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The neurocutaneous syndromes comprise a diverse group of rare genetic disorders with both neurological and cutaneous manifestations. Each syndrome has a distinct phenotype. Symptoms are variable and depend on the syndrome. Neurocutaneous syndromes often present in childhood or adolescence; for example, tuberous sclerosis typically presents in early childhood. The age range of presentation is broad, depending on the specific condition and severity of expression. The majority are autosomally inherited conditions. De novo mutations can occur. Most neurocutaneous syndromes do not have a specific treatment, and management is predominantly supportive and aimed at symptom reduction and appropriate monitoring. This chapter discusses neurocutaneous syndromes, including their symptoms, demographics, etiologies, natural history, complications, diagnosis, prognosis, and treatment.

Neurological complications of systemic malignancy are frequent. They may reflect direct local effects of the tumour; CNS infection; side effects of chemotherapy or radiotherapy; nutritional or metabolic derangements; or a paraneoplastic syndrome. The paraneoplastic neurological syndromes are a group of disorders associated with a malignancy outside the nervous system. The pathophysiology is immune-mediated, with the tumour’s expression of neuronal proteins invoking antibody formation, which in turn results in neurological symptoms. This chapter will mainly focus on these syndromes.

IntroductionLong term outcomes in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are reported to be favourable in 80% of cases, but there is little information regarding recovery following prolonged severe neurological disability.Case 1A 16-year-old female presented with headache, fever and acute encephalopathy. Anti-NMDAR antibodies were positive, with an ovarian teratoma. Treatment included oophorectomy, plasmapheresis, corticosteroids, cyclophosphamide, intravenous and intrathecal rituximab, and alemtuzumab. After 15 months hospitalisation, she remained in a vegetative state, with tracheostomy and percutaneous gastrostomy tubes, on multiple anticonvulsants. She received no further immunotherapy. MRI brain 2 years after illness onset demonstrated severe generalised cerebral atrophy. Three years after onset, she began to improve. At 5+ years she was seizure-free without anticonvulsants, fully independent and ambulatory, without overt cognitive deficits or personality change. MRI brain at 5 1/2 years demonstrated recovery of cerebral volume to within normal limits, but residual hippocampal atrophy.Case 2A 21-year-old female presented with headache, fever and personality change. NMDAR-antibodies were positive. A malignant ovarian teratoma was treated with salpingoophorectomy and chemotherapy (bleomycin/etoposide/cyclophosphamide). She received plasmapheresis, immunoglobulins, corticosteroids, intravenous and intrathecal rituximab, and bortezomib. She was finally discharged after 21 months hospitalisation with tracheostomy and gastrostomy tubes, severe behavioural disturbance, minimal functional limb movement, and fully dependent care needs. Clinical recovery began 2 1/2 years after disease onset, and at 4 years she is fully independent with residual cognitive deficits, engaging in volunteer work.ConclusionsThese cases highlight the potential for dramatic delayed clinical improvement despite prolonged severe neurological disability in anti-NMDAR encephalitis.

The flavonoid components of tea have been associated in epidemiological studies with a decreased risk of cardiovascular disease. Flavonoids have been shown to have antioxidant and vasodilator effects in vitro; we therefore postulated that drinking green or black tea attenuates the well-characterized acute pressor response to caffeine and lowers blood pressure during regular consumption.To determine whether green and black tea can attenuate the transient pressor effect of caffeine, or lower blood pressure during regular consumption.In the first study, the acute effects of four hot drinks - green tea and black tea (at a dose equivalent to four standard cups), water matched to the teas for caffeine content ('caffeine') and water - were assessed in 20 normotensive men using a Latin-Square designed study. Clinic blood pressure was measured before and 30 and 60 min after each drink had been ingested. In the second study, the effects on blood pressure of regular green and black tea ingestion were examined in 13 subjects with high-normal systolic blood pressure and mild systolic hypertension (systolic blood pressure in the range 130-150 mmHg) using a three-period crossover study. Five cups per day of green tea, black tea and caffeine (in hot water and matched to the teas) were consumed for 7 days each, in random order. Twenty-four hour ambulatory blood pressure was measured at the end of each seven-day intervention. Results are presented as means and 95% confidence intervals (CI).An acute pressor response to caffeine was observed. Relative to caffeine, there were further acute increases in systolic and diastolic blood pressure at 30 min among those drinking green tea [5.5 mmHg (95%CI -1.4 to 12.4) and 3.1 mmHg (95%CI -0.1 to 6.3), respectively] and black tea [10.7 mmHg (95%CI 4.0 to 17.4) and 5.1 mmHg (95%CI 1.8 to 8.4), respectively]. The changes in blood pressure at 60 min were not significant The effect on 24-h ambulatory systolic and diastolic blood pressure of regular drinking of green tea [increases of 1.7 mmHg (95%CI -1.6 to 5.0) and 0.9 mmHg (95%CI -1.3 to 3.1), respectively] or black tea [increase of 0.7 mmHg (95%CI -2.6 to 4.0) and decrease of 0.7 mmHg (95%CI -2.9 to 1.5), respectively] was not significant relative to caffeine.Contrary to our initial hypothesis, tea ingestion caused larger acute increases in blood pressure than caffeine alone. However, any acute effects of tea on blood pressure did not translate into significant alterations in ambulatory blood pressure during regular tea consumption.