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12 Possible Causes for Early Upper Motor Neuron Signs, Generalized Dystonia, Lower Motor Neuron Signs - Later

  • Mitochondrial Membrane Protein-Associated Neurodegeneration

    Conclusions MPAN is characterized by a juvenile‐onset, slowly progressive phenotype with predominant lower limb spasticity, generalized dystonia, and cognitive impairment.[ingentaconnect.com] Early upper motor neuron signs (pyramidal signs, e.g. spasticity) are constant findings and are later followed by signs of lower motor neuron dysfunction (deep tendon reflex[malacards.org] Conclusions MPAN is characterized by a juvenile-onset, slowly progressive phenotype with predominant lower limb spasticity, generalized dystonia, and cognitive impairment.[push-zb.helmholtz-muenchen.de]

  • Juvenile Primary Lateral Sclerosis

    CONCLUSIONS: We report generalized dystonia and cerebellar signs in association with ALS2-related disease.[foundationdystoniaresearch.org] El Escorial criteria for the diagnosis of amyotrophic lateral sclerosis 7 : it requires the presence of signs of lower motor neuron (LMN) degeneration by clinical, electrophysiological[radiopaedia.org] During the second year of life, they lose the ability to walk (some patients never walk due to early severe spasticity) and then develop slowly progressive upper motor neuron[orpha.net]

  • Amyotrophic Lateral Sclerosis

    It can be localized to a certain group of muscles or can be generalized (180).[functionalneurology.com] It is one of many motor neuron diseases, but what makes it stand out is that it presents with both upper and lower motor neuron signs.[symptoma.com] Patients present with a pure upper motor neurone syndrome with either absent or minimal lower motor neurone signs.[doi.org]

  • Lower Motor Neuron Syndrome with Late-Adult Onset

    Generalized dystonia (e.g., dystonia musculorum deformans) is an inherited condition in which persistent and often painful twisting and writhing movements of any muscle group[britannica.com] Unlike amyotrophic lateral sclerosis, lower motor neuron findings, such as atrophy and fasciculations, are not as prominent.[verywell.com] […] bulbospinal muscular atrophy Generalized epilepsy with febrile seizures-plus Generalized epilepsy-paroxysmal dyskinesia syndrome Generalized isolated dystonia Genetic central[se-atlas.de]

  • Spastic Quadriplegic Cerebral Palsy Type 2

    With significant dystonia/involuntary movements Dystonia and involuntary movements (specifically choreoathetosis) are not as common in CP as is generally believed.[adc.bmj.com] ALS is a progressive neurodegenerative disease involving both the upper motor neuron (UMN) and lower motor neuron (LMN).[cmm.ucsd.edu] No signs of lower motor neuron involvement were observed, whereas motor evoked potentials demonstrated predominant involvement of the upper motor neurons.[neurology.org]

  • Meningeal Disorder

    Generalized dystonia (e.g., dystonia musculorum deformans) is an inherited condition in which persistent and often painful twisting and writhing movements of any muscle group[britannica.com] motor neuron weakness; Tabes dorsalis (involvement of spinal cord dorsal roots) Lyme Disease- deer tick Symptoms – early rash; later neurologic manifestations after several[brainaacn.org] […] is bent forwards, backwards, or to the side that are present in normal infants and are incorporated in many bodily movements of normal children and adults tonic seizure generalized[strokecenter.org]

  • Autosomal Recessive Spinocerebellar Ataxia Type 17

    EFNS guidelines on the molecular diagnosis of neurogenetic disorders: general issues, Huntington's disease, Parkinson's disease and dystonias.[ncbi.nlm.nih.gov] Familial Amyotrophic Lateral Sclerosis (SOD1 Mutation) Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both the upper motor neurons[aetna.com] Clues to SCAs Age at onset: childhood onsetSCA 7, 13, DRPLA Young adult: SCAl, 2, 3, 21older adult: SCA 6 Prominent anticipation SCA 7, DRPLA Upper motor neuron signs SCA[slideshare.net]

  • Alopecia - Hypogonadism - Extrapyramidal Syndrome

    The index patient was a 25-year-old woman who presented with generalized dystonia involving the trunk and all four limbs.[cags.org.ae] […] neuronopathy with early upper-motor neuron findings followed later by signs of lower-motor neuron dysfunction, and a slowly progressive disease course.[nbiacure.org] Physical examination on admission disclosed generalized dystonia involving the trunk and all four limbs.[e-sciencecentral.org]

  • Amyotrophic Lateral Sclerosis Type 17

    A recent study reported patients with ALS2 with nonsense and frameshift mutations in the Alsin gene who presented with generalized dystonia and cerebellar signs [ 10 ].[link.springer.com] neuron disease variant with only lower or upper motor neuron signs Primary lateral sclerosis (upper motor neuron only) ?[neuromuscular.wustl.edu] Early on, the primary sign may be fasciculations, but eventually, progresses with both upper and motor neuron signs and symptoms.[verywell.com]

  • Spinocerebellar Ataxia with Rigidity and Peripheral Neuropathy

    Machado-Joseph disease presenting as severe generalized dystonia in a German patient. J Neurol 1999 ; 246 : 840 –2. Garcia Ruiz PJ , Mayo D, Hernandez J, et al.[pmj.bmj.com] Amyotrophic lateral sclerosis •Upper motor neuron loss: -Corticospinal tracts.[quizlet.com] SCA-7 typically presents with progressive ataxia, upper motor neuron signs (brisk reflexes), macular or retinal degeneration with visual loss, and slow saccades.[neuroweb.us]

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