Occurrence of depressive symptoms in Shy-Drager syndrome.

We investigated the frequency and severity of depressive symptoms among patients with Shy-Drager Syndrome (SDS) and correlated depression with the extent of the patients' disability. Data were collected from 15 patients and their spouse caregivers through a mailed questionnaire. The patients were asked to complete the Beck Depression Inventory (BDI) questionnaire, while caregivers were asked to complete the self-assessment Parkinson's Disease Disability Scale and The Northwestern University Disability Scale for Parkinson's Disease. Data were statistically analyzed using descriptive statistics and Pearson-Product moment correlations. The prevalence of depressive symptoms was 85.7%; 28.6% of SDS patients scored in the moderately to severely depressed range. There was no significant correlation between the severity of depressive symptoms and disability (r = 0.02, p = 0.94) and the ability to perform activities of daily living (r = 0.0, p = 1.0). The prevalence of depressive symptoms in patients with SDS is common. The patient's level of depression does not correlate with physical disability. Pharmacologic management and interventions aimed at increasing active coping methods should improve quality of life.

Autonomic dysfunction in chronic intestinal pseudo-obstruction.

Fifteen tests were used to assess adrenergic, non-vagal cholinergic, and cardiovagal functions in 11 patients with chronic intestinal pseudo-obstruction (CIP). The three aims of this study were: 1) to ascertain the presence of and spectrum of autonomic involvement; 2) to assess the level of autonomic dysfunction; and 3) to compare the results of autonomic function tests with gastrointestinal motility patterns. Gastrointestinal motility displayed a neuropathic pattern in 10 patients. Adrenergic functions were abnormal in nine patients and non-vagal cholinergic functions in 10 patients. Cardiovagal functions were abnormal in only seven patients. The autonomic dysfunction was localized mostly to the postganglionic pathways. One patient, who had a myopathic pattern and muscle degeneration on small bowel biopsy, demonstrated normal responses to autonomic function tests. The patients with neuropathic CIP demonstrated widespread, mostly postganglionic autonomic dysfunction. Neuropathic CIP can occur with or without cardiovagal involvement.

Painful trigeminal neuropathy: clinical and pharmacological observations.

UNLABELLED: A 74-year-old woman had a 5-year history of constant burning pain and numbness of the central face of subacute onset. The central region of the face, oral cavity, and nose lacked all sensation. Corneal reflexes and the jaw jerk were absent. Blood tests, rectal biopsy, neurodiagnostic studies, and surgical exploration of the trigeminal nerve were normal. Blink reflexes were absent. Facial nerve motor latencies and EMG of the facial and masseter muscles were normal. Responses to the thermoregulatory sweat test, intradermal histamine, and simulated diving were present. Oral administration of 500 mg L-dopa aggravated her pain and produced transient hypalgesia in the C2 through C6 dermatomes. Infraorbital nerve biopsy demonstrated loss of large myelinated fibers. IN CONCLUSION: (1) Only the central region of the face is exclusively supplied by the trigeminal nerves. (2) Somato-autonomic reflexes coupled with electrophysiological studies localized the lesion to the large fibers. (3) Large fiber loss and central brain stem reorganization may explain the burning pain. (4) Dopamine may modulate trigeminal nociception.

A novel psychophysiological treatment for vasovagal syncope.

The objective of this study was to evaluate the efficacy of transactional psychophysiological therapy (TPT) in a patient with recurrent vasovagal syncope (VVS) and to quantify the capacity of human dialogue to effect significant and consistent measurable therapeutic cardiovascular (CV) changes. A 31-year-old nurse with recurrent VVS and a reproducibly abnormal tilt-table test was refractory to pharmacological and conventional psychiatric treatments. She was treated with TPT. Her CV responses during psychotherapy were incorporated into the dialogue as an important source of communicative information, and she was taught psychophysiological techniques to correct exaggerated CV responses. These responses, during 16 weekly and 12 subsequent monthly sessions, were analysed using a one-way multiple analysis of variance. As TPT progressed, the magnitude and lability of CV responses as well as frequency of VVS were reduced. She has been relatively asymptomatic for 14 years posttherapy. In conclusion, (1) TPT may be an effective primary/adjunctive treatment for patients with VVS; (2) TPT may reduce syncopal episodes, perhaps by normalizing limbic input to the brainstem baroreflex system.

Head-up tilt table test: how far and how long?

There is great variation between laboratories in the angle and duration of tilt used in the head-up tilt table test. We compared the hemodynamic response to three sequential randomized 20-min tilts of 60 degrees, 80 degrees and 90 degrees in 20 normal subjects. We studied 66 patients to determine the duration of tilt needed to document three types of orthostatic intolerance. Blood pressure and heart rate were monitored non-invasively. Thirty-nine patients with orthostatic hypotension of diverse causes and 17 patients with orthostatic tachycardia plus were tilted to 90 degrees for 10 min. Ten patients with vasovagal syncope were tilted to 90 degrees for 30 min. A multivariate analysis of variance performed to assess the hemodynamic responses to 60 degrees, 80 degrees and 90 degrees tilts did not show a statistically significant difference (F = 0.459). Logistic regression analyses revealed that the duration of tilt required to document orthostatic hypotension was 5 min (73% cases correctly identified), orthostatic tachycardia plus 10 min (86.5% cases correctly identified) and neurally mediated syncope 10 min (86.5% cases correctly identified). Tilt duration is a more important variable than tilt angle and the duration of the tilt should depend on the suspected cause of orthostatic intolerance.

The value of the isometric hand-grip test--studies in various autonomic disorders.

Time-related methodological differences have confounded the value of isometric hand-grip (IHG) exercise as a test of sympathetic function. This study examines the blood pressure response to IHG in 71 normal subjects and 76 dysautonomic patients and compares it with the tilt table test. All participants were supine and performed IHG at 30% of maximum contraction for 5 min. Change in diastolic blood pressure (DBP) was the most sensitive and specific measurement in diagnosing abnormals. The multivariate group by time (1-5 min) effect was significant (F = 10.14, p < 0.001) with 5 min as the most important time measurement (Wilk's lambda = 0.70, p < 0.001). The DPB rise for normals was 25.15 +/- 1.31 mmHg (mean +/- SE), range 22.55-27.76 mmHg, with a sensitivity of 78.95% and a specificity of 71.83%. A multivariate analysis of variance showed intraindividual reproducibility of nine normals over four trials (F = 0.950, p > 0.25). Gender and grip strength were additional important variables. In comparison, the tilt table test demonstrated a sensitivity of 82.76% and a specificity of 73.68%. Isometric hand-grip is therefore a specific, sensitive, reproducible, simple and non-invasive test of sympathetic function with relatively well-studied reflex pathways. Its sensitivity and specificity compare favourably with the tilt table test. It should be incorporated in routine autonomic testing.

Intracranial hypotension.

Intracranial hypotension (IH) is present when cerebrospinal fluid (CSF) pressure is 60mm H2O or lower and there has been no previous dural puncture. IH is more common in women than in men (3:1). Orthostatic headache is the cardinal symptom. Visual, auditory, and other symptoms occur. Postulated mechanisms include sagging of the brain, dilation of intracranial veins, and activation of adenosine receptors. Examination may disclose visual field defects. The condition may be primary (probably related to an occult dural leak) or secondary to many causes that include lumbar puncture, trauma, pneumonectomy, diabetic coma, and uremia. Patients with postural headache should undergo neuroimaging prior to lumbar puncture. Radionuclide cisternography is the most sensitive means of demonstrating a CSF fistula. Severe, intractable headache associated with IH may respond to intravenous of oral caffeine. An epidural blood patch and epidural infusion of normal saline are treatment measures for symptoms of IH that follow lumbar puncture.

Oculocephalic sympathetic dysfunction in posttraumatic headaches.

Oculocephalic sympathetic functions were assessed in five patients with posttraumatic headaches using the thermoregulatory sweat test and biochemical pupillary responses. Four patients demonstrated bilateral sympathetic dysfunction following whiplash injury, and one patient demonstrated unilateral sympathetic dysfunction following forehead injury. Biochemical pupillary responses were diagnostic in the early posttraumatic period, while the thermoregulatory sweat test was abnormal up to 56 months following the injury. This study documents serious injury to the cervical sympathetic nerves in patients with posttraumatic headaches following whiplash injury, and shows the reliability of the thermoregulatory sweat test in identifying patients with long-term oculocephalic sympathetic dysfunction. It also shows dissociated postganglionic cranial sympathetic dysfunction. Our experience and a review of the pertinent literature shows no convincing clinical or experimental evidence to establish oculocephalic sympathetic dysfunction as a direct cause of head pain, but it may exert an effect on cephalic pain through the trigeminovascular system.

Orthostatic intolerance and orthostatic tachycardia: a heterogeneous disorder.

A series of autonomic function tests were performed in eight patients who had orthostatic intolerance and orthostatic tachycardia (> 36 bpm). All eight had an abnormal tilt-table test (mean tachycardia 50 bpm compared with 22 +/- 7 bpm in 31 normal subjects). Plasma volume was low in four out of six patients. There was a subnormal response to the cold pressor test in three out of eight patients. Intravenous atropine-induced tachycardia was subnormal in two out of eight patients. A segmental or patchy anhidrosis was present in six out of eight patients. Pilocarpine (0.0625%) induced miosis (three out of six) and exaggerated Valsalva ratio (one out of eight) were observed. A follow-up of six patients, 8-17 years after the autonomic evaluation, showed spontaneous and complete improvement in two out of six, partial improvement in one out of six, and persistence of symptoms in three out of six patients. A repeat autonomic evaluation in two patients with persistent symptoms, 2 and 5 years after the initial assessment, showed mild progression of sympathetic and parasympathetic dysfunction. This syndrome appears characterized by orthostatic tachycardia, low plasma volume, lack of adrenergic supersensitivity, and sudomotor abnormalities. A longitudinal follow up demonstrates patients with and without recovery. The syndrome appears heterogeneous in terms of onset, clinical manifestations, laboratory studies and outcome.

Cholinergic dysfunction in Shy-Drager syndrome: effect of the parasympathomimetic agent, bethanechol.

To determine the frequency, severity and organ distribution of cholinergic dysfunction in the Shy-Drager syndrome, eleven patients were prospectively studied. In addition to documenting adrenergic insufficiency, a battery of twelve tests was employed to assess cholinergic function. Six tests demonstrated pupillary, lacrimal, salivary, urinary bladder, sexual and sudomotor dysfunction in the majority of patients. Cardiac vagal function as studied by the heart rate response to deep breathing, the Valsalva manoeuvre, cold face test, apnoeic facial immersion and atropine test was affected in all patients. Oesophageal motility was abnormal in six patients. Cholinergic dysfunction in patients with the Shy-Drager syndrome was widespread but of variable severity and distribution. Subcutaneous administration of the parasympathomimetic agent bethanechol demonstrated hyperresponsiveness of lacrimal, salivary, oesophageal, bowel, bladder and sudomotor functions. It is suggested that the Shy-Drager syndrome is primarily a preganglionic cholinergic disorder with transsynaptic degeneration accounting for the development of postganglionic cholinergic as well as adrenergic dysfunction.

Bilateral Horner's syndrome in cluster type headaches.

A patient with cluster type headaches demonstrated bilateral and alternating ocular sympathetic dysfunction during a spontaneous as well as a nitroglycerin-induced attack. Biochemical evaluation revealed postganglionic pupillary dysfunction on the symptomatic side and preganglionic pupillary dysfunction contralaterally. These findings defy a simple explanation regarding a central or peripheral origin of the oculocephalic sympathetic dysfunction.

Pathophysiological studies of neuromuscular function in subacute organophosphate poisoning induced by phosmet.

A 51 year old man developed progressive cranial and proximal muscle weakness, hyperreflexia and mental change. The disorder progressed over 9 days following the fifth weekly spraying with the organophosphate (OP) insecticide, phosmet, with limited symptoms of acute toxicity. Marked decremental responses of 50-80% on slow and fast rates of stimulation were improved to 15% by edrophonium or neostigmine. Intracellular recordings at the endplate region of intercostal muscle revealed small miniature endplate potentials (mepps), reduced mean acetylcholine sensitivity and normal membrane potentials. Electronmicroscopy revealed degeneration and regeneration of the endplates. This study demonstrates that OP poisoning due to phosmet can produce a subacute postsynaptic neuromuscular syndrome without marked symptoms of acute toxicity.

Sensory innervation of the canine esophagus, stomach, and duodenum.

The sensory innervation of the postpharyngeal foregut was investigated by injecting the enzyme horseradish peroxidase (HRP) into the walls of the esophagus, stomach, or duodenum. The transported HRP was identified histochemically, labeled neurons in the spinal and vagal ganglia were counted, and the results were plotted using an SAS statistical program. The spinal sensory fields of each viscus were defined using three determinations: craniocaudal extent, principal innervation field, and peak innervation field. The data revealed that innervation fields are craniocaudally extensive, the sensory field of each viscus overlaps significantly with its neighbor, yet each viscus can be characterized by a field of peak innervation density. Craniocaudal innervation of the esophagus spans as many as 22-23 paired spinal ganglia (C1-L2). There are two peak innervation fields for the cervical (C2-C6 and T2-T4) and for the thoracic (T2-T4 and T8-T12) sectors of the esophagus. The sensory innervation of the stomach extends craniocaudally over as many as 25 paired spinal ganglia (C2-L5). The peak innervation field of the stomach spans a large area comprising the cranial, middle, and the immediately adjoining caudal thoracic ganglia (T2-T10). The duodenum is innervated craniocaudally by as many as 15 paired thoracolumbar ganglia (T2-L3). Peak innervation originates in the middle and caudal thoracic ganglia and cranial lumbar (T6-L1) ganglia. There is a recognizable viscerotopic organization in the sensory innervation of the postpharyngeal foregut; successively more caudal sectors of this region of the alimentary canal are supplied with sensory fibers from successively more caudal spinal dorsal root ganglia. Vagal afferent innervation of the esophagus, stomach, and duodenum is bilateral and originates predominantly, but not exclusively, from vast numbers of neurons in the nodose (distal) ganglia. The esophagus is innervated bilaterally and more abundantly by jugular (proximal) ganglia neurons than is either the stomach or duodenum. The physiological significance of the findings are discussed in relation to the phenomena of visceral pain and referred pain.

Headache spectrum in Arnold-Chiari malformation.

Exertional headaches in patients with Arnold-Chiari malformation (ACM) are well described. We report four patients with Type I ACM and recurrent headaches. These patients presented, respectively, with low spinal fluid pressure headache, migraine without aura, migraine with aura, and migraine with prolonged aura. This report suggests the need for observing patients with recurrent headaches for any physical stigmata of craniovertebral junction anomalies, and the need to exclude ACM in such patients. Possible implications of the association between ACM and different types of headaches are discussed.

Posttraumatic headache with ptosis, miosis and chronic forehead hyperhidrosis.

Injury to the right lateral forehead was followed by headaches, and chronic ipsilateral ptosis, miosis, and forehead hyperhidrosis. Episodes of headache were accompanied by an increase in ptosis, miosis and forehead hyperhidrosis. The headaches abated within 6 weeks but the ptosis and miosis, due to postganglionic sympathetic insufficiency, were persistent. Spontaneous forehead hyperhidrosis, was also persistent at the time of last follow-up, 15 months later. Autonomic assessment of the oculocephalic sympathetic dysfunction, localization of the lesion and possible explanation of the autonomic findings are discussed.

Autonomic dysfunction in Lambert-Eaton myasthenic syndrome.

Autonomic symptoms were observed in 6 patients with clinically and electrophysiologically documented Lambert-Eaton myasthenic syndrome (LEMS). Of the 6 patients, 2 were extensively investigated in the laboratory. In contrast to previous reports which recognized only cholinergic dysautonomia, abnormalities of sympathetic as well as parasympathetic function were evident. Of the 6 patients, 4 had small cell lung cancer (SCLC). In one male patient, chemotherapy for SCLC resulted in an early improvement of autonomic dysfunction and the electrophysiological defect, documenting simultaneous regression of dysautonomia and LEMS. In addition, the patients with SCLC and LEMS had a survival thus far of 3-13 years suggesting that a subgroup of SCLC patients have a better prognosis.

Vaccine-associated poliomyelitis in a household contact with Netherton's syndrome receiving long-term steroid therapy.

Paralytic poliomyelitis developed in a man 51 days after his two-month-old daughter received her first dose of trivalent live oral poliovirus vaccine. The patient was receiving long-term glucocorticosteroid therapy (tapered to 12.5 mg per day for the eight months prior to his poliomyelitis) for Netherton's syndrome, a congenital syndrome characterized by bamboo-like hair, hyperkeratotic and hyperhidrotic skin, and multiple allergies. The patient was ventilator-dependent and quadriplegic throughout most of his hospital stay and died in the hospital 10 months after the onset of paralysis. "Vaccine-like" type 3 poliovirus was isolated from a stool specimen and his serum showed a significant rise in neutralizing antibody titer against type 3 virus. This case report represents the first documented case of vaccine-associated poliomyelitis in a household contact receiving glucocorticosteroids, although evidence of immunosuppression was not documented. Nevertheless, the case reinforces current recommendations not to administer oral poliovirus vaccine to persons known to be immune deficient or suppressed or to normal persons with close contacts known to be immune deficient or suppressed.

Orthostatic hypotension-induced autonomic dysreflexia.

Three patients with chronic traumatic cervical myelopathy had severe orthostatic hyperhidrosis. Orthostatic challenge revealed that hypotension preceded hyperhidrosis, hypertension, and chills, all manifestations of autonomic dysreflexia. Treatment of orthostatic hypotension with fludrocortisone acetate relieved these symptoms. Therefore, orthostatic hypotension may trigger autonomic dysreflexia and the usual way of managing such patients, propping them upright, may be counterproductive.