery (Japanese edition) before and at least 2 months after bifemelane treatment. Simultaneous with the evaluation of aphasia, the PET scans were performed by using the steady-state method with 15O gases inhalation. All aphasic features, except fluency, improved significantly after bifemelane treatment. Regional cerebral blood flow (rCBF) and the cerebral metabolic rate for oxygen (CMRO2) were increased at every brain region of interest, and the oxygen extraction fraction was decreased. Both rCBF and CMRO2 were significantly increased on the left inferior frontal gyrus, superior temporal gyrus, white matter of the parietal lobe, and insula. These results suggest that bifemelane improves both cerebral oxygen metabolism and cerebral circulation and contributes to the improvement of aphasia caused by cerebrovascular disease.

[A comparative study between amyotrophic lateral sclerosis patients with and without mechanical ventilation].

Fifty-three patients with amyotrophic lateral sclerosis (ALS) were examined as to duration of illness, cause of death and need for respirator assistance. In 47 patients with ALS, respiratory failure is the most common cause of death accounting for 62% of all fatalities. Eleven patients with mechanical ventilation died of pneumonia and respiratory failure in four cases, atelectasis, gastrointestinal bleeding and asphyxia in one. Thirteen of 53 patients with ALS required mechanical ventilation. Six cases were put on a volume-limited respiratory or a pressure-limited respirator with O2 blender, and seven cases were put on a pressure-limited respirator only, which was unable to control oxygen concentration. As to the duration of their illness, patients with mechanical ventilation survived significantly longer than those without mechanical ventilation, so patients using a respirator could expect to live significantly longer than those without one. Patients using a volume limited respirator or a pressure-limited respirator with O2 blender had significantly longer duration of mechanical ventilation than those using a pressure-limited respirator only. It is concluded that a volume-limited respirator or a pressure-limited respirator with O2 blender is useful, if mechanical ventilation is needed, for ALS patients presenting with respiratory failure.