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Pathological bradyarrhythmia is rare in horses but should be especially considered when presented with a horse that has signs consistent with episodic weakness or collapse. This paper reviews the literature describing our current knowledge of, and possible mechanisms causing, clinically significant bradyarrhythmia in horses.

Background: Hypokalaemic periodic paralysis (HPP) is a rare muscular disorder characterised by episodic weakness associated with hypokalaemia. The disease can either be inherited or acquired and misdiagnosis of the disease is quite common. Most of the data available on the disease is from the western world. Studies reporting aetiological, clinical and metabolic profiles of Indian population are sparse. Hence we tried to provide insights of the disease among the Indian population. Aim: To study the aetiological, clinical and metabolic profile of patients diagnosed with Hypokalaemic Periodic Paralysis (HPP). Materials and Methods: This is an observational and analytical study on HPP diagnosed patients, during September 2011 to September 2014 in Kasturba Hospital, Manipal. A total of 23 patients were studied. Detailed history, clinical evaluation and metabolic workup for secondary causes of HPP were analysed. Results: Of the 23 patients, 57% had primary HPP while 43% had secondary HPP. The group of patients with primary HPP comprised of 92% males and 8% females with mean age of 28 years and the mean duration of symptoms of 18 hours. The group with secondary HPP comprised of 70% males and 30% females with mean age of 38.7 years and the mean duration of symptoms of 60 hours. The secondary causes of HPP were thyrotoxicosis (50%), infective diarrhea (20%), Crohn’s disease (10%), renal tubular acidosis (RTA) Type I (10%) and Conn’s syndrome (10%). Conclusion: In our study primary HPP was found to be more common than secondary HPP. Males were predominantly affected in both groups. HPP should be ruled out before starting therapy for Guillain-Barre Syndrome (GBS).

Kolevska J., R. Husnik, V. Brunclik, V. Mandys, Z. Vernerova, P. Schanilec, L. Barto‰ova, M. Svoboda:A Case of Canine Insulinoma . Acta Vet. Brno 2004, 73: 353-358. The paper reports on a ten-year-old German Shepherd presented with a history of episodic weakness and convulsions after exercise. The patient showed status epilepticus upon presentation. Glucose concentrations repeatedly determined by glucometer were below detection threshold. After intravenous administration of glucose the condition always temporarily improved, however after 1‐2 hours the situation reverted. Insulinoma was diagnosed on the basis of parallel determination of hypoglycaemia (glucose 3.3 mmol⋅l -1 ) and hyperinsulinaemia (2.398 pmol⋅l -1 ). A poor prognosis was stated and the owner decided for euthanasia. Necropsy revealed a tumour of pancreas. Subsequent pathological, immunohistochemical and electron microscopic examinations confirmed the diagnosis of insulinoma.

To evaluate the usefulness of the Doppler-derived effective orifice area (EOA) in assessing the hemodynamic severity of subaortic stenosis (SAS) in dogs, 2-dimensional and Doppler echocardiographic examinations were performed in 16 dogs with SAS, 22 normal adult dogs, and 22 normal puppies. The EOA was calculated by the continuity equation using the stroke volume determined in the right ventricular outflow tract. The EOA was significantly lower (P or =1.25 cm2/m2. Among the demographic and echocardiographic parameters measured in this study, only the indexed EOA was significantly associated (P = .03) with the occurrence of adverse events (eg, syncope, episodic weakness, ventricular arrhythmias). This study demonstrates the usefulness and feasibility of the indexed EOA as measured by Doppler echocardiography for noninvasive assessment of SAS severity in dogs.

A 13-year-old neutered female Yorkshire terrier presented with a history of progressive episodic weakness and disorientation of 4 months duration. Physical and neurologic examinations were normal at presentation. Abdominal ultrasound revealed a mass involving the right adrenal gland. Standard planar scintigraphy was performed at 4, 18, and 24 hours after intravenous injection 185 MBq (5mCi) of 123I-labeled metaiodobenzylguanidine (123I-MIBG). An area of focal intense uptake was identified in the area of the right adrenal gland. A pheochromocytoma was confirmed histologically after surgical excision.

A 2-year-old, neutered male, domestic shorthair cat was presented for investigation of dyspnea and episodic weakness. Clinical and ultrasonographic features were consistent with right ventricular cardiomyopathy. Pathological findings documented Uhl’s anomaly. Although rare, Uhl’s anomaly should be a differential diagnosis for cats with right-sided congestive heart failure. In particular, Uhl’s anomaly could be misdiagnosed as arrhythmogenic right ventricular cardiomyopathy due to the similarity of clinical and echocardiographic findings.

OBJECTIVE Disappearance of episodic weakness during pregnancy and an increase in myotonia after delivery have not been reported in hyperkalemic periodic paralysis (hyperPP). CASE REPORT In a 36-year-old white woman, with typical manifestations of episodic weakness of the limb muscles since age 3 years, hyperPP was diagnosed by demonstration of the T407M SCNA4 gene point mutation. At age 34 years, the index patient became pregnant for the first time. During the first trimester of her pregnancy, she noted a marked decrease in the frequency of the hyperPP attacks. During the second and third trimesters, the attacks of weakness completely disappeared and after delivery the attacks restarted with the same intensity and frequency as before pregnancy, but myotonia persisted between the attacks. Disappearance of the attacks during pregnancy was attributed to the hormonal changes. CONCLUSIONS Episodic attacks of weakness in hyperPP may disappear during the second and third trimesters of pregnancy, and myotonia between the attacks may persist after delivery.

Hypokalemic periodic paralysis is a condition, characterized by episodic weakness of the skeletal muscle. It may be presented as familial or sporadic (secondary). In sporadic cases, the patient may present with paralysis without any previous self or familial history. It is known that these patients are more susceptible to hypokalemia than normal individuals. In low serum K+ concentrations that the normal population can tolerate, these patients may easily suffer from paralysis. A sporadic case of hypokalemic quadriparesis associated with acute gastroenteritis without any self or family history is reported. The patient had experienced two episodes of quadriparesis within a short interval and responded dramatically to potassium replacement therapy.

Answers on p 421. A 62 year old retired woman was admitted to hospital with a 10 month history of pain and weakness in the muscles of both arms and legs. Her problems began with episodic weakness in both legs such that on occasions her legs gave way and she fell to the ground. She had a gradual deterioration in her muscle strength and after a fall five months before admission she …

Hypercalcemia was found in a dog examined for episodic weakness and disorientation. A right adrenal mass identified on abdominal ultrasonographic examination was diagnosed as a pheochromocytoma based on increased 1231-metaiodobenzylguanidine uptake and characteristic histopathological features. Serum intact parathyroid hormone (iPTH), total serum calcium, and ionized serum calcium were increased prior to surgery. Although both total and ionized serum calcium concentrations decreased to within normal reference ranges following surgery through postoperative day 17, subsequent high concentrations with increased serum iPTH concentrations confirmed concurrent primary hyperparathyroidism. A right parathyroid tumor was suspected based on a technetium-99m-sestamibi (99mTc-sestamibi) scan.

We report a rare case of episodic limb weakness caused by a tentorial dural arteriovenous fistula (DAVF) draining into the perimesencephalic veins. A middle-aged man presented with episodes of transient quadriparesis preceded by recurrent vomiting. The patient was initially suspected of having periodic paralysis but, due to the presence of recurrent vomiting and brisk reflexes, MR imaging of the brain was performed which revealed marked T2 hyperintensity of the brainstem and cervical cord along with multiple prominent flow voids. Cerebral angiography showed a Borden type 3 DAVF at the left tentorial margin which was draining into the perimesencepahlic and perimedullary veins and refluxing into the cervical epidural veins. The patient made a remarkable recovery after surgical interruption of the fistula.

Objective: To describe genotype-phenotype correlations in non-dystrophic myotonia (NDM). Background NDM are heterogeneous disorders caused by mutations in skeletal muscle sodium (SCN4A) and chloride channel (CLCN1) genes. Although NDMs are distinct, it is at times difficult to distinguish them clinically. Design/Methods: 95 participants with NDM were recruited from 6 centers across the United States, England, and Canada. Mutational and clinical analyses were performed in 93. They were categorized into chloride channel (CLCN1), sodium channel (SCN4A), myotonic dystrophy type 2 (DM2), and unknown mutations. We included DM2 since they clinically present as NDM. We quantitated symptoms and myotonia measurements. Results: Of the 93 subjects reviewed, 29 had CLCN1, 31 SCN4A, and 7 DM2 mutations with 26 as yet unknown. Stiffness was the most prominent symptom in all subtypes and occurred earlier in the SCN (median-5 yrs) vs CLCN (10 yrs) and DM2 (35 yrs) (p=0.0001). Painful myotonia was reported in SCN (25/31), CLCN (16/29), and DM2 (5/7) (p=0.19). Episodic weakness was reported in all subtypes: 23/31 SCN, 12/29 CLCN, and 4/7 DM2. Frequency of fixed weakness was higher in DM2 (6/7) compared to SCN (7/30) and CLCN (9/29) (p=0.012). Muscle hypertrophy was most common in CLCN (21/29) followed by SCN (13/31). DM2 patients did not have hypertrophy. Eye closure myotonia was observed more in SCN (23/31) compared to CLCN (7/29) or DM2 (2/7) (p=0.0005). Grip myotonia warm-up was noted in 20/29 CLCN, 8/31 SCN, and 1/7 DM2 subjects. Paradoxical hand grip & eye closure myotonia was seen in 16/31 SCN. Conclusions: While clinical features can help differentiate various NDM subtypes, mutational analysis is required due to an overlap in the clinical findings in NDM patients. Supported by: NIH/Office of Rare Disease Research Network sponsored Consortium for Clinical Investigations of Neurological Channelopathies (CINCH). Disclosure: Dr. Trivedi has nothing to disclose. Dr. Bundy has nothing to disclose. Dr. Raja Rayan has nothing to disclose. Dr. Salajegheh has nothing to disclose. Dr. Statland has nothing to disclose. Dr. Venance has received personal compensation for activities with Genzyme Corporation. Dr. Wang has nothing to disclose. Dr. Fialho has nothing to disclose. Dr. Hart has nothing to disclose. Dr. Gorham has nothing to disclose. Dr. Herbelin has nothing to disclose. Dr. Amato has received personal compensation for activities with MedImmune, Amgen, and Biogen Idec as a medical advisory board member. Dr. Hanna has nothing to disclose. Dr. Griggs has nothing to disclose. Dr. Barohn has received personal compensation for activities with Talecris Biotherapeutics, Genzyme Corporation and Speakers Bureau. Dr. Barohn has received research support from Alexion Pharmaceuticals.

Answers on p 795. A 37 year old women presented with a 10 year history of episodes of weakness and palpitations. On one or two occasions each year she was admitted to her local hospital where her serum potassium concentration was always below the reference range with the lowest concentration reported at 2.2 mmol/l (3.5–5.0 mmol/l). The episodes …

Hypokalemic periodic paralysis is characterized by episodic weakness, the presence of precipitating factors, and the tendency to resolve spontaneously. Physical examination of the patient between attacks often shows no abnormalities. Potassium replacement therapy may be needed for severe episodes.

Presentation of Case First admission. A sixty-eight-year-old man was admitted to the hospital because of weakness. He had been well until two months previously, when crampy pain developed in both lower quadrants of the abdomen, accompanied by urinary frequency; the pain persisted for one week. Three weeks later he began to have episodic weakness of the legs, which gradually became constant, interfering with walking and preventing the climbing of stairs. Anorexia, decreased alertness and a loss of 8 pounds in weight occurred. Three days before admission, while sitting quietly in a car, he suddenly lost consciousness for five minutes. He . . .

• Familial infantile myasthenia is a rare type of myasthenia that usually occurs in connection with respiratory depression. The condition is characterized by (1) absence of myasthenia in the mother, (2) occurrence of a similar disorder among siblings, (3) respiratory depression at birth, (4) episodic weakness and apnea during the first two years of life, and (5) improvement with age. Since the condition responds to anticholinesterase medication, early diagnosis is important. Familial infantile myasthenia is a potential cause of sudden infant death and should be considered in infants with unexplained respiratory distress.

Hypokalemic, 1 hyperkalemic, 2 and normokalemic 3 types of familial periodic paralysis have been reported. We studied a woman whose clinical syndrome and serum potassium changes were compatible with the hyperkalemic type of paralysis. In contrast to most of the previously described patients, her family did not demonstrate the pedigree pattern of an autosomal dominant gene with complete or almost complete penetrance. 2 In addition, she had a low serum calcium and carbon dioxide combining power during an attack. Only one other report describes hypocalcemia during an attack. 4 Our patient may represent a heretofore unreported variant of familial periodic paralysis or, possibly a similar but new metabolic defect. Report of Case A 35-year-old, married, Caucasian female secretary complained of episodic weakness and paralysis of her legs and arms. These attacks began when she was 18 months old. A typical attack was characterized by gradual weakness of the arms and