A previously healthy 40-year-old man presented to our movement disorders clinic in 2000 with a mild right akinetic-rigid syndrome; the symptoms had started 1 year before. The neurological examination was otherwise normal. There was no previous exposure to dopamine antagonists and no history of cerebral vascular events. The family history was also unremarkable. PD was diagnosed after careful examination and testing, including blood tests, brain MRI, and I-FP-CIT SPECT. Parkin mutation search was negative. One year later, a dull nuchal pain and subsequent dropped head (DH) emerged (Fig. 1), which progressed for a few weeks and then stabilized; no other new neurological manifestations appeared. At this time the patient was on no medication. Laboratory investigations were normal, including complete blood count, electrolytes, liver enzymes, glucose, creatinine, blood urea nitrogen, thyroid function tests, serum CK and inflammation markers; acetylcholine receptor antibodies were negative. IBZM SPECT was compatible with PD. EMG was conducted at that time and repeated 1 year later, showing no changes regarding the trapezius, sternocleidomastoid or scapular muscles; repetitive stimulation was normal. Cervical MRI showed mild disk bulging, but no other changes were apparent. Levodopa/carbidopa (100/25 mg) was introduced tid, with mild improvement of head drop. Cervical MRI was repeated in 2007 but no new relevant findings were observed, and cervical muscles were apparently intact. Repeat EMG examination disclosed normal muscle activity in the trapezius and sternocleidomastoid muscles, but a myopathic pattern was seen in the cervical paraspinal muscles bilaterally. Paraspinal muscle biopsy was then performed, and read by a muscle disorders expert neuropathologist, but only mild nonspecific changes were apparent (Fig. 2). The clinical course has been otherwise quite as expected, with akinesia and rigidity predominantly involving right sided limbs, and the emergence of motor fluctuations and disabling dyskinesias. Using the Unified Parkinson’s Disease Rating Scale part III, the patient scored a total of 40 while off medication for 12 h, and 9 during the best on state after the acute levodopa challenge. Formal cognitive tests revealed MMSE 30/30, Frontal Assessment Battery 16/18 and the total score of Mattis Dementia Rating Scale was 138/144; all these are normal scores as compared to an ageand education-matched normative population. The patient did not have any behavioural disturbances, as assessed by the clinical interview and using the Beck Depression Inventory and the Brief Symptom Inventory. He denied any clinically significant dysautonomic features, such as erectile dysfunction, bladder problems, constipation or symptomatic orthostatic hypotension, and there were no symptoms suggestive of REM sleep behaviour disorder, although no formal autonomic or sleep studies have been conducted. The patient underwent bilateral deep brain stimulation (DBS) of the subthalamic nucleus in 2010, with significant improvement of the parkinsonian symptoms and fluctuations but no effect on the dropped head. Very similar results had been achieved from a previous acute levodopa A. Oliveira J. Massano M. J. Rosas Movement Disorders and Functional Surgery Unit, Centro Hospitalar de Sao Joao, Porto, Portugal