{'fraction_non_alphanumeric': 0.020757020757020756, 'fraction_numerical': 0.004884004884004884, 'mean_word_length': 5.87248322147651, 'pattern_counts': {'":': 0, '<': 0, '<?xml version=': 0, '>': 1, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
Neurological disorders that may deteriorate with intensive physical rehabilitation

Rare neurological conditions may deteriorate with intensive rehabilitation. Two rare conditions occurring in adulthood with underlying mitochondrial dysfunction are presented – mitochondrial encephalopathy, lactic acidosis with stroke-like episodes and multiple acyl-CoA dehydrogenase deficiency. The cases highlight that mitochondrial dysfunction should be thought of if clinical deterioration occurs despite intensive rehabilitation. It is widely accepted that physical rehabilitation is beneficial in a wide range of acute and chronic neurological conditions. There is evidence that multidisciplinary neurorehabilitation can reduce disability, improve quality of life and lead to earlier discharge home. Rarely, however, some neurological disorders do not improve and may paradoxically deteriorate with conventional physical therapies. To highlight these exceptions, we present two patients with rare disorders, who both deteriorated during periods of intense rehabilitation and improved spontaneously once physical therapies were ceased. A 60-year-old woman with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) presented with new onset aphasia and right-sided hemiparesis. The diagnosis of MELAS had previously been made on the basis of a classical clinical presentation of bilateral sensorineural hearing loss, myopathy characterised by facial and proximal limb weakness and a focal seizure disorder with a raised serum lactate during exacerbations. Additionally, she had an extensive family history of the disorder (in her mother, three brothers and two sisters) and an affected brother was known to carry the m.3243A > G mutation. A magnetic resonance imaging revealed an area of left hemispheric acute infarction with predominantly cortical involvement. During her hospital admission whilst receiving intensive rehabilitation with physiotherapy, occupational therapy and speech therapy, she progressively declined with worsening right-sided weakness, cognitive impairment and drowsiness. There was no evidence of nonconvulsive status on an electroencephalogram. Serial magnetic resonance imaging revealed progressive increase in the size of the acute infarction without evidence of haemorrhagic transformation (Fig. 1). After discussion with the patient’s family, a palliative approach was commenced, and goal-based physical therapies were ceased. She was discharged home with community palliative care support. After 2 weeks of resting at home, she significantly improved, regaining independent mobility, functional communication and improved level of alertness. She has been able to reside at home, independent with activities of daily living and mobile short distances without aid, for 16 months. A 70-year-old woman presented with a long history of unexplained intermittent proximal limb weakness since the age of 18 years. Examination revealed upper and lower limb proximal weakness (Medical Research Council grade 4/5), normal bulbar function, retained reflexes and a normal sensory examination. Electromyography of affected muscles revealed small motor units. Following a neurological consultation, she was struggling to live independently at home due to limb weakness, and she was offered a period of rehabilitation. During her rehabilitation period whilst receiving daily physiotherapy, she significantly deteriorated with worsening proximal weakness (proximal upper and lower limb muscle power with Medical Research Council grade 3/5) and immobility requiring a wheelchair. At that stage, residential care was considered. A muscle biopsy showed Figure 1 Axial fluid-attenuated inversion recovery (FLAIR) MRI sequence of the brain at the level of the upper pons. Left: on admission showing a small region of FLAIR hyperintensity in the left anterior temporal region. Right: 3 months later after rehabilitation showing significant increase in the distribution of the FLAIR hypertensity extending into the left temperoparietal and occipital regions. bs_bs_banner