Online Clinical Case Study (May 2016)

Clinical Cardiology Series
臨床心臟科個案研究

The content of the May Cardiology Series is provided by:
Dr. TAN GuangMing
MBChB, MRCP, FHKCP, FHKAM (Med), Specialist in Cardiology
Dr. CHEUNG Shing Him, Gary
MBBS, MRCP, FHKCP, FHKAM (Med), Specialist in Cardiology
五月臨床心臟科個案研究之內容承蒙譚廣明醫生張誠謙醫生提供。

Too painful to swallow

Madam Tong, a 56-years-old lady with good past health, presented with recurrent syncope for one month. Each episode lasted for several minutes and proceeded by severe throat pain to both liquid and solid. Initial investigations including ECG, computed tomography of brain, routine blood test, echocardiogram and upper endoscopy were all normal. An electroencephalography captured an episode of LOC preceded by severe throat pain. Prominent frontal slowing was observed during this episode but no definite epileptic discharge was seen. Concurrent ECG recording is shown in the Figure 1.


1. What is shown in the Figure 1?
A. Artifacts
B. ECG leads dislodgement
C. Atrial fibrillation
D. Ventricular fibrillation
E. Sinus long pause
   
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2.

What is the diagnosis?
A. Generalized tonic-conic seizure
B. Esophageal spasm
C. Glossopharyngeal neuralgia
D. Sick sinus syndrome
E. Malingering

   
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3. What is the underlying pathology?
A. Encephalitis
B. Carcinoma of the esophagus
C. Vascular compression of the glossopharyngeal nerve root
D. Degenerative disease
E. Depression
   
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4. What further investigation should be performed?
A. Video electroencephalogram
B. Esophageal manometer
C. MRI scan of brain and brain stem
D. 24-hour ECG monitoring (Holter)
E. CT scan of the thorax
   
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5. What are the treatment options?
A. Anti-epileptics
B. Esophageal myotomy
C. Vascular decompression
D. Permanent pacemaker
E. C+D
   
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Answers
1.D,   2.C,   3.C,   4.C,   5.E

Discussion
The association of pain during swallowing should alert one to consider glossopharyngeal neuralgia (GN) as one of the differential diagnoses. GN is first described by Weisenburg in 1910 in a patient with a cerebellopontine angle (CPA) tumor1. Thereafter, multiple case series has been reported, and most of the cases were idiopathic. Other causes include Eagle syndrome (long styloid process), CPA tumor, parapharyngeal space occupying lesion, and multiple sclerosis. Vascular compression of the glossopharyngeal nerve root has been proposed as a possible pathology based on observations from the study of trigeminal neuralgia.

The association of GN with cardiac syncope was first noted by Harris in 1939 and subsequently reported by Rushton in 4 of his 217 cases of GN2. The proposed mechanism is the activation of the dorsal motor nucleus of the vagus nerve by the increased input from the glossopharyngeal nerve during swallowing, via the tractus solitarius in the brainstem. The dorsal nucleus is the principal autonomic nucleus supplying parasympathetic fibers to the heart, activation of which will cause significant bradycardia or even asystole such as in our patient3. Another possible mechanism is carotid hypersensitivity as a result of the functional spillover from the glossopharyngeal nerve since the carotid sinus nerve (nerve of Hering) is an afferent branch of the glossopharyngeal nerve4.

The first line treatment of GN is by carbamazepine or gabapentin, but relapse is common and unpredicatable5. Surgical microvascular decompression has been used successfully in the treatment of trigeminal neuralgia, and has shown promise in the treatment GN with a 76% of complete resolution of pain at 48 month6. However, longer term follow up data is not available. Permeant pacemaker has been shown to reduce recurrent syncope in patients with neutrally mediated syncope in the ISSUE-3 trial7. In our patient, even surgical decompression effectively relieved her symptoms, a permanent pacemaker was implanted because of the uncertain recurrence rate and the severity of her sinus pause associated with myoclonic seizure.

References:

  1. Weisenburg T. Cerebello-pontine tumor diagnosis for six years as tic douloureux: the symptoms of irritation of the ninth and twelfth cranial nerves. JAMA 1910; 54: 1600-1604.
  2. Rushton JG, Stevens C, Miller RH. Glossopharyngeal (vagoglossopharyngeal) neuralgia. A study of 217 cases. Arch Neurol 1981; 38: 201-205.
  3. Kamosh LJ, Gardner WJ, Stowell A. Glossopharyngeal neuralgia. Physiological consideration of the role of ninth and tenth cranial nerves. Report of cases. TransAmNeurol Assoc 1947;72:205-7.
  4. Kong Y, Heyman A, Entman ML, et al. Glossopharyngeal neuralgia associated with bradycardia, syncope and seizures. Circulation 1964;30:109-13.
  5. Rozen TD: Trigeminal neuralgia and glossopharyngeal neuralgia. Neurol Clin 2004; 22:185-206.
  6. Resnick DK, Jannetta PJ, Bissonnette D, et al. Microvascular decompression for glossopharyngeal neuralgia. Neurosurgery 1995; 36: 64-69.
  7. Brignole M, Menozzi C, Moya A, et al. Pacemaker therapy in patients with neutrally mediated syncope and documented asystole: Third International Study on Syncope of Uncertain Etiology (ISSUE-3): a randomized trial. Circulation. 2012 May 29;125(21):2566-71.
   
   

Dermatology Series 皮膚科病例研究

Dermatology Series for May 2016 is provided by:
Dr. CHANG Mee, Mimi, Dr. TANG Yuk Ming, William, Dr. CHAN Hau Ngai, Kingsley, Dr. KWAN Chi Keung and Dr. LEUNG Wai Yiu
Specialists in Dermatology & Venereology
五月皮膚科個案研究之內容承蒙張苗醫生鄧旭明醫生陳厚毅醫生關志強醫生梁偉耀醫生提供。

A young woman with hair loss

A 30-year-old woman presented with progressive hair loss for 1 year. It was a red and mildly scaling patch on the occipital scalp which enlarged with time. There was mild burning sensation initially. There were no other systemic symptoms. Clinically, it was a well-defined patch of alopecia with erythema, central atrophy and peripheral brown dyspigmentation. The follicular openings are lost.

 

Answers

1. What is the most likely diagnosis?
The patient is most likely suffering from discoid cutaneous lupus (DLE).
 

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2. What are the differential diagnoses?
Other differential diagnosis for such symptomatic scarring alopecia include lupus profundus, lichen planopilaris, folliculitis decalvans, dermatomyositis, radiation dermatitis and inflammatory tinea capitus with secondary scarring.
 

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3. How do we investigate?
We perform dermatoscopic examination to evaluate the scalp surface, wood's lamp examination and fungal scraping and hair plucking for mycology to exclude tinea infection, and skin biopsy for histology and immunofluorescence to confirm DLE and exclude the differential diagnoses. Systemic evaluation and serum autoimmune screening are advised.
 

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4. What is the cause of her condition?

Discoid lupus erythematosus (DLE) is a form of chronic cutaneous lupus, which is a cutaneous manifestation of autoimmune dysfunction (lupus erythematosus). It is more common in pigmented races and in young women. About 5-10% of DLE patients progress into systemic lupus erythematosus (SLE). It also forms one of the diagnostic criteria for SLE.

DLE usually presents as enlarging erythemato-violaceous patches on the scalp, face and ears. Patients usually complain of itch, burning and painful sensations. It is characterized by follicular plugging, adherent scaling (lifting of which can show follicular hyperkeratotic spikes, also known as 'carpet tack scales'), telangiectasia, hypo- and hyperpigmentation. If not treated early, cutaneous atrophy, scarring alopecia and cosmetic disfigurement will develop (follicular openings are absent and lost).

 

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5. How do we treat her?
  The patient is on treatment with hydroxychloroquine and intralesional steroid injection (topical steroid is also frequently used). She is advised to have active sun protection. Other second line treatment includes systemic steroid or systemic retinoid. Third line treatment includes dapsone, immunosuppressants (mycophenolate mofetil, methotrexate) or thalidomide.
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