Serum anti-aquaporin 4 (AQP4) antibody is detected with high specificity in patients with neuromyelitis optica (NMO), characterised by recurrent optic neuritis and longitudinally extensive transverse myelitis [1,2]. Three NMO patients with anti-AQP4 antibody were reported to have hypothalamic lesions, showing reduced hypocretin-1 levels in cerebrospinal fluid (CSF) and symptomatic narcolepsy [3]. Although the hypothalamus and hypocretin-1 regulate autonomic functions as well as sleep, autonomic functions in NMO patients with hypothalamic lesions have not been described well. Here we report the case of a patient with anhidrosis and narcolepsy as initial symptoms of disorders related to anti-AQP4 antibody.A 41-year-old woman was experiencing excessive daytime sleepiness. Cataplexy was not evident. One month after, she became aware of anhidrosis on the left side of her body including the face. The following month, she was admitted to our hospital because of hypersomnia and anhidrosis that extended throughout her entire body.Physical and neurological examination revealed hypotension (93/62 mmHg) and dry skin. Brain magnetic resonance imaging (MRI) showed a high signal intensity area around the third ventricle on fluid-attenuated inversionrecovery image (Fig. 1a). Multiple sleep latency test showed shortening of the mean sleep latency (4.8 min; normal: [8 min) and sleep-onset REM periods. Lumbar puncture showed only a decrease of CSF hypocretin-1 levels (177 pg/ml, normal:[200 pg/ml). Serum anti-AQP4 antibody was positive. Although sympathetic skin responses (SSR) were absent in all limbs (Fig. 1c), intracutaneous acetylcholine injection caused normal sweating reaction.The patient was diagnosed with NMO spectrum disorders having narcolepsy and anhidrosis, secondary to hypothalamic lesions associated with anti-AQP4 antibody, and treated with intravenous methylprednisolone (1 g/day) for 3 days, followed by oral prednisolone (1 mg/kg/day). After treatment, her excessive sleepiness and anhidrosis disappeared in a few days. MRI showed improvement of the abnormal intensity area (Fig. 1b), and CSF hypocretin-1 levels had recovered to 213 pg/ml. Moreover, repeated tests of SSR showed positive responses (Fig. 1d).Anhidrosis of the entire body was detected and confirmed by SSR in our patient associated with anti-AQP4 antibody as well as symptomatic narcolepsy. After treatment, the reduced CSF hypocretin-1 levels returned to normal in parallel with improvement of excessive daytime sleepiness, similar to previously reported NMO cases [3]. The hypothalamus is the highest level of integration of autonomic function; tumors, trauma, inflammation, or vascular disorders in this structure are known to cause autonomic dysfunctions such as hypothermia or abnormal