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1.  Trends in American Board of Psychiatry and Neurology specialties and neurologic subspecialties 
Neurology  2010;75(12):1110-1117.
Objective: To review the current status and recent trends in the American Board of Psychiatry and Neurology (ABPN) specialties and neurologic subspecialties and discuss the implications of those trends for subspecialty viability.
Methods: Data on numbers of residency and fellowship programs and graduates and ABPN certification candidates and diplomates were drawn from several sources, including ABPN records, Web sites of the Accreditation Council for Graduate Medical Education and the American Medical Association, and the annual medical education issues of the Journal of the American Medical Association.
Results: About four-fifths of neurology graduates pursue fellowship training. While most recent neurology and child neurology graduates attempt to become certified by the ABPN, many clinical neurophysiologists elect not to do so. There appears to have been little interest in establishing fellowships in neurodevelopmental disabilities. The pass rate for fellowship graduates is equivalent to that for the “grandfathers” in clinical neurophysiology. Lower percentages of clinical neurophysiologists than specialists participate in maintenance of certification, and maintenance of certification pass rates are high.
Conclusion: The initial enthusiastic interest in training and certification in some of the ABPN neurologic subspecialties appears to have slowed, and the long-term viability of those subspecialties will depend upon the answers to a number of complicated social, economic, and political questions in the new health care era.
doi:10.1212/WNL.0b013e3181f39a41
PMCID: PMC3463033  PMID: 20855855
ABMS = American Board of Medical Specialties; ABPN = American Board of Psychiatry and Neurology; ACGME = Accreditation Council for Graduate Medical Education; MOC = maintenance of certification; RRC-N = Residency Review Committee in Neurology.
2.  Safety and tolerability of putaminal AADC gene therapy for Parkinson disease 
Neurology  2009;73(20):1662-1669.
Background:
In Parkinson disease (PD), the benefit of levodopa therapy becomes less marked over time, perhaps because degeneration of nigrostrial neurons causes progressive loss of aromatic l-amino acid decarboxylase (AADC), the enzyme that converts levodopa into dopamine. In a primate model of PD, intrastriatal infusion of an adeno-associated viral type 2 vector containing the human AADC gene (AAV-hAADC) results in robust response to low-dose levodopa without the side effects associated with higher doses. These data prompted a clinical trial.
Methods:
Patients with moderately advanced PD received bilateral intraputaminal infusion of AAV-hAADC vector. Low-dose and high-dose cohorts (5 patients in each) were studied using standardized clinical rating scales at baseline and 6 months. PET scans using the AADC tracer [18F]fluoro-l-m-tyrosine (FMT) were performed as a measure of gene expression.
Results:
The gene therapy was well tolerated, but 1 symptomatic and 2 asymptomatic intracranial hemorrhages followed the operative procedure. Total and motor rating scales improved in both cohorts. Motor diaries also showed increased on-time and reduced off-time without increased “on” time dyskinesia. At 6 months, FMT PET showed a 30% increase of putaminal uptake in the low-dose cohort and a 75% increase in the high-dose cohort.
Conclusion:
This study provides class IV evidence that bilateral intrastriatal infusion of adeno-associated viral type 2 vector containing the human AADC gene improves mean scores on the Unified Parkinson’s Disease Rating Scale by approximately 30% in the on and off states, but the surgical procedure may be associated with an increased risk of intracranial hemorrhage and self-limited headache.
GLOSSARY
= l-amino acid decarboxylase;
= adeno-associated viral type 2 vector containing the human AADC gene;
= dopamine;
= [18F]fluoro-l-m-tyrosine;
= 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine;
= medium spiny neurons;
= not significant;
= Parkinson disease;
= Unified Parkinson’s Disease Rating Scale.
doi:10.1212/WNL.0b013e3181c29356
PMCID: PMC2839805  PMID: 19828868
3.  Uptake of dopamine and 5-hydroxytryptamine by platelets of patients with Parkinsonism. 
The uptake of tritiated dopamine and 5-hydroxytryptamine by platelets from 11 patients with Parkinsonism who were not receiving any medication, and from 11 control subjects matched for sex and for age within a decade was compared. No significant differences were found in the uptake of either amine. Our findings, therefore, provide no support for the belief that there is a generalised defect of dopamine systems in Parkinson's disease.
PMCID: PMC493100  PMID: 690635
4.  Clinical aspects of spasmodic dysphonia. 
The clinical features of 12 patients with spasmodic dysphonia are described. In 11 patients, the voice was strained, harsh, tight, and tremulous, and was low in volume and pitch. Speech, which was sometimes barely intelligible, was interrupted by irregular stoppages and catches of the voice; it required considerable effort, and was accompanied by facial grimacing. The dysphonia was part of a more widespread neurological disorder (idiopathic torsion dystonia) in one case, while it coexisted with blepharospasm in another, and with postural tremor in two. There was a buccolingual hyskinesia in another of these 11 patients, but this may have been related to her previous drug regime. In the twelfth patient, who had a familial tremor, the voice was characterised by marked breathiness, with intermittent aphonia. The disorder is probably due to a focal dystonia of the laryngeal musculature, and this would be consistent with the type of neurological disorders that were associated with it in our cases. Symptomatic benefit follows the therapeutic division of one of the recurrent laryngeal nerves, in selected cases.
PMCID: PMC493030  PMID: 650244
5.  Arterial blood pressure in patients with Parkinson's disease 
The casual arterial blood pressure of 411 patients with Parkinson's disease was compared with that of a representative sample of the general population. The patients were also divided into various sub-groups, and comparisons of blood pressure were made between them. The results provided no support for the widely held belief that blood pressure tends to be low in patients with Parkinson's disease.
PMCID: PMC491862  PMID: 1117302
6.  Results of surgical treatment for patients with a spinal angioma 
The clinical, angiographic, and operative findings in 16 patients with a spinal angioma are reviewed, and the results of surgery are assessed. The surgical procedure employed in 15 patients consisted of excision of the fistulous portion of the malformation, achieved by intradural ligation of its feeding vessels and excision of its draining veins throughout the limits of the exposure. In 14 patients, a previously progressive deterioration in their clinical condition was arrested by surgical correction of the malformation and in 12 of these patients there was worthwhile improvement in functional capacity. The optimal time for surgical treatment is considered and suggestions are made for the manner in which patients should be investigated.
Images
PMCID: PMC494840
7.  Basis of nocturnal polyuria in patients with autonomic failure 
Five dysautonomic patients with the Shy-Drager syndrome were studied to determine the basis of their nocturnal polyuria. The results indicated excessive postural modification of renal function in dysautonomic patients. This may, in fact, relate to excessive release of ADH while these patients are up and about, and excessive inhibition while they are recumbent. Treatment with vasopressin produced an inconsistent response.
PMCID: PMC494741  PMID: 4844135
8.  Levodopa therapy for Parkinsonism in the Shy-Drager syndrome 
The results of levodopa treatment in five patients with Parkinsonism of the Shy-Drager syndrome are reported. There was no improvement in the neurological signs or functional disability in any patient. Mean values for lying and standing blood pressure increased significantly in three of the five patients during treatment, and were unchanged in the other two patients. The significance of these results is discussed. It is concluded that levodopa is not of therapeutic value in patients with this disorder.
PMCID: PMC494332  PMID: 4714096
9.  Dystrophia myotonica presenting with dysphagia. 
British Medical Journal  1971;2(5759):443.
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PMCID: PMC1796183  PMID: 5576006
10.  Assessment of Autonomic Function in Patients with a Parkinsonian Syndrome 
British Medical Journal  1971;4(5779):80-84.
The integrity of the autonomic nervous system was assessed in 11 Parkinsonian patients with symptoms suggestive of autonomic dysfunction. Three had the additional clinical features of the Shy-Drager variant of idiopathic orthostatic hypotension and were found to have a gross disturbance of vasomotor, sudomotor, pilomotor, and bladder function; assessment indicated that a lesion was present at sympathetic ganglionic level or beyond in two cases, though a more centrally placed lesion may well have been present also, as in the third case. In the remaining eight patients with paralysis agitans no unequivocal functional disturbance was found except in the bladder; nevertheless, the low resting blood pressure and the supersensitivity to intravenously infused L-noradrenaline in the three patients in whom it was tested is taken to imply defective regulation from higher centres, with a consequent reduction in impulse traffic at sympathetic nerve terminals. Such a concept is supported by experimental studies in animals and would account for the low renin and aldosterone secretion rates and reduced noradrenaline formation reported by others in patients with paralysis agitans.
PMCID: PMC1799334  PMID: 5097425
12.  Involvement of peripheral vasomotor fibres in carpal tunnel syndrome. 
It is often assumed that large diameter nerve fibres are affected selectively in patients with compressive peripheral nerve lesions. However, in five of 13 patients with the carpal tunnel syndrome, plethysmography revealed that the normal digital vasoconstrictor response to inspiration was abolished in the index finger but preserved in the little finger of the affected hand. This indicates that the function of sympathetic efferent fibres destined for a median-innervated digit is sometimes disturbed also in patients with median nerve entrapment at the wrist.
PMCID: PMC490280  PMID: 479905
13.  Bell's palsy and its treatment. 
Postgraduate Medical Journal  1973;49(567):46-51.
PMCID: PMC2495368  PMID: 4354407
14.  Autonomic dysfunction in syringomyelia. 
Postgraduate Medical Journal  1972;48(556):113-115.
PMCID: PMC2495158  PMID: 5015116
16.  Parkinson's disease--a progress report. 
Western Journal of Medicine  1996;165(4):248-250.
PMCID: PMC1303763  PMID: 8987441
17.  Treatment of Parkinson's disease. 
Western Journal of Medicine  1994;161(3):303-308.
Pharmacotherapy with levodopa for Parkinson's disease provides symptomatic benefit, but fluctuations in (or loss of) response may eventually occur. Dopamine agonists are also helpful and, when taken with low doses of levodopa, often provide sustained benefit with fewer side effects; novel agonists and new methods for their administration are therefore under study. Other therapeutic strategies are being explored, including the use of type B monoamine oxidase inhibitors to reduce the metabolic breakdown of dopamine, catechol-O-methyltransferase inhibitors to retard the breakdown of levodopa, norepinephrine precursors to compensate for deficiency of this neurotransmitter, glutamate antagonists to counteract the effects of the subthalamic nucleus, and various neurotrophic factors to influence dopaminergic nigrostriatal cells. Surgical procedures involving pallidotomy are sometimes helpful. Those involving cerebral transplantation of adrenal medullary or fetal mesencephalic tissue have yielded mixed results; benefits may relate to the presence of growth factors in the transplanted tissue. The transplantation of genetically engineered cell lines will probably become the optimal transplantation procedure. The cause of Parkinson's disease may relate to oxidant stress and the generation of free radicals. It is not clear whether treatment with selegiline hydrochloride (a type B monoamine oxidase inhibitor) delays the progression of Parkinson's disease, because the drug also exerts a mild symptomatic effect. Daily treatment with vitamin E (a scavenger of free radicals) does not influence disease progression, perhaps because of limited penetration into the brain.
Images
PMCID: PMC1011414  PMID: 7975571
18.  The effect on plasma prolactin levels of interictal epileptiform EEG activity. 
Because of the known effects of seizures on plasma prolactin, the plasma prolactin levels were measured before and after generalised interictal epileptiform activity was provoked in the EEG in five epileptic patients. The findings were compared with those obtained in five normal subjects and three epileptic patients who were also exposed to flicker stimulation, but who did not develop a photoconvulsive EEG response. There was no significant difference in baseline prolactin values, and levels did not change with photic stimulation or in response to the presence of generalised epileptiform activity in the EEG.
PMCID: PMC1028855  PMID: 3090207
19.  Periodic EEG complexes in infectious mononucleosis encephalitis. 
The presence of periodic EEG complexes in patients with an acute viral encephalitis is generally held to suggest that infection is due to herpes simplex. We now report a patient with clinical and laboratory findings of infectious mononucleosis, and neurologic involvement manifested by lymphocyte meningitis, coma, seizures, aphasia, hemiparesis and hemianopsia. Serial EEGs showed periodic, predominantly left-sided slow wave complexes occurring every 4 to 5 seconds, which disappeared with clinical resolution of the illness. In view of our findings and the similar findings reported previously by others in another case of infectious mononucleosis encephalitis, an EEG showing periodic complexes in the clinical setting of acute viral encephalitis should not be considered pathognomonic of herpes encephalitis, and infectious mononucleosis should be included in the differential diagnosis.
PMCID: PMC491482  PMID: 7119833
20.  Pattern-onset visual evoked potentials in suspected multiple sclerosis. 
Visual evoked potentials (VEPs) were obtained by monocular stimulation using a checkerboard pattern-reversal and pattern-onset technique. In 11 normal subjects, pattern-onset VEPs were generally larger, better defined, and less ambiguous than those elicited by pattern-reversal, because of the biphasic waveform characteristically obtained with pattern-onset stimulation. In 68 of 105 patients with possible multiple sclerosis, VEPs were normal in latency by both methods, and in nine adequate comparison was not possible. The incidence of normal VEPs to pattern-reversal was similar to that found in several other studies of patients with possible multiple sclerosis. Among the remaining 28 patients in whom VEP abnormalities were found, an increased latency was detected in 75% with the pattern-reversal technique, and in 96% by pattern-onset. In these patients, VEP abnormalities were obtained by monocular stimulation of each of 46 eyes, and among these the pattern-reversal technique yielded abnormalities in 59% and the pattern-onset method in 98%. These results indicate that VEPs elicited by pattern-onset are useful in investigating patients with suspected multiple sclerosis, and the diagnostic yield may be greater than with conventional pattern-reversal techniques.
PMCID: PMC491065  PMID: 7288448

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