The authors encountered 2 siblings who showed progressive ophthalmoplegia and bulbar palsy. The patients were studied with various laboratory aids and thought to be as oculopharyngeal muscular dystrophy. The oculopharyngeal muscular dystrophy is a rare form of muscular dystrophy. The authors present the cases as familial oculopharyngeal muscular dystrophy.
Bulbar Palsy, Progressive ; Humans ; Muscular Dystrophies ; Muscular Dystrophy, Oculopharyngeal* ; Ophthalmoplegia ; Siblings

A l9-year-old boy was admitted because of progressive spastic quadriparesis and bulbar palsy which developed from 3 years ago. The results of all performed laboratory tests were normal, but neuroradiologic tests revealed intracerebral calcification and degeneration of oilateral pyramidal tracts. Idiopathic familial intracerebral calcification was rarely occurred and presented usually with various extrapyramidal and cortical symptoms. Mild pyramidal symptoms were also associated in some reported cases, but none of them showed predominant spasticity as well as suspicious pyramidal tract degeneration on magnetic resonance imaging.
Bulbar Palsy, Progressive ; Humans ; Magnetic Resonance Imaging ; Male ; Muscle Spasticity ; Pyramidal Tracts* ; Quadriplegia

Bulbar Palsy, Progressive ; etiology ; Child, Preschool ; Hand, Foot and Mouth Disease ; complications ; Humans ; Male ; Pulmonary Edema ; etiology

BACKGROUND: Recently, anti-ganglioside complex (GSC) antibodies were discovered among the various subtypes of Guillain-Barré syndrome. GSC is the novel glycoepitopes formed by two individual ganglioside molecules. CASE REPORT: We present a 36-year-old man with overlap Miller Fisher syndrome and acute bulbar palsy who had anti-GSC antibody that provided diagnostic robustness. CONCLUSION: Anti-GSC testing could be considered important in patients who show atypical manifestation with negative antibody reaction against each constituent ganglioside.
Adult ; Antibodies ; Bulbar Palsy, Progressive ; Gangliosides ; Guillain-Barre Syndrome ; Humans ; Miller Fisher Syndrome

Distortion of vowels in dysarthria associated with amyotrophic lateral sclerosis can be detected at the perceptual, physiological, and acoustical levels of analysis. Sound spectrography was used to analyse the formants of vowels which reflect position and space of articulatory organs. We analyse status and progression of dysarthria in 54 year old women with amyotrophic lateral sclerosis using sound spectrography. Target formant frequencies were measured from select words containing the vowels /a/, /e/, /i/, /o/, /u/. Results revealed that dysarthric patient exhibited smaller vowel space areas and less systematic changes in vowel spaces for pronouncing different vowels in comparison with normal control. Changes of vowel formants in sound spectrographic analysis reflected progression of dysarthric symptom in this patient. We conclude that acoustic analysis of vowels using sound spectrography is a useful tool to visualize and quantitatively analyse the severity and progression of dysarthria due to paralytic articulatory organ.
Acoustics ; Amyotrophic Lateral Sclerosis ; Bulbar Palsy, Progressive* ; Dysarthria ; Female ; Humans ; Middle Aged ; Sound Spectrography*

This paper introduces GAO Wei-bin's academic thought in treatment of medulla oblongata paralysis with acupuncture. Through analyzing the etiologies and locations of medulla oblongata paralysis, in accordance with "selecting the nearby acupoints of the affected area", the acupoints are selected from the nape region, the nape acupuncture therapy and the corresponding new points are developed. Based on the human anatomy of the nape region, the anatomic structures of new points (e.g. Gongxue, Tunyan-1, Tunyan-2, Fayin, Zhiqiang and Tiyan) and their effect mechanism are explained. The treatment principle, "distinguishing the symptoms from the root causes, mutual treatment for both symptoms and root causes", is proposed, and the importance of electric stimulation of nape acupuncture is suggested in treatment of medulla oblongata paralysis.
Humans ; Acupuncture ; Acupuncture Therapy ; Acupuncture Points ; Bulbar Palsy, Progressive/therapy* ; Medulla Oblongata ; Paralysis

Blindness or serious visual impairment is a common and well known sequela of methanol intoxication in man, while other manifestations such as focal neurological deficits and abnormal brain CT scan are reported rarely. We experienced a case of acute methanol intoxication, which showed not only visual impairment but also bulbar symptoms and characteristic bilateral symmetrical hypodensity area in basal ganglia, especially putamen on brain CT scan. We tried 'pulse therapy'-continuouis dripping of methyl prednisolone (Solumedrol 1gm/day) for 5 days-with transient improvement of visual impairment, but bulbar symptoms were stationary. One month later, visual impairment was recurred, and the 'pulse therapy' was tried again but without benefit. On follow-up brain CT scan, the size of previous hypodensity area in basal ganglia was decreased.
Basal Ganglia ; Blindness ; Brain* ; Bulbar Palsy, Progressive* ; Follow-Up Studies ; Methanol* ; Prednisolone ; Putamen ; Tomography, X-Ray Computed* ; Vision Disorders

Acupuncture Therapy ; Aged ; Aged, 80 and over ; Bulbar Palsy, Progressive ; etiology ; therapy ; Female ; Humans ; Male ; Middle Aged ; Stroke ; complications

Professor GAO Weibin academically advocates, based on basic theory of TCM and theories of different schools, modern science technology should be used for the methods and principles of acupuncture and Chinese medicine for neuropathy, so as to explore and summarize the rules, characteristics and advantages of TCM for nervous system disease, especially bulbar paralysis. During the treatment of bulbar paralysis, professor GAO creatively proposes the effective acupuncture points such as Gongxue, Tunyan-1, Tunyan-2, Fayin, Tiyan and Zhifanliu from the aspects of neuroanatomy, and analyzes their anatomical structure and action mechanism.
Acupuncture Points ; Acupuncture Therapy ; history ; Bulbar Palsy, Progressive ; history ; therapy ; China ; History, 20th Century ; History, 21st Century ; Humans

OBJECTIVETo compare the efficacy between elongated needle therapy and regular needle therapy at Tiantu (CV 22) on the basis of xingnao kaiqiao (activiting brain and regaining consciousness) acupuncture therapy so as to explore the effective therapeutic method in treatment of dysphagia induced by bulbar palsy.

METHODSSeventy one cases of dysphagia induced by bulbar palsy were randomized into two groups. The xingnao kaiqiao acupuncture therapy was applied at Shuigou (GV 26), Neiguan (PC 6), Sanyinjiao (SP 6) and the others in the two groups. In the elongated needle therapy group, on the basis of xingnao kaiqiao acupuncture therapy, the elongated needle was used to puncture Tiantu (CV 22). In the regular needle therapy, the regular acupuncture technique was used at Tiantu (CV 22). In both groups, the treatment was given once a day in a week except Sunday and lasted for 4 weeks totally. Before and after treatment, the swallowing condition and the standardized swallowing assessment (SSA) were observed in the patients and the efficacy was compared between the two groups.

RESULTSThe total effective rate was 97.2% (35/36) in the elongated needle therapy group, which was better than 77.1% (27/35) in the regular needle therapy group (P<0.05). After treatment, SSA score was reduced significantly as compared with that before treatment in the two groups (both P<0.05). SSA score in the elongated needle therapy group was reduced much more apparently as compared with that in the regular needle therapy group after treatment (P<0.05).

CONCLUSIONOn the basis of the xingnao kaiqiao acupuncture therapy, the elongated needle therapy at Tiantu (CV 22) achieves the superior effect on bulbar palsy-induced dysphagia as compared with the regular acu- puncture at Tiantu (CV 22).

Acupuncture Points ; Acupuncture Therapy ; Aged ; Bulbar Palsy, Progressive ; complications ; Deglutition Disorders ; etiology ; therapy ; Female ; Humans ; Male ; Middle Aged ; Treatment Outcome