What is the diagnosis and treatment for Split Hand Amyotrophic Lateral Sclerosis (ALS)?

From the Guidelines

Split hand amyotrophic lateral sclerosis (ALS) is a specific presentation of ALS that requires a multidisciplinary approach for optimal management, focusing on symptom management, slowing disease progression, and improving quality of life. This condition is characterized by predominant weakness and wasting of the hand muscles, particularly affecting the thenar (thumb side) muscles more severely than the hypothenar (pinky side) muscles, creating a distinctive "split hand" appearance. The diagnosis of split hand ALS is based on clinical presentation, electromyography, and nerve conduction studies.

Treatment Approach

The treatment approach for split hand ALS includes:

• FDA-approved medications such as riluzole (50 mg twice daily) and edaravone (60 mg IV daily for 14 days, followed by 14-day drug-free periods) to slow disease progression and extend survival 1
• Supportive care, including physical therapy to maintain mobility, occupational therapy for adaptive equipment, and respiratory support as needed
• Nutritional management, which may require eventual placement of a percutaneous endoscopic gastrostomy (PEG) tube, as weight loss is associated with decreased survival in ALS patients 1
• Dietetic counseling to adapt the texture of solid and liquids to facilitate swallowing and avoid aspiration, as well as postural maneuvers such as chin-tuck posture to protect the airway during swallowing 1

Nutritional Management

Nutritional management is crucial in split hand ALS, as weight loss is associated with decreased survival. Oral nutritional supplementation and enteral nutrition may stabilize body weight in ALS patients 1. However, the evidence for improved survival with enteral nutrition by PEG is conflicting, with the American Academy of Neurology stating that it may improve survival, while the European Federation of Neurological Societies reports no convincing evidence 1.

Multidisciplinary Care

Early diagnosis and multidisciplinary care at specialized ALS centers are essential for optimal management of this progressive neurodegenerative condition. A team of healthcare professionals, including neurologists, physical therapists, occupational therapists, and nutritionists, can provide comprehensive care and support to improve quality of life and slow disease progression.

From the FDA Drug Label

Riluzole tablets is indicated for the treatment of amyotrophic lateral sclerosis (ALS) 2 The efficacy of riluzole tablets was demonstrated in two studies (Study 1 and 2) that evaluated riluzole tablets 50 mg twice daily in patients with amyotrophic lateral sclerosis (ALS) 2

The diagnosis of Split Hand Amyotrophic Lateral Sclerosis (ALS) is not directly addressed in the provided drug labels. The treatment for Amyotrophic Lateral Sclerosis (ALS) is riluzole. However, there is no information on the specific treatment for Split Hand ALS.

From the Research

Diagnosis of Split Hand Amyotrophic Lateral Sclerosis (ALS)

• Split Hand is characterized by dissociated hand muscle atrophy, with preferential atrophies in the thenar and first dorsal interosseous muscles, and relative sparing of the hypothenar muscle 3
• This symptom is recognized as specific to amyotrophic lateral sclerosis (ALS) and distinct pathomechanisms are assumed to underlie this phenomenon 3

Treatment of Split Hand Amyotrophic Lateral Sclerosis (ALS)

• Riluzole is a neuroprotective treatment that can be used to slow down the progression of ALS 4, 5, 6
• Edaravone is another treatment that has been approved for the treatment of ALS, and it has been shown to slow down disease progression when combined with riluzole 4, 6
• Physical therapy, including stretching exercises, proprioceptive neuromuscular facilitation techniques, and functional mobility training, can help to preserve optimal quality-of-life throughout the course of the disease 7
• A multidisciplinary approach, including the involvement of neurologist, physicians, nurse practitioners, physical and occupational therapists, speech language therapists, dietitians, and psychologists, is recommended for the management of ALS 5

Management of ALS

• A quarterly-based management is proposed, coordinated by an ALS center in close collaboration with the general practitioner, home care organization, and a dedicated health network 5
• Assessments and follow-up should concern mainly motor impairments and physical disability, adaptations, nutritional and respiratory functions, as well as associated symptoms such as pain, spasticity, and mood disorders 5
• Decisions about nutritional and respiratory assistive devices require a collaborative approach involving specialized practitioners and paramedical professionals, and should be anticipated in agreement with the patient, the family, and the trusted person 5