Implantable Subcutaneous Peripheral Nerve Stimulation Improves Degenerative Ataxia.

Degenerative cerebellar ataxias have no pharmacological or rehabilitation evidence-based treatment so far. Patients remain highly symptomatic and disabled despite receiving the best medical treatment available. This study investigates the clinical and neurophysiologic outcomes of the use of subcutaneous cortex stimulation (in keeping with the established protocol of peripheral nerve stimulation applied in chronic intractable pain) in degenerative ataxia. We report a case of a 37-year-old right-handed man who developed moderate degenerative cerebellar ataxia at the age of 18 years. His symptoms progressively worsened and impaired his daily activities. We observed clinical improvement for at least one month following an initial two-week trial of parietal transcranial direct current stimulation. Although preoperative non-invasive transcranial neuromodulation application does not predict invasive cortex stimulation outcome, we pursued a long-lasting effect by implanting parietal and occipital subcutaneous electrodes. At 12 months following permanent implantation, the patient exhibited amelioration of his symptoms and a change in neurophysiologic parameters. Central neuromodulation based on peripheral stimulation is considered part of neurosurgical clinical practice for the treatment of a variety of neurological disorders. The underpinning neurophysiological mechanism that explains the effectiveness of the method has not been fully elucidated. We believe that further studies are warranted to investigate these promising results in such devastating conditions.

Spinal synovial cysts. A case series and current treatment options.

Synovial cysts constitute an uncommon degenerative lesion of the spine. They are usually asymptomatic but they may also cause symptoms of variable severity. The authors present three cases of such cysts, two in the lumbar region of a 55-year-old female and a 66 year old female and one in the cervical region of a 56-year-old male patient. All patients presented with radiculopathy. Magnetic Resonance Images revealed a cystic lesion at the L4/5 level in the first case, at L5/S1 level in the second case and at the C7/T1 junction level in the third case. Treatment has been microsurgical resection of all cysts with no post-operative complications and an excellent outcome. A discussion of current management options for this unusual disease is presented and a decision making flow chart is proposed.

Medulloblastoma in a child with fragile X syndrome.

Fragile X syndrome is the most common cause of inherited intellectual impairment and has been associated with decreased incidence of cancer. We present the case of an 11-year-old boy with a diagnosis of fragile X syndrome who presented with gait imbalance, headache, and episodes of vomiting. Radiological investigation revealed the presence of a posterior fossa tumor. The patient was operated upon and the tumor proved to be a medulloblastoma (MB). To the best of our knowledge this is the first reported case of MB in a child with fragile X syndrome.