Autosomal Recessive Lower Motor Neuron Disease with Childhood Onset
Disease progression was rapid, and the majority of patients died from respiratory failure within 1–5 years after onset of disease.[link.springer.com]
[…] tendon reflexes, and Babinski signs.[neuropathology-web.org]
Hereditary pure lower motor neuron disease with adult onset and rapid progression. J Neurol 2001 ; 248 : 290 –96.[academic.oup.com]
Infantile-Onset Ascending Hereditary Spastic Paralysis
Like previously described cases, the patient presented with severe spastic paraparesis and showed rapid progression of paresis to the upper limbs.[ncbi.nlm.nih.gov]
[…] tendon reflexes, and Babinski signs.[neuropathology-web.org]
Results: Like previously described cases, the patient presented with severe spastic paraparesis and showed rapid progression of paresis to the upper limbs.[eprints.uniss.it]
Progressive Muscular Atrophy
Rapid progression and typical ubiquitinated inclusions in lower motor neurons were present in 77 (95%) of the cases.[ncbi.nlm.nih.gov]
[…] tendon reflexes, and Babinski signs.[neuropathology-web.org]
Kovacs, Sandor Czirjak, Peter Pusztai, Ibolya Varga and Karoly Racz, Rapid Progression of Amyotrophic Lateral Sclerosis in an Acromegalic Patient After Surgical Resection[doi.org]
Progressive Bulbar Palsy
A 12-month-old boy with progressive cranial nerve palsies followed by ventilatory failure demanding artificial ventilation, generalized muscle weakness, and rapid progression[ncbi.nlm.nih.gov]
Pseudobulbar palsy is a clinical syndrome of dysarthria, dysphagia, a hyperactive gag reflex and labile emotional responses.[radiopaedia.org]
Usually the onset is gradual but younger patients may show a more rapid progression.[southerncross.co.nz]
Hereditary Hyperekplexia
Dystonia 12 Rapid-onset dystonia-parkinsonism (RDP) is a very rare movement disorder, characterized by the abrupt onset of parkinsonism and dystonia, often triggered by physical[amp.pharm.mssm.edu]
Patients may also have umbilical and inguinal hernias and hyperactive brainstem reflexes such as head retraction, palmomental and snout reflexes.[cags.org.ae]
[…] disease (NHD), also referred to as polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL), is a rare inherited leukodystrophy characterized by progressive[amp.pharm.mssm.edu]
Motor Neuron Disease
Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed.[ncbi.nlm.nih.gov]
[…] tendon reflexes, and Babinski signs.[neuropathology-web.org]
Usually the onset is gradual but younger patients may show a more rapid progression.[southerncross.co.nz]
Pseudobulbar Palsy
Romanian O paralizie simetrică poate evolua rapid.[mymemory.translated.net]
brainstem reflexes (eg, jaw jerk), the dissociation of automatic and volitional movements of the bulbar muscles with preservation of automatic movements, and the absence[medlink.com]
It occurs pretty late and the beginning is slow, but it is highly progressive and people usually die within few years after the onset.[wikilectures.eu]
Spinal and Bulbar Muscular Atrophy
progression leading to compromised ambulation in the mid-20s.[ncbi.nlm.nih.gov]
[…] tendon reflexes, and Babinski signs.[neuropathology-web.org]
As with other trinucleotide repeat disorders, anticipation is frequently observed and larger CAG expansions are associated with earlier onset and a more rapid clinical progression[mayomedicallaboratories.com]
Upper Motor Neuron Disease
[…] tendon reflexes, and Babinski signs.[neuropathology-web.org]
Due to the debilitating nature and its rapid progression, MND is a significant issue affecting society with ethical and legal debates centered around end of life decisions[en.wikibooks.org]
Bulbar-onset MND is not uniformly associated with a rapid progression, and symptoms may remain isolated to this territory with apparently normal EMG studies.[pn.bmj.com]
Juvenile Primary Lateral Sclerosis
Bulbar-onset MND is not uniformly associated with a rapid progression, and symptoms may remain isolated to this territory with apparently normal EMG studies.[pn.bmj.com]
[…] tendon reflexes, and Babinski signs.[neuropathology-web.org]
Lower motor neurone-predominant monomelic clinical sub-types of MND present the greatest diagnostic challenge but are frequently more slowly progressive.[pn.bmj.com]