A CADASIL Case Presenting with Progressive Bulbar Palsy
Caused by Acute Simultaneous Multiple Subcortical Infarcts.
Various Types of Presentation of the Patients Presentation Number of Patients Percentage Quadriparesis 36 90% Paraplegia 2 5% Respiratory Distress 12 30% B/L Facial Palsy 3 7.5% Bulbar Palsy
4 10% Neck muscle weakness 6 15% Sensory disturbance 13 32.5% Ophthalmoplegia 3 7.5% Autonomic Dysfunction 12 30% Table 2.
Acute bulbar palsy
as a variant of Guillain Barre syndrome Neurology 2016; 86(8): 742-7.
However, isolated bulbar palsy
(IBP), an often under-understood variant of ALS, has symptoms confined to bulbar region for extended periods.[sup], IBP is characterized with insidious onset of dysarthria or dysphagia, which aggravates slowly, and relative preservation of limb and respiration function initially.
Ciara Keating, six, suffers from severe cerebral palsy and life-threatening Bulbar palsy
Katie suffers from bulbar palsy
after a difficult birth when she and twin Holly arrived prematurely.
While some people live for many years with MND, for most the life expectancy is two to five years from the onset of symptoms.There are four types of MND -- Amyotrophic Lateral Sclerosis (ALS), Progressive Muscular Atrophy (PMA), Progressive Bulbar Palsy
(PBP), and Primary Lateral Sclerosis (PLS).
* Progressive bulbar palsy
(PBP) This form affects about a quarter of people diagnosed, and involves both the upper and lower motor neurones.
A brain tumour was eliminated but the physical findings and bulbar palsy
led to a diagnosis of motor neurone disease (MND).
On the other hand, in the mid-1990s my mother was diagnosed with a fatal motor neuron disease called Bulbar Palsy
. For three long years, she battled with increasing paralysis, breathing difficulty and loss of speech until the day came when she was not able even to close her eyelids.
"I have a form called progressive bulbar palsy
and the average life expectancy is between six months and three years - thankfully I'm still going strong."
Progressive bulbar palsy
affects about a quarter of people with MND.
Patient C was seen at home by Dr Malik in 2004 because she was having choking spasms as a result of Motor Neurone Disease and another muscle-wasting disease, Progressive Bulbar Palsy