Hyperactive Brainstem Reflexes & Rapid Progression: Causes & Reasons

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Patient File

Sex unknown, Age unknown

Reported Symptoms

Hyperactive Brainstem Reflexes, Rapid Progression,

Negated

None

Unsure

None

Possible Causes

Distal Spinal Muscular Atrophy Type 4

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

Disease progression was rapid, and the majority of patients died from respiratory failure within 1–5 years after onset of disease. [link.springer.com]

Hereditary pure lower motor neuron disease with adult onset and rapid progression. J Neurol 2001 ; 248 : 290 –96. [academic.oup.com]

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Infantile-Onset Ascending Hereditary Spastic Paralysis

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

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]

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]

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Progressive Muscular Atrophy

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

Rapid progression and typical ubiquitinated inclusions in lower motor neurons were present in 77 (95%) of the cases. [ncbi.nlm.nih.gov]

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]

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Progressive Bulbar Palsy

Pseudobulbar palsy is a clinical syndrome of dysarthria, dysphagia, a hyperactive gag reflex and labile emotional responses. [radiopaedia.org]

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]

Usually the onset is gradual but younger patients may show a more rapid progression. [southerncross.co.nz]

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Hereditary Hyperekplexia

Patients may also have umbilical and inguinal hernias and hyperactive brainstem reflexes such as head retraction, palmomental and snout reflexes. [cags.org.ae]

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]

[…] 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]

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Pseudobulbar Palsy

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]

Romanian O paralizie simetrică poate evolua rapid. [mymemory.translated.net]

Pseudobulbar palsy is a clinical syndrome of dysarthria, dysphagia, a hyperactive gag reflex and labile emotional responses. [radiopaedia.org]

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Motor Neuron Disease

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

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]

Usually the onset is gradual but younger patients may show a more rapid progression. [southerncross.co.nz]

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Myelopathy

Additional cranial nerve abnormalities and/or a hyperactive jaw jerk reflex would suggest the presence of a brainstem or intracranial lesion.2 Cognitive dysfunction can help [journals.sagepub.com]

Outcomes following laminoplasty, laminectomy and anterior surgery Dorsal decompression of any type correlates with more rapid progression of OPLL. [nature.com]

In 75% of the patients, episodic worsening with neurological deterioration occurs, 20% have slow steady progression, and 5% experience rapid onset and progression. [4] Common [physio-pedia.com]

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Spinal and Bulbar Muscular Atrophy

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

progression leading to compromised ambulation in the mid-20s. [ncbi.nlm.nih.gov]

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]

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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]

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Juvenile Primary Lateral Sclerosis

[…] tendon reflexes, and Babinski signs. [neuropathology-web.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]

In about 25% of cases, ALS begins with brainstem symptoms (dysarthria, difficulty swallowing) followed by extremity weakness. [neuropathology-web.org]

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Worster-Drought Syndrome

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]

At the beginning of the period of recovery, a rapid and total disappearance of neologisms was observed, while only literal ... more A progressive loss of comprehension and [scinapse.io]

[…] distinguished from bulbar paralysis and from disorders of the cranial nerves and neuromuscular junction (eg, botulism and myasthenia gravis) by normal eye movements, preserved or hyperactive [medlink.com]

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Amyotrophic Lateral Sclerosis Type 5

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

A novel P66S mutation in exon 3 of the SOD1 gene with early onset and rapid progression. [atm.amegroups.com]

There was rapid progression of motor weakness beginning in the lower limbs, with loss of walking ability by 6 years of age for IV-8 and by 3 years of age by IV-9. [journals.plos.org]

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Amyotrophic Lateral Sclerosis Type 4

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

However, others may experience a rapid progression of symptoms and quickly become unable to care for themselves. [healthline.com]

Homozygous deletion of SMN2 gene 5 More common in sporadic ALS Survival time: 2 years shorter Effect not replicated in other reports Disease progression more rapid Titin expression [neuromuscular.wustl.edu]

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Lymphedema Hypoparathyroidism Syndrome

Sleep disturbance Motor polyneuropathy Microcornea Head tremor Overgrowth Attention deficit hyperactivity disorder Primitive reflex Cataplexy Aggressive behavior Thin upper [mendelian.co]

Rapid treatment of thyroid storm is essential to preserve life. [quizlet.com]

Bulbar signs Bilateral sensorineural hearing impairment Thick lower lip vermilion Resting tremor Abnormality of mitochondrial metabolism Atrophy/Degeneration affecting the brainstem [mendelian.co]

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Epilepsia Partialis Continua

[…] with hyperactivity of medullary brainstem reticular formation; and (c) primary generalized epileptic myo-clonus as a fragment of primary generalized epilepsy, which may represent [neupsykey.com]

EPC may occur as a single episode, repetitive episodes, it may be chronic progressive or non-progressive. [ncbi.nlm.nih.gov]

EPC may occur as a single episode, repetitive episodes, or as a chronic progressive or non-progressive disorder. In some cases, EPC may spread or generalize. [ncbi.nlm.nih.gov]

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Distal Hereditary Motor Neuropathy Type 8

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

progression (see OMIM:105400 ). [flybase.org]

Acute - 0-3 months rapid onset and progress severe hypotonia/weakness GENERALLY DEATH IN 3 YRS WITHOUT VENT What happens with Type 2 SMA? [quizlet.com]

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Dystonia with Cerebellar Atrophy (DYTCA)

[…] deep tendon reflexes Atrophy/Degeneration affecting the brainstem Spastic paraplegia Urinary bladder sphincter dysfunction Pain Sporadic Abnormality of brainstem morphology [mendelian.co]

[…] of coordination of movements), ballism (violent involuntary rapid and irregular movements), bradykinesia (slow movement), cerebral palsy (static encephalopathy), chorea (rapid [neupsykey.com]

[…] impairment Vestibular dysfunction Myotonia Bulbar signs Ophthalmoparesis Bulbar palsy Focal seizures with impairment of consciousness or awareness Slurred speech Hemiplegia Hyperactive [mendelian.co]

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Adult Primary Lateral Sclerosis

[…] tendon reflexes, and Babinski signs. [neuropathology-web.org]

progression, with death occurring 2–3 years after onset. [jmg.bmj.com]

Individual variation in rate of progression also is seen in ALS, from rapid decline over a period of months in a small proportion of patients to slow progression over 20 to [clinicalgate.com]

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