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PubMed1993

[Should Tolosa-Hunt syndrome still be an exclusion diagnosis?].

Since Hunt et al's description (1961) Tolosa-Hunt syndrome has been a matter of controversies about its nosological identity and differential diagnosis. We report 7 cases diagnosed between 1979 and 1990. Four of them had a low-resolution CT and the diagnosis was made after a long follow-up, according to classical criteria of exclusion. The last 3 cases had a modern high-resolution CT or a MRI (2 cases) and the diagnosis was established in a few days. MRI has considerably simplified the differential diagnosis but it has not completely resolved it. Alterations of shape and signal of the cavernous sinus are similar to those of lymphoma, sarcoidosis and meningioma. When MRI is normal diabetic ophthalmoplegia, migrainous ophthalmoplegia and giant-cell arteritis must also be considered. We conclude that the diagnosis of the Tolosa-Hunt syndrome can generally be made in a few days, without a cavernous sinus biopsy. There is no reason to doubt that this syndrome is a specific nosological entity.

Aged↗
PubMed1996

[Primary glaucoma in two German hunting terriers and a wire-haired fox terrier].

Primary glaucoma was diagnosed in a five- and a six-year-old female German Hunting Terrier and a four-year-old female Wirehaired Foxterrier. The five-year-old Hunting Terrier had an absolute glaucoma in the right eye. The left eye showed a dysplastic pectinate ligament (DLP) and a narrow iridocorneal angle. The right eye was enucleated and the fellow eye fell sick five months later. Inspite of cyclocryotherapy glaucoma cannot be controlled without medication until now. In the six-year-old Hunting Terrier the iridocorneal angle could not be evaluated, because the right eye had an acute glaucoma and the left eye a chronic glaucoma with seclusio pupillae. A cyclocryotherapy was done on both eyes. Now the left eye is blind and vision is reduced in the right eye. Intraocular pressure values are within the normal range. The four-year-old Foxterrier had a chronic glaucoma in the right eye, because of DLP and a narrow iridocorneal angle. In the left eye the angle was narrow too, but rudimentary white trabecula with flow holes existed. This eye did not fall sick until now. In the right eye lowering of pressure could be obtained by medication only.

Animals↗
PubMed2006

SPIR MRI usefulness for steroid treatment management in Tolosa-Hunt syndrome.

A 40-year-old man underwent surgery for a right middle ear cholesteatoma. One month later, he presented with a subacute ocular pain that was followed one day later by the appearance of vertical diplopia attributable to a right superior rectus paresis, lid ptosis and hypoaesthesia in the territory of the I and the II right trigeminal branches. A fat-suppressed (selective partial inversion recovery, SPIR) gadolinium-enhanced MRI favours the detection of inflammatory pathological tissue inside the right cavernous sinus, and in this patient it suggested a diagnosis of Tolosa-Hunt syndrome. The pain disappeared quickly after steroid treatment was started whereas the ocular nerve involvement improved only slightly during the first week of treatment. After two months, the patient only complained of diplopia on up-gaze, but the therapy was discontinued two months later on the basis of both clinical signs and MRI findings. SPIR MRI may be useful not only to support a diagnosis of Tolosa-Hunt syndrome, but also to follow-up the disease course and to manage steroid treatment.

Adrenal Cortex Hormones↗
PubMed2004

Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome.

Painful ophthalmoplegia is an important presenting complaint to emergency departments, ophthalmologists, and neurologists. The etiological differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies including vascular (eg, aneurysm, carotid dissection, carotid-cavernous fistula), neoplasms (eg, primary intracranial tumors, local or distant metastases), inflammatory conditions (eg, orbital pseudotumor, sarcoidosis, Tolosa-Hunt syndrome), infectious etiologies (eg, fungal, mycobacterial), and other conditions (eg, microvascular infarcts secondary to diabetes, ophthalmoplegic migraine, giant cell arteritis). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that if left untreated, can be associated with significant morbidity or mortality. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids, but should be diagnoses of exclusion.

Adult↗
PubMed2024

A Donald F. Hunt Story (John's Version).

A personal narrative of my time in the Hunt laboratory and beyond is provided. The impact of the Hunt laboratory on the analysis of peptides and proteins by tandem mass spectrometry is described in the context of the time.

History, 20th Century↗
PubMed2004

Paediatric Tolosa-Hunt syndrome.

Tolosa-Hunt syndrome is characterized by a dull, persistent pain around the affected eye, ophthalmoplegia and, sometimes, involvement of other cranial nerves passing through the cavernous sinus. Corticosteroid administration is valuable in the treatment and frequently has a dramatic effect. We report a boy with Tolosa-Hunt syndrome who fails to respond to the initial steroid treatment. The role of the MRI in the management of this condition is discussed.

Child↗
PubMed2004

Reidel's thyroiditis and Tolosa-Hunt syndrome, a rare association.

A previously undescribed association of Reidel's thyroiditis with Tolosa-Hunt syndrome is reported. A 33 year-old Caucasian female presented with a two-year history of painful progressive goitre, histologically a Reidel's thyroiditis. She underwent left partial thyroidectomy. Post-operatively she developed severe right retro-orbital pain, right temporal headache and a partial right occulomotor nerve palsy. A diagnosis of Tolosa-Hunt syndrome was made. Some important aspects of this case are discussed.

Adult↗
PubMed2005

[Tolosa-Hunt syndrome: analysis of six cases].

Tolosa-Hunt syndrome is an entity of rare occurrence and unknown etiopathogenesis, expressed clinically by unilateral orbitary pain associated with simple or multiple oculomotor paralysis, which resolves spontaneously but may recur. We present a series of six cases taken care in our ambulatory, which fulfill the diagnostic criteria for Tolosa Hunt syndrome according to the International Headache Society Classification of 2004. There are also emphasized the demographic data of this series of patients, their 12 months follow-up, investigative studies and responsiveness to corticosteroids. As the diagnosis is always of exclusion, literature revision is focused on differential diagnosis of painful ophthalmoplegic syndromes.

Adult↗
PubMed2000

Tolosa Hunt Syndrome--intractable pain treatment with acupuncture?

PURPOSE: The Tolosa Hunt Syndrome (THS) is a painful granular inflammation of the cerebral vessels followed by pain and disorders of the extrabulbar muscles. The therapy consists of corticosteroids and analgetics. There was a 70 year old woman who suffered from painful paresis of the abducent and oculomotor nerves following an infection with Borrelia Burgdorferi--but without ocular symptoms. The treatment with corticosteroids reduced the palsy but she complained of excessively painful attacks in the region of the first branch of the trigeminal nerve. Opiold analgetic therapy did not bring about any relief. Acupuncture is an irritative method with a physical effect on the nervous system: its pain-reducing effect is caused by the activation of transmitters like endorphins in thalamus and brain stem. Knowing this effect, the THS patient, after informed consent, was treated with acupuncture. To measure the extent of pain, a visual analog scale (0: no pain - 10: maximum pain) was used. Acupuncture was performed according to the empirical rules of the Traditional Chinese Medicine (TCM), during a period of 10 weeks and 12 weeks. There was a significant pain relief after acupuncture from VAS 10 to VAS 5. The effect vanished during the next four months. After a second series of 12 sessions pain reduction was reported from VAS 10 to 4. One year after the last Tolosa Hunt Syndrome - intractable pain pain strength ranged between VAS 4 - 6. Therefore acupuncture seems to be a good additional method for reduction of intractable pain.

Acupuncture Therapy↗
PubMed1989

Roger Hunt negotiates a new academic affiliation. Interview by Donald E.L. Johnson.

Roger Hunt is president and chief executive officer of Lutheran General Hospital, Park Ridge, Illinois. The north suburban Chicago hospital recently signed a letter of intent to shift its primary academic affiliation to the University of Chicago Medical School, which is on the south side of the city. More than 20 miles and more than an hour of freeway and city street driving separate the organizations. Health Care Strategic Management's editor and publisher, Donald E.L. Johnson, talked with Roger Hunt about Lutheran General's strategy for selecting a medical school and creating a teaching program that works for all concerned.

Academic Medical Centers↗
PubMed2001

Mycotic aortic aneurysm in a patient with Tolosa-Hunt syndrome.

Mycotic aneurysms have been associated with many clinical conditions. A tender pulsatile abdominal mass in association with fever, chills, and unrelenting back pain is suggestive of a leaking mycotic aneurysm. However, the extracranial manifestations of Tolosa-Hunt syndrome (THS) may mimic several of these symptoms. We report the case of a woman who was successfully treated with high-dose steroids for THS. Two months later, she was admitted to another hospital with rigors and unremitting back and abdominal pain. CT-guided aspiration of an L5-S1 paravertebral mass was done. The aspirate and blood cultures grew Staphylococcus aureus. Intravenous antibiotics and analgesics were administered with good relief. A month after discharge from that hospital, she was admitted to our hospital with classic signs and symptoms of a leaking mycotic aneurysm. She was treated surgically and has remained asymptomatic for 21 months. Tolosa-Hunt syndrome associated with mycotic aortic aneurysms has not been previously reported.

Abdominal Pain↗
PubMed1990

Progressive myoclonic ataxia (the Ramsay Hunt syndrome).

It has been suggested from studies of patients with progressive myoclonus epilepsy that the term Ramsay Hunt syndrome should be abandoned, as its use has led to nosologic confusion, and because, in the light of modern diagnostic techniques, the majority of cases can be allocated to specific disease categories, chiefly, Unverricht-Lundborg disease (Baltic myoclonus) and mitochondrial encephalomyopathy. Review of 30 cases of this syndrome, defined as progressive ataxia and myoclonus and infrequent seizures in the absence of dementia, showed that a clinical or biochemically supported diagnosis could not be made in 43%. This low diagnostic yield probably reflects differences in ascertainment of patients; those described here were referred with a syndrome of progressive myoclonic ataxia (the Ramsay Hunt syndrome) rather than progressive myoclonus epilepsy. These two syndromes share common causes, but a smaller proportion of patients with progressive myoclonic ataxia can currently be diagnosed precisely during life.

Adolescent↗
PubMed1986

Electrocochleography and brain-stem potentials in Ramsay Hunt syndrome.

Audiometric investigations and electrophysiologic recordings of cochlear and brain-stem auditory evoked potentials (BAEPs) were performed in 13 patients to elucidate further the type of hearing disorders in Ramsay Hunt syndrome. Transtympanic electrocochleography showed no enhancement of summating potential and did not suggest secondary endolymphatic hydrops. The recording of BAEPs was clearly abnormal in several of the 13 patients. The striking feature of the abnormalities in these patients was the prolongation of the latencies of waves III and V with the preservation of wave I, which clearly suggests retrocochlear involvement. In all the patients tested, abnormalities of the BAEPs were present only on the affected side. It is possible, on the basis of BAEP findings, to suggest that in Ramsay Hunt syndrome both cochlear and retrocochlear involvement may occur.

Adult↗
PubMed1997

Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment.

Although the antiviral agent acyclovir is currently used for the treatment of Ramsay Hunt syndrome, its effects on facial nerve and hearing recovery remain controversial. We retrospectively analyzed the effects of acyclovir-prednisone treatment in 80 Ramsay Hunt patients. Of 28 patients for whom treatment was begun within 3 days of the onset of facial paralysis, the recovery from paralysis was complete in 21 (75%). By comparison, of 23 patients for whom treatment was begun more than 7 days after onset, recovery from facial paralysis was complete in only 7 (30%). A significant difference in facial nerve recovery was found between these groups. Early administration of acyclovir-prednisone was proved to reduce nerve degeneration by nerve excitability testing. Hearing recovery also tended to be better in patients with early treatment. There was no significant difference in facial nerve outcome between intravenous and oral acyclovir treatment.

Acyclovir↗
PubMed1986

Ramsay Hunt syndrome and coeliac disease: a new association?

Two patients with the syndrome of Ramsay Hunt (dyssynergia cerebellaris myoclonica, DCM), associated with malabsorption due to adult coeliac disease, are reported. Both presented with progressive cerebellar ataxia, action myoclonus, and epilepsy. One had gastrointestinal symptoms (recurrent diarrhea and weight loss which responded satisfactorily to a gluten-free diet), but the other did not. In both patients, jejunal biopsy revealed subtotal villous atrophy; serum folate and vitamin E level were also reduced. Neither a gluten-free diet nor vitamin supplements improved the neurological picture. However, some symptomatic relief was afforded by treatment with clonazepam, sodium valproate, carbamazepine, and piracetam. It could be argued that the association between these two disorders is coincidental. However, since we have found this combination in 2 of 14 consecutive cases with DCM, a causal relationship seems likely, although the underlying mechanism remains unknown. Patients with the Ramsay Hunt syndrome should be investigated for malabsorption, and also undergo small intestinal biopsy.

Adult↗
PubMed1995

The physical demands of riding in National Hunt races.

Heart rate (fc) and post-competition blood lactate concentration ([La+]) were studied in seven male professional National Hunt jockeys over 30 races. The fc response for individual races followed a similar pattern for all subjects. The mean peak fc recorded during competition was 184 beats.min-1 (range 162-198 beats.min-1) with average fc during the races ranging from 136 to 188 beats.min-1. During consecutive races the recovery fc did not return to resting values. The mean [La+] was 7.1 mmol.l-1 (range 3.5-15.0 mmol.l-1). The conclusions of this study suggest that riding in National Hunt races is a physically demanding occupation. The muscular activity in this profession requires a high metabolic drive and produces a significant cardiorespiratory response.

Adult↗
PubMed1987

The echolocation and hunting behavior of the bat, Pipistrellus kuhli.

The echolocation and hunting behavior of Pipistrellus kuhli was studied in the field using multi-exposure photography synchronized with high-speed tape recordings. During the search phase, the bats used 8-12 ms signals with sweeps (sweep width 3-6 kHz) and pulse intervals near 100 ms or less often near 200 ms. The bats seemed to have individual terminal frequencies that could lie between 35 and 40 kHz. The duty cycle of searching signals was about 8%. The flight speed of hunting bats was between 4.0 and 4.5 m/s. The bats reacted to insect prey at distances of about 70 to 120 cm. Given the flight speed, the detection distance was estimated to about 110 to 160 cm. Following detection the bat went into the approach phase where the FM sweep steepened (to about 60 kHz bandwidth) and the repetition rate increased (to about 30 Hz). The terminal phase or 'buzz', which indicates prey capture (or attempted capture), was composed of two sections. The first section contained signals similar to those in the approach phase except that the pulse duration decreased and the repetition rate increased. The second section was characterized by a sharp drop in the terminal frequency (to about 20 kHz) and by very short pulses (0.3 ms) at rates of up to 200 Hz. Near the beginning of the buzz the bat prepared for capturing the prey by extending the wings and forming a tail pouch. A pause of about 100 ms in sound emission after the buzz indicated a successful capture (Fig. 4).(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMedDate not supplied

Steroid-responsive optic neuropathy in a patient with Tolosa-Hunt syndrome: electrophysiologic findings.

We examined visual evoked potentials and pattern electroretinograms in a patient with Tolosa-Hunt syndrome associated with optic nerve involvement. The 82-year-old woman developed unilateral painful ophthalmoplegia and visual loss in the right eye. Magnetic resonance imaging showed an abnormal soft-tissue area in the right cavernous sinus and the right orbital apex. Symptoms responded rapidly to treatment with corticosteroid. Visual evoked potentials to flash and pattern stimuli were both remarkably reduced and delayed in the right eye in the acute stage; however they improved to almost normal after steroid therapy. The pattern electroretinogram recorded in the acute stage was normal bilaterally. These results indicate that optic nerve involvement in Tolosa-Hunt syndrome can be mild and reversible.

Aged↗