Abstract

Common causes of anterior horn cell disease are poliomyelitis, motor neuron disease and spinal muscular atrophy. The primary pathology is the progressive loss of anterior horn cells. We presents a 29 year old female suffering from the motor neuron disease with anterior horn cell involvement since the last five years. She had exhausted all treatment options so was given intrathecal autologous bone marrow derived stem cell therapy as part of the neuroregenerative rehabilitation therapy protocol at our centre. The patient showed functional as well as neurological improvements after receiving the therapy. A detailed case report is presented herewith. Read more...

Abstract

Background: Amyotrophic Lateral Sclerosis (ALS) is a rapidly progressive neurodegenerative disorder which has a fatal prognosis. The primary pathology is loss of motor neurons in the brain and spinal cord. Recently cellular therapy has been studied for ALS in various animal models and has emerged as potential therapeutic modality. Autologous bone marrow mononuclear cells (BMMNCSs) have been studied and found to be ethical, safe and feasible.

Case Report: We present a detailed case report of 41 years old female diagnosed with amyotrophic lateral sclerosis since 3 years. She was given intrathecal autologous bone marrow mononuclear cell therapy combined with riluzole, neurorehabilitation and 6 weeks of lithium. On the outcome measures, Amyotrophic Lateral Sclerosis Functional Rating (ALSFRS-r) score increased from 29 to 32 and Functional Independence Measure (FIM) score increased from 48 to 64.

Conclusion: The highlight of the case study is halting of disease progression with symptomatic improvements over a period of 12 months after intervention. Read more...

Abstract

Motor Neuron Disease (MND) is a progressive neurodegenerative disorder. It selectively damages the motor neurons and is currently an incurable disease. Cellular therapy holds a promising future in the management of MND. We herewith present a case of a 63-year-old man who underwent intrathecal

transplantation of autologous bone marrow mononuclear cells (BMMNCs) as a therapeutic treatment modality in a clinical case ofMND. The transplantation was followed by multidisciplinary neurorehabilitation. Improvements were noted in the muscle strength, fine motor activities,  fasciculation, cramps and walking. The outcome measures of Amyotrophic Lateral Sclerosis Functional Rating ScaleRevised (ALS-FRSR) score improved from 33 to 37; Bergs Balance Score improved from 43 to 50 and 6-minute walk test improved from 283.8 m to 303.6m. His Functional Independence Measure (FIM) remained unchanged at 113.These improvements may be attributed to cellular therapy along with standard treatment and neurorehabilitation. Cellular therapy, if administered in the early stages of disease may have beneficial effects in the treatment of MND. However, rigorous and heterogeneous methodologies are required for definitive findings. Read more...

Abstract

Amyotrophic Lateral Sclerosis (ALS) is a rapidly progressive neurodegenerative disorder. The primary pathology includes progressive loss of motor neurons in the brain and spinal cord. Cellular therapy has recently emerged as a promising treatment modality for patients with ALS. Autologous bone marrow mononuclear cell (BMMNCs) transplantation has been shown to be safe and feasible treatment option in various neurological disorders. Herein we present a detailed case report of a 40 year old female suffering from ALS since 3 years. She was given intrathecal autologous BMMNCs transplantation along with riluzole, lithium and intensive rehabilitation. We monitored the patient over a period of 17 months. Post therapy we observed that there was a slowing of the disease progression, and improvements in neurological symptoms. The outcome measures used were Amyotrophic Lateral Sclerosis Functional Rating (ALSFRS-r) Scale and Functional Independence Measure (FIM) scale. Research shows that ALSFRS-r score deteriorates about 17% every 6 months, but in this patient the ALSFRS-r score dropped only by 8% over 17 months after cell transplantation. This demonstrates that cellular transplantation is safe and has beneficial effects for slowing down the progression of ALS. Read more...

Abstract

Various cellular therapies are being increasingly investigated for the treatment of Amyotrophic Lateral  Sclerosis, a progressive neurodegenerative disease with selective loss of anterior horn cells. Lithium is known to enhance the potency of transplanted cells, while being well tolerated by ALS patients. Additionally, rehabilitation significantly improves outcomes in various neurodegenerative disorders. We present a 47-year-old male patient suffering from ALS for 2 years, whose treatment involved intrathecal transplantation of autologous Bone Marrow-Derived Mononuclear Cells and long-term Lithium, followed by multidisciplinary neurorehabilitation, and standard Riluzole treatment. ALSFRSr score improved from 39 to 41; FIM remained stable at 101; 6MWT distance improved from 396 m to 480 m and Berg Balance score remained stable at 56 over a span of 18 months. Symptomatic improvements were seen in speech, swallow, stamina, walking and muscle strength; fasciculations and cramps reduced drastically. The highlight of this case is the maintenance of the patient’s condition in view of a degenerative prognosis. Cellular therapy along with long-term Lithium and holistic rehabilitation, in addition to standard Riluzole treatment—together termed as Neuroregenerative Rehabilitation Therapy—is a novel approach for halting disease progression and qualitatively improving living conditions, for ALS patients and caregivers alike.  Read more...

Abstract

The known higher incidence of Amyotrophic Lateral Sclerosis (ALS) in men and older age suggests a role of sex steroidal hormones in the disease process. Animal models of ALS have shown lower levels of plasma testosterone. Testosterone is known to exert neuroprotective and neurotrophic actions on neurons. Our objective was to study the association of total testosterone (TT) levels with disease severity on ALS Functional Rating Scale-Revised (ALS FRS-R) scale and King’s Staging. This cross-sectional study included 64 males with definite/probable ALS. Patients’ morning plasma TT levels were tested, and ALS FRS-R and King’s Staging was marked. Standard score was used to compare the deviation of patients’ TT levels from average TT levels of age matched healthy males. A scatter plot was constructed, and correlation analysis was performed using Spearman’s Rank Correlation. 39/64 patients (60.9 %) had TT levels that were lower by 1/more standard deviation, than age matched average levels in healthy men. There was a statistically significant positive correlation of TT levels with ALS FRS-R (r=.326, p=0.009) and negative correlation with King’s Staging (r=-.312, p=0.012). Thus, with declining function and disease progression, standard scores of TT levels decreased. Maintaining plasma TT levels, as close to age matched average levels, may be explored as an adjuvant therapy in ALS.  Read more...