Onset of age, site and respiratory symptoms are strongly associated with respiratory decline in sporadic amyotrophic lateral sclerosis: a long-term longitudinal study.

Objective: The objective of this study is to identify factors influencing progression of respiratory decline from the onset of neurological symptoms to respiratory failure in patients with amyotrophic lateral sclerosis (ALS).

Methods: In 100 patients with sporadic ALS, %vital capacity (%VC) was continuously measured from the first visit to the respiratory endpoint (REP). Cox proportional hazards model identified factors influencing the duration from onset of ALS to REP (Onset-REP).

[Thrombolytic recombinant tissue plasminogen activator (rt-PA) treatment in the acute ischemic stroke with limb arterial embolism; three case reports].

We report three female patients, aged 77, 89, and 92 years, with arterial emboli in their limbs that developed before and after recombinant tissue plasminogen activator (rt-PA) treatment for cerebral infarction. Arterial embolism in one limb developed in two patients before rt-PA treatment and in one during rt-PA treatment at the time of the first medical examination. Thrombectomy was performed in two patients.

Hereditary Angioedema Type 1 with Recurrent Dizziness.

A 41-year-old woman presented with recurrent dizziness. After an attack of dizziness, she felt edematous sensations in her hands. However, according to photographs taken during the attack, the edema on the back of the patient's hands and fingers appeared mild.

[Parkinson's disease associated with SAPHO syndrome: a case report].

A 77-year-old woman with Parkinson's disease presented with left chest pain. Physical examination revealed tenderness at her second left sternocostal joint. There was no skin rash.

[Effectiveness of levodopa treatment for diabetic chorea with reduced striatal accumulation in dopamine transporter SPECT: a case report].

We report the case of a 77-year-old woman with diabetic chorea, which presented as hemiballism of the right limbs. Initial blood examination revealed that sugar and hemoglobin A1c levels were 732 mg/dl and 12.2%, respectively.

Multiple cerebellar infarcts: clinical and pathophysiologic features.

We investigated the clinical features of 15 patients with multiple isolated ischemic lesions in the cerebellum. The main initial symptoms were vertigo and nausea/vomiting in 8 patients each, and headache in 6 patients. Dysarthria and cerebellar ataxia of the limbs and trunk were observed in 14 patients.

Rapidly progressive fatal respiratory failure (Ondine's curse) in the lateral medullary syndrome.

A 70-year-old man presented with unilateral lateral medullary infarction, and then died of rapidly progressive respiratory failure within a day. The clinical manifestations were hiccups, hoarseness, dysarthria, nystagmus, left central facial paralysis, paralysis of the left soft palate, dysphagia, decreased superficial sensation over the right face and upper limb, and cerebellar ataxia in the left upper and lower limbs. The arterial blood gas analysis revealed mild hypoventilation.

[Clinical characteristics of elderly Japanese patients with amyotrophic lateral sclerosis; with special reference to the development of respiratory failure].

Objective: To clarify the characteristics of elderly-onset amyotrophic lateral sclerosis (ALS).

Subjects And Methods: We analyzed the pattern of progression of clinical symptoms and respiratory dysfunction in 26 sporadic ALS patients (19 men, 7 women; mean age 73.2 +/- 6.

Respiratory failure in a patient with antecedent poliomyelitis: amyotrophic lateral sclerosis or post-polio syndrome?

We report a 69-year-old man who developed paralytic poliomyelitis in childhood and then decades later suffered from fatal respiratory failure. Six months before this event, he had progressive weight loss and shortness of breath. He had severe muscular atrophy of the entire right leg as a sequela of the paralytic poliomyelitis.

Gene expression profile of spinal motor neurons in sporadic amyotrophic lateral sclerosis.

The causative pathomechanism of sporadic amyotrophic lateral sclerosis (ALS) is not clearly understood. Using microarray technology combined with laser-captured microdissection, gene expression profiles of degenerating spinal motor neurons isolated from autopsied patients with sporadic ALS were examined. Gene expression was quantitatively assessed by real-time reverse transcription polymerase chain reaction and in situ hybridization.

Polyamine concentrations in the brain of vitamin B12-deficient rats.

To study the pathophysiology of the neuronal degeneration in vitamin B12 deficiency, we investigated the concentrations of the polyamines putrescine, spermidine, and spermine in brain regions and liver using high-performance liquid chromatography with fluorescence detection. Male Wistar rats were fed either a control or vitamin B12-deficient diet for 20 weeks. No remarkable behavioral changes were observed.

Progression and prognosis in multiple system atrophy: an analysis of 230 Japanese patients.

We investigated the disease progression and survival in 230 Japanese patients with multiple system atrophy (MSA; 131 men, 99 women; 208 probable MSA, 22 definite; mean age at onset, 55.4 years). Cerebellar dysfunction (multiple system atrophy-cerebellar; MSA-C) predominated in 155 patients, and parkinsonism (multiple system atrophy-parkinsonian; MSA-P) in 75.