suMMARY The frequency of shoulder disturbances, particularly frozen shoulder, has not been assessed previously in Parkinson's disease. In a survey of 150 patients compared with 60 matched control subjects a significantly higher incidence of both a history of shoulder complaints (43% vs. 23%) and frozen shoulder (12.7% vs. 17%) was found in the Parkinson's disease population. Those developing a frozen shoulder had initial disease symptoms indicative ofakinesia twice as frequently as tremor while the ratio was reversed in those without frozen shoulder. In at least 8% of the patients frozen shoulder was the first symptom of disease, occurring 0-2 years prior to the onset of more commonly recognised features. Parkinson's disease should be added to the list of causes of frozen shoulder, and clinicians must be aware that the latter is often the presenting symptom of Parkinson's disease.The occurrence of shoulder joint abnormalities has not, to our knowledge, been studied in the setting of Parkinson's disease. Although it seems intuitive that immobilised patients in the later stages of their illness might have a high incidence of shoulder disturbances, we have been impressed that a number of our patients have experienced difficulties before other features of Parkinson's disease were recognised. A systematic survey was undertaken to determine how important a problem this was in the Parkinson's disease population. The incidence of complaints related to the shoulder joints in a consecutive series of patients was examined, with specific interest in determining the frequency of frozen shoulder and the relation between its development and the timing and nature ofthe onset of Parkinson's disease.

Epilepsy represents a multifaceted group of disorders divided into two broad categories, partial and generalized, based on the seizure onset zone. The identification of the neuroanatomic site of seizure onset depends on delineation of seizure semiology by a careful history together with video-EEG, and a variety of neuroimaging technologies such as MRI, fMRI, FDG-PET, MEG, or invasive intracranial EEG recording. Temporal lobe epilepsy (TLE) is the commonest form of focal epilepsy and represents almost 2/3 of cases of intractable epilepsy managed surgically. A history of febrile seizures (especially complex febrile seizures) is common in TLE and is frequently associated with mesial temporal sclerosis (the commonest form of TLE). Seizure auras occur in many TLE patients and often exhibit features that are relatively specific for TLE but few are of lateralizing value. Automatisms, however, often have lateralizing significance. Careful study of seizure semiology remains invaluable in addressing the search for the seizure onset zone.

ABSTRACT:The amplitude and temporal modulation of the segmented EMG activity in flexor carpi radialis, evoked by imposed angular wrist extension, was studied with respect to the level of pre-existing background activity in rigid parkinsonian (PK) and dystonia musculorum deformans (DMD) patients. The interdependence of the evoked M1 and M2-3 segments on pre-existing background EMG activity and initial velocity of imposed displacement was established previously for a normal population. Individual responses of 21 parkinsonian and 12 dystonic patients were compared to the established normal “response volume”. The augmented magnitude of the M2-3 segment in rigid PK patients, which correlates to the measure of rigidity, could not be accounted for by the low level of pre-existing EMG activity. Therefore, increased descending facilitation does not impinge directly on alpha motoneurons. Paradoxical excitation in the shortened muscle and resetting of tonic tremor of the stretched muscle by the imposed wrist extension are two other demonstrated abnormalities which may also contribute to PK rigidity. In contrast, DMD patients demonstrated normal amplitude modulation of the M1 and M2-3 segments, but exhibited a disturbance of normal temporal mechanisms that result in constant duration of the M1 and M2-3 responses with imposed force step loads.

Paralysis of horizontal gaze, pendular nystagmus, and progressive scoliosis were manifestations of an autosomal recessive genetic disease in four siblings. Bilateral facial myokymia with continuous facial contraction developed in the oldest patient. Electromyographic examination of his facial muscles after facial nerve block at the stylomastoid foramen showed absence of all muscle potentials, consistent with a supranuclear origin of the myokymia. Normality of convergence, vertical gaze, and pupillary constrictor reflex activity assured integrity of midbrain ocular motor function. Absence of horizontal vestibulo-ocular reflexes signified involvement of the pontine tegmentum in this distinctive heredofamilial syndrome.

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Geraghty and colleagues [3] recently reported a subgroup of patients with essential tremor (ET) who later developed Parkinson's disease (PD). In agreement with certain previous studies El, 2 , 4 , 8 ] but not with others [5-71, they concluded that some patients with essential tremor are genetically at risk for Parkinson's disease, the prevalence of parkinsonism in their population of patients with ET being 24 times greater than expected. Approaching the same question in a different manner, we recently obtained detailed family histories emphasizing the presence of tremor and features of parkinsonism in 159 patients with idiopathic PD. We compared the data from these patients with that for 104 age-and sex-matched controls who did not have P D and with that of 90 patients with various forms of idiopathic dystonia, a condition recognized to have an association with ET. Patients and controls were excluded if they were adopted or knew little about their families. Immediate family members were excluded if they had died early in life before an age at which tremor might have developed. For this same reason, the incidence of tremor in a parent and ssbling group was calculated separately from that for all immediate family members, as children may have been too young to develop tremor. This effect must be considered when comparing the dystonia group with the other two groups because these patients, and therefore their parents and siblings, were younger than those in the P D and control groups.Of the PD group, 17.0% had at least one immediate family member with tremor that was unassociated with other features of parkinsonism or another obvious cause. This figure was significantly (p < 0.01) greater than the 5.6% found in the control group. O f dystonics, 10% had a similar family history that was not markedly different from that of the other groups. The frequency of tremor in immediate family members (all immediate family memberdparents and siblings only) was also significantly higher in the PD group (2.7%/3.7%) compared with the controls (0.8%/l.l%) while the frequency in the patients with dystonia (l.?%/ 2.1%) did not differ from either of the other groups. Patients who were a younger age at the onset of PD (under 40) and those with tremor as the first symptom of their P D were more likely to have a positive family history of tremor, but these associations were not statistically significant. The frequency of a positive family history of PD and the prevalence of P D in first-degree relatives was the same in the three groups.In most cases a parent was the only family member affected by tremor. Although only 2 patients (1.3%) with P D had a history of long-standing ET before the development of parkinsonian features, 6 (including these two) (3.8%) had several relatives affected by tremor in a fashion suggestive of autosomal dominant transmission. Four (4.4%) of the dystonic patients but none of the controls had similar family histories.We did not find the striking association between ET and PD reported by Geraghty and colle...