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At least 19 recordsLinked to original sources
PubMed1992

Progressive bulbar paralysis of childhood. A reappraisal of Fazio-Londe disease.

Fazio-Londe disease is a label sometimes applied to a degenerative disease of the motor neurons characterized by progressive bulbar paralysis in children. It is very rare with only 22 case reports describing 24 children including four sibling pairs. In two reports mothers and sons were affected. The neuropathology is described in only four cases. Previous authors have recognized that the condition is very heterogeneous. The clinical features of five children with this type of progressive bulbar paralysis, diagnosed at this hospital between 1969 and 1989, are reviewed, and in two cases neuropathological findings are detailed. Based on this experience, suggested criteria for diagnosis include clinical features of a pure motor neuronopathy affecting the bulbar nuclei, exclusion of other causes of progressive bulbar paralysis and positive support for the diagnosis from electromyography and/or pathological examination. A review of the literature, combined with the present series, suggests that there are at least three distinct subtypes: a very rare autosomal dominant form (as described by Fazio) and two variants with probable autosomal recessive inheritance either with early onset of respiratory symptoms and rapid progression to death or later onset, less prominent respiratory symptoms and protracted clinical course. There is strong concordance for each clinical pattern within families.

Bulbar Palsy, Progressive↗
PubMed1981

[Progressive bulbar paralysis. Report of a juvenile case (author's transl)].

The authors report the case of a 16 year-old girl with the following features: clinically, a progressive bulbar paralysis, a weakness and wasting of muscles predominantly in the upper limbs; pathologically, a severe neuronal loss in the motor nuclei of the VIIth, IXth, XIIth cranial nerves and in the anterior horns of the cervical and thoracic spinal cord, a demyelinisation of the corticospinal tracts. A classification of progressive bulbar paralysies in infants and children is proposed. In the first group, the peripheral motoneuron is the only involved. Such cases are often called Fazio-Londe disease and can be related to those cases of infantile spinal amyotrophy either of the Werdnig-Hoffmann type or, most often, of the Wolfhardt-Kugelberg type. In the second group, the corticospinal tract is also involved. Some of these cases can be included in the spinocerebellar degenerations but others, such as the case reported here, are strongly reminiscent of the adult amyotrophic lateral sclerosis.

Adolescent↗
PubMed1981

[Progressive bulbar paralysis in childhood with pyramidal signs (author's transl)].

We report on a 8 year-old boy who presented with progressive bulbar paralysis. Remarkable were the presence of pyramidal signs, the visual disturbances, the peculiar gait and the intermittent progression of the disease. Our case supports the idea that spinal muscular atrophies form a group of diseases with variable expression. We can classify our patient between the juvenile form and the adult form of spinal muscular atrophy with progressive bulbar palsy. We could follow this boy for almost 4 years. He died at the age of twelve years. Post mortem examination was not permitted.

Bulbar Palsy, Progressive↗
PubMed1997

Light chain myeloma with oro-pharyngeal amyloidosis presenting as bulbar paralysis.

A 53-year old woman developed slowly progressive dysarthria, mild enlargement of the tongue and dysphagia since 1 year ago. All neurological differential diagnoses that could have explained the bulbar symptoms were excluded. The swallowing sequence was pathologic and immunoglobulins were markedly reduced. A bone marrow biopsy revealed light chain myeloma grade III. Amyloid deposits were found in the tongue but not in the kidneys. Oro-pharyngeal amyloidosis was held responsible for the described complaints. It is concluded that multiple myeloma must be considered in the differential diagnosis of bulbar paralysis and that biopsy of specific lesions is necessary to confirm local amyloidosis.

Amyloidosis↗
PubMed1980

Progressive bulbar paralysis associated with neural deafness. A nosological entity.

A complete autopsy verification of progressive bulbar palsy associated with neural deafness was performed. Hearing loss and speech difficulties developed in a five-year-old girl. When she was 24 years old, clinical examination demonstrated deafness and bulbopontine paralysis together with retinitis pigmentosa, peripheral amyotrophies, pyramidal signs, and ataxia. The patients died at 27 years and the autopsy disclosed degenerative changes characterized by simple atrophy and loss of neurons accompanied by gliosis and loss of myelinated fibers. The structures principally affected were the anterior horns and the motor nuclei of the brain stem together with the eighth cranial nerve nuclei. Loss of myelinated fibers was found in the spinocerebellar and pyramidal tracts and in the fasciculus gracilis. Our study suggests that progressive bulbar paralysis with neural deafness should be considered as a nosological entity.

Adolescent↗
PubMed2002

[Bulbar paralysis and flaccid tetraparesis in thyrotoxicosis].

We describe the case of an aged patient, presenting an acute onset of flaccid paralysis of the limbs and bulbar weakness, complicated by ab ingestis pneumonia. These neurological findings were the first signs of a thyrotoxic crisis and showed a progressive regression together with the healing of the hyperthyroidism obtained, within two months, utilizing thyroid inhibitors. At the onset, a marked hypokalaemia was present, making possible the hypothesis of a thyrotoxic periodic paralysis. The hypokalaemia was corrected after many infusions of KCl i.v., without obtaining significant modifications of the neurological picture. Taking into consideration the evolution and the results of the instrumental procedures (EMG, MRI) we believe this a rare case of thyrotoxic acute myopathy, with bulbar involvement, casually associated to hypokalaemia.

Aged↗
PubMed1983

Progressive bulbar paralysis in childhood: a case report.

The case of a progressive bulbar paresis in a nine and a half year old child is reported. The first symptoms were present at birth; however, the subsequent evolution was very low. Lesion of the motor nuclei of the V, VII, IX, XII, cranial nerves was evident on electromyographic investigation. Damage to the acoustic brain stem pathway was documented by the brain stem evoked potentials although audiometry was normal. No other neuronal systems or districts appeared to be damaged. The case suggests Fazio-Londe disease, although the involvement (albeit partial) of the auditory pathways recalls Van Laere syndrome. This supports the view that motor neuron disease in infancy is not an autonomous entity but a variant in a wide spectrum of progressive neuronal diseases.

Audiometry↗