A case report on severe hypothyroidism associated with complete bilateral ptosis: A rare presentation / Journal of the ASEAN Federation of Endocrine Societies

@#Thyroid hormones exert a major control over many vital functions of the human body and thus have an important role in maintaining homeostasis. Therefore, the hypothyroid state is associated with a wide spectrum of symptoms affecting almost all bodily functions. Among the major organs affected, nervous system and body metabolism are severely affected. Facial appearance, including ocular changes, is a characteristic of the hypothyroid state. The most prominent ocular features of hypothyroidism include loss of eyelashes and eyebrows, especially on the temporal side, puffiness of the eyelids, ocular irritation and ocular protrusion. Mild drooping of the eyelids is also seen in some patients due to loss of sympathetic tone; however the occurrence of complete bilateral ptosis is rare in the setting of hypothyroidism. Our patient, who presented with sudden onset bilateral ptosis, had no manifestations of diseases involving the ocular structure, cranial nerves or central nervous system primarily; the cause may be attributed to severe hypothyroidism considering the dramatic response to thyroxine replacement therapy.

Two Cases of Delayed Post-radiation Lower Cranial Nerve Palsies

Radiation-induced lower cranial neuropathy shows a clinical presentation similar to tumor recurrence or amyotrophic lateral sclerosis. We experienced two patients with bulbar palsies several years after radiotherapy for nasopharyngeal cancer. Brain magnetic resonance imaging showed no evidence of tumor recurrence. Electrophysiologic studies demonstrated mild denervation changes and myokymic discharges in muscles innervated by cranial nerves. Bulbar palsies progressed for 1 year then became stable. We emphasize the importance of myokymic discharges in the differential diagnosis of radiation-induced cranial neuropathy as radiation plexopathies.

An interesting case report of Morvan's syndrome from the Indian subcontinent.

The French physician Augustine Marie Morvan first used the term 'la choriotae fibrillare' to describe a syndrome characterized by peripheral nerve hyperexcitability, dysautonomia, insomnia and fluctuating delirium. There are no published reports of the condition from the Indian subcontinent. We report the first such case from the region. Our patient, a 24-year-old male, presented with easy fatigability and stiffness in both the calves for 18 months; continuous twitching of muscles of all four limbs and jaw for two months; hyperhydrosis, palpitations, urinary symptoms, burning dysesthesia in hands, insomnia and abnormal sleep behavior for about a month. Patient had bilateral hyper-reflexia with extensor plantar on the right and equivocal response on the left. Electromyography revealed continuous muscle fiber activity. Thyroid function test, electroencephalography, computerized tomography scan (head) and routine cerebrospinal fluid analysis were normal. The patient showed marked clinical and electrophysiological improvement on prednisolone along with symptomatic therapy over the next two months.

Hemifacial Spasm: A Neurosurgical Perspective

Hemifacial spasm (HFS) is characterized by tonic clonic contractions of the muscles innervated by the ipsilateral facial nerve. Compression of the facial nerve by an ectatic vessel is widely recognized as the most common underlying etiology. HFS needs to be differentiated from other causes of facial spasms, such as facial tic, ocular myokymia, and blepharospasm. To understand the overall craniofacial abnormalities and to perform the optimal surgical procedures for HFS, we are to review the prevalence, pathophysiology, differential diagnosis, details of each treatment modality, usefulness of brainstem auditory evoked potentials monitoring, debates on the facial EMG, clinical course, and complications from the literature published from 1995 to the present time.

Therapeutic Effect of Botulinum Toxin Injection in Eyelid Myokymia Patients

PURPOSE: To evaluate clinical manifestations and effects of botulinum toxin in eyelid myokymia patients. METHODS: Between March 2001 and March 2005, botulinum toxin injection therapy was performed in 12 eyelid myokymia patients. We evaluated duration of disease, systemic disease, drug history, and range, grade, frequency of myokymia at the first visit. Botulinum injections were performed in patients with normal results of blood tests and neuroimaging tests. Improvement of symptoms, duration of effectiveness, and complications were evaluated after the botulinum toxin injection therapies. Data was analyzed with the Mann-Whitney U test. RESULTS: Improvement of symptoms was noticed in 10 patients (83.3%). Among the 10 patients, 5 patients were decided as cured patients because they did not require any retreatment. Range and grade of myokymia didn't affect on effect of treatment. Pain, edema, excessive tearing, and unnatural face were noticed as complications of treatment, but all complications disappeared 2 weeks after the treatment. CONCLUSIONS: Botulinum toxin injection therapy is noninvasive and effective treatment in benign eyelid myokymia patients without causal neurologic disease.

A Case of Morvan Syndrome

Neuromyotonia, or Isaacs' syndrome, consists of continuous muscle fiber activity caused by hyperexcitability of the peripheral nerves. Rarely, these patients also develop CNS symptoms characterized by confusion, insomnia, hallucinations, and agitation. A rare disease consisting of neuromyotonia, autonomic symptoms, and CNS dysfunction is called Morvan's syndrome. We report a 24-year-old man who presented with insomnia, malaise, anorexia, hyperhidrosis, palpitation and myokymia in both the lower extremities. The pathomechanism of Morvan's syndrome is related to the voltage-gated K+ channel (VGKC) antibodies.

X-linked Charcot-Marie-Tooth disease with myokymia: report of a family.

The clinical and electrophysiologic profiles of two brothers suffering from Charcot-Marie-Tooth disease are presented. Both had widespread muscle twitching in the legs which showed electrophysiologic features of myokymia. Pedigree analysis suggested an x-linked recessive form of inheritance. This appears to be the first report of an Indian family with x-linked Charcot-Marie-Tooth disease.

The Effects of Thoracic Sympathetic Ganglion Block and Gabapentin in a Patient with Myokymia and Neuropathic Pain after a Thoracotomy / 대한마취과학회지

Myokymia is one of involuntary movement, which is characterized by undulatory muscle spasm, similar to the worm's crawl. Sometimes muscle pain, itchy sensation, dysautonomia and other symptoms are associated with it. Derangement of the peripheral or central nervous system after nerve or tissue damage is suspected as the source of impulse generators causing this symptom. We encountered a patient with neuropathic pain and myokymia after thoracotomy. Although several medications and nerve blocks have been applied, all have failed to provide symptom relief. We experienced improvement of the pain and involuntary movement with a thoracic sympathetic ganglion block and gabapentin.

Treatment with Botulinum Toxin A in Continuous Facial and Neck Myokymia: A case report

Myokymia is a clinical phenomenon characterized by undulating, vermicular, rippling and wavelike movements spreading across the muscle surface. Facial myokymia is an unusual complication of brainstem hemorrhage. It tends to occur in brainstem tumor or multiple sclerosis. We report a 51-year-old man with continuous facial and neck myokymia after brainstem hemorrhage, who revealed focal myokymic discharges in face, neck and pharyngolaryngeal muscles in-nervated by cranial nerve V, VII, X, and XI. After injection of 20-80 units of Botulinum toxin type A (Dysport ) to the left orbicularis oris, mentalis, mylohyoid and posterior belly of digastric muscles, amplitude of continuous myokymic discharges was markedly reduced. We recommend Botulinum toxin injection as a very effective therapeutic method in managing focal movement disorders.

Cramp Fasciculation Syndrome

Cramp-fasciculation syndrome can be characterized by acute onset of action induced muscle cramps without weakness or muscle atrophy. The neurological examination is normal except for fasciculation or myokymia. Electromyography (BMG) shows fasciculation and muscle biopsy may show neurogenic abnormalities. We describe a 65 years old woman who had muscle cramps and fasciculations in the hands and bilateral feet for 2 months. Laboratory studies including CBC, erythrocyte sedimentation rate, serum electrolytes, blood chemistries, creatinekinase, serum magnesium, serum ionized calcium, thyroid function tests, parathyrold function tests, urinalysis were all normal. Motor and sensory nerve conduction studies were normal. EMG study showed fasciculation in the right abductor pollicis brevis muscle and the bilateral anterior tibialis muscles There were no showers of electrical potentials following the M-wave after repetitive median, ulnar, and peroneal nerve stimulations at the frequency of 1, 2, and 5-Hz. She improved dramatically after vitamin E treatment.

Cramp Fasciculation Syndrome

Cramp-fasciculation syndrome can be characterized by acute onset of action induced muscle cramps without weakness or muscle atrophy. The neurological examination is normal except for fasciculation or myokymia. Electromyography (BMG) shows fasciculation and muscle biopsy may show neurogenic abnormalities. We describe a 65 years old woman who had muscle cramps and fasciculations in the hands and bilateral feet for 2 months. Laboratory studies including CBC, erythrocyte sedimentation rate, serum electrolytes, blood chemistries, creatinekinase, serum magnesium, serum ionized calcium, thyroid function tests, parathyrold function tests, urinalysis were all normal. Motor and sensory nerve conduction studies were normal. EMG study showed fasciculation in the right abductor pollicis brevis muscle and the bilateral anterior tibialis muscles There were no showers of electrical potentials following the M-wave after repetitive median, ulnar, and peroneal nerve stimulations at the frequency of 1, 2, and 5-Hz. She improved dramatically after vitamin E treatment.

A Case of the Generalized Myokymia Associated with Lung Cancer

There are widely recognized unusual remote neurological manifestations of internal malignancy. However, as far as the author's knowledge, the generalized myokymia associated with myokymic discharge has not been reported as paraneoplastic syndrome earlier. The patient was a 63-year-old female with generalized myokymia and paresthesia for 4months. The nerve conduction study revealed mild sensorimotor polyneuropathy and EMG study showed the generalized myokymic discharges, which were not changed by sleeping or brachial plexus block. The carbamazepine reduced the amount of myokymia and myokymic discharges but not the benzodiazepine. The diagnosis of lung cancer was confirmed by needle aspiration biopsy, and the cell types of lung cancer were mixed undifferentiated and small cell type. By the administration of anti-cancer drugs including cisplatin, the myokymia and myokymic discharges were completely disappeared. The patient died at 8 months after the diagnosis.

A Case of Cervical cord Tumor with Focal Myokymic Discharge in Intrinsic Hand Muscles

Myokymic discharge is a train of motor unit potentials that occur spontaneously, recur regularly, and sometimes may be associated with clinical myokymia. We report a 39-years-old male with progressive muscle weakness and atrophy of right upper extremity, who revealed focal myokymic discharges in the intrinsic hand muscles where no denervation was evident on EMG study. The cervical CTMM and MRI study showed a large cervical cord tumor at C3-C6 spine level. Postoperative pathological diagnosis of the tumor was anaplastic astrocytoma.

Electrophysiological Studies on Myokymia: four facial and five limb myokymia

No abstract available.