amyotrophic lateral sclerosis type 28

Amyotrophic Lateral Sclerosis (ALS) Type 28, also known as ALS28, is a rare and specific subtype of Amyotrophic Lateral Sclerosis.

Characteristics of ALS28:

• Adult onset: ALS28 typically affects adults, with symptoms appearing in the fourth to sixth decade of life.
• Progressive limb muscle weakness and atrophy: The disorder is characterized by slowly progressive limb muscle weakness and atrophy, leading to gait difficulties.
• Parkinsonism: In addition to motor neuron symptoms, ALS28 also presents with parkinsonian features, such as tremors, rigidity, and bradykinesia (slow movement).
• Motor neuron degeneration: The disorder is marked by the progressive degeneration of motor neurons in the brain and spinal cord.

Comparison with other forms of ALS:

• ALS28 is distinct from other forms of ALS, such as sporadic ALS or familial ALS, due to its specific combination of symptoms.
• It shares some characteristics with other rare subtypes of ALS, but its unique presentation sets it apart.

Prevalence and prognosis:

• The prevalence of ALS28 is not well established, as it is a rare subtype of ALS.
• Prognosis for ALS28 patients is generally poor, with a rapid progression of symptoms leading to significant disability and eventual death.

It's worth noting that the information available on ALS28 is limited due to its rarity. Further research is needed to better understand this specific subtype of ALS and to develop effective treatment strategies.

Early Signs and Symptoms of ALS

ALS, also known as Amyotrophic Lateral Sclerosis, typically announces itself with persistent weakness or spasticity in an arm or leg (80% of all cases), causing difficulty using the affected limb [1]. Other early symptoms vary but can include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches, and progressive weakness [2-5].

Progression of Symptoms

The symptoms of ALS progress over time and ultimately lead to ventilatory failure because affected individuals lose the ability to control their breathing muscles. As the disease progresses, individuals may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms [9].

Key Signs and Symptoms

• Persistent weakness or spasticity in an arm or leg (80% of all cases)
• Tripping, dropping things
• Abnormal fatigue of the arms and/or legs
• Slurred speech
• Muscle cramps and twitches
• Progressive weakness
• Fatigue
• Poor balance
• Slurred words
• Weak grip
• Tripping when walking

References

[1] ALS typically announces itself with persistent weakness or spasticity in an arm or leg (80% of all cases), causing difficulty using the affected limb. [2] Other early symptoms vary but can include tripping, dropping things, abnormal fatigue of the arms and/or legs, slurred speech, muscle cramps and twitches and ... [3] The symptoms of ALS progress over time and, ultimately, the disease leads to ventilatory failure because affected individuals lose the ability to control ... [4] Progressive limb weakness. Asymmetrical onset; combined UMN and LMN signs in arm(s); spastic paraparesis; occasionally fasciculations in arms. [5] UMN signs are mild weakness, spasticity, abnormally brisk reflexes, and pathological pyramidal reflexes; LMN signs are progressive weakness, ... [9] The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking, or other minor symptoms. Sometimes this stage occurs before ...

Based on the provided context, it appears that Amyotrophic Lateral Sclerosis (ALS) type 28 is not a recognized or established classification of ALS in medical literature.

However, I can provide information on the diagnostic tests for ALS in general. According to search result [10], laboratory tests are performed primarily to rule out other disease processes; results generally are normal in ALS.

The diagnosis of ALS typically involves a combination of clinical examinations, laboratory and genetic tests, nerve conduction/needle electromyography (EMG), and sometimes a spinal tap. These tests can help exclude other conditions that may cause similar symptoms.

Here are some diagnostic tests for ALS:

• Clinical examination: A thorough physical exam by a neurologist to assess muscle strength, reflexes, and other motor functions.
• Nerve conduction studies (NCSs): Tests that measure the speed and strength of electrical signals traveling through nerves.
• Electromyography (EMG): Tests that record the electrical activity of muscles at rest and during contraction.
• Spinal tap: A procedure to collect cerebrospinal fluid for analysis, which can help rule out other conditions.

It's worth noting that a definitive diagnosis of ALS is often made by excluding other possible causes of symptoms through a combination of these tests.

Based on the context provided, it seems you're looking for information on drug treatments for a specific subtype of Amyotrophic Lateral Sclerosis (ALS), which is not clearly specified in the snippet.

However, I can provide some general information on ALS treatment. ALS is a progressive neurodegenerative disease with no cure. The primary goal of treatment is to manage symptoms and slow disease progression.

Two FDA-approved medications are currently available for treating ALS:

1. Riluzole (Rilutek): This is the only medication approved by the

The differential diagnosis of amyotrophic lateral sclerosis (ALS) involves identifying conditions that may present with similar symptoms to ALS. Based on the search results, here are some key points to consider:

• Bilateral tongue fasciculation: This symptom strongly suggests a diagnosis of ALS [1]. However, it's essential to note that other conditions can also cause fasciculations.
• UMN and LMN involvement: ALS affects both upper motor neurons (UMN) and lower motor neurons (LMN), making clinical diagnosis typically supported by the presence of both UMN and LMN signs [3].
• Bulbar symptoms: When a patient presents with bulbar symptoms, the differential diagnosis includes myasthenia gravis, Kennedy's disease, syringobulbia, and foramen magnum lesions [5].
• ALS mimic syndromes: ALS must be differentiated from unrelated disorders that may have similar presentations, such as "ALS mimic syndromes" [6].
• Predominant UMN or LMN involvement: In patients with predominant UMN or LMN involvement, the differential diagnosis becomes broader. For example, in patients with predominant UMN involvement, conditions like syrinx, mass, stroke, and demyelinating forms of other degenerative diseases should be considered [8].

Some key conditions to consider in the differential diagnosis of ALS type 28 include:

• Myasthenia gravis: A chronic autoimmune disorder that can cause muscle weakness and fatigue.
• Kennedy's disease: A rare genetic disorder that affects motor neurons, leading to progressive muscle weakness and atrophy.
• Syringobulbia: A congenital or acquired condition characterized by a fluid-filled cavity in the brainstem, which can cause bulbar symptoms.
• Foramen magnum lesions: Lesions located near the foramen magnum (the opening at the base of the skull) that can compress the spinal cord and cause neurological symptoms.

It's essential to note that a definitive diagnosis of ALS requires a comprehensive clinical evaluation, including medical history, physical examination, laboratory tests, and electrodiagnostic studies [7].

References:

[1] M de Carvalho (2024) [3] M de Carvalho (2023) [5] H Mitsumoto (1997) [6] LC Wijesekera (2009) [7] Apr 11, 2024 [8] P Masrori (2020)