Immediate and long-term impact of the COVID-19 pandemic on delivery of surgical services.

BACKGROUND: The ongoing pandemic is having a collateral health effect on delivery of surgical care to millions of patients. Very little is known about pandemic management and effects on other services, including delivery of surgery. METHODS: This was a scoping review of all available literature pertaining to COVID-19 and surgery, using electronic databases, society websites, webinars and preprint repositories. RESULTS: Several perioperative guidelines have been issued within a short time. Many suggestions are contradictory and based on anecdotal data at best. As regions with the highest volume of operations per capita are being hit, an unprecedented number of operations are being cancelled or deferred. No major stakeholder seems to have considered how a pandemic deprives patients with a surgical condition of resources, with patients disproportionally affected owing to the nature of treatment (use of anaesthesia, operating rooms, protective equipment, physical invasion and need for perioperative care). No recommendations exist regarding how to reopen surgical delivery. The postpandemic evaluation and future planning should involve surgical services as an essential part to maintain appropriate surgical care for the population during an outbreak. Surgical delivery, owing to its cross-cutting nature and synergistic effects on health systems at large, needs to be built into the WHO agenda for national health planning. CONCLUSION: Patients are being deprived of surgical access, with uncertain loss of function and risk of adverse prognosis as a collateral effect of the pandemic. Surgical services need a contingency plan for maintaining surgical care in an ongoing or postpandemic phase.

ANTECEDENTES: La pandemia en curso tiene un efecto colateral sobre la salud en la prestación de atención quirúrgica a millones de pacientes. Se sabe muy poco sobre el manejo de la pandemia y sus efectos colaterales en otros servicios, incluida la prestación de servicios quirúrgicos. MÉTODOS: Se ha realizado una revisión de alcance de toda la literatura disponible relacionada con COVID-19 y cirugía utilizando bases de datos electrónicas, páginas web de sociedades, seminarios online y repositorios de pre-publicaciones. RESULTADOS: Se han publicado varias guías perioperatorias en un corto período de tiempo. Muchas recomendaciones son contradictorias y, en el mejor de los casos, se basan en datos anecdóticos. A medida que las regiones con el mayor volumen de operaciones per cápita se ven afectadas, se cancela o difiere un número sin precedentes de operaciones. Ninguna de las principales partes interesadas parece haber considerado cómo una pandemia priva de recursos a los pacientes que necesitan una intervención quirúrgica, con pacientes afectados de manera desproporcionada debido a la naturaleza del tratamiento (uso de anestesia, quirófanos, equipo de protección, contacto físico y necesidad de atención perioperatoria). No existen recomendaciones sobre cómo reanudar la actividad quirúrgica. La evaluación tras la pandemia y la planificación futura deben incluir a los servicios quirúrgicos como una parte esencial para mantener la atención quirúrgica adecuada para la población también durante un brote epidémico. La prestación de servicios quirúrgicos, debido a su naturaleza transversal y a sus efectos sinérgicos en los sistemas de salud en general, debe incorporarse a la agenda de la OMS para la planificación nacional de la salud. CONCLUSIÓN: Los pacientes se ven privados de acceso a la cirugía con una pérdida de función incierta y riesgo de un pronóstico adverso como efecto colateral de la pandemia. Los servicios quirúrgicos necesitan un plan de contingencia para mantener la atención quirúrgica durante la pandemia y en la fase post-pandemia.

Long-term morbidity after multivisceral resection for retroperitoneal sarcoma.

BACKGROUND: More than 60 per cent of patients treated surgically for primary retroperitoneal sarcoma survive for at least 5 years. Extended surgical resection has been proposed for primary disease, but long-term morbidity data are lacking. A cross-sectional study was conducted to assess the long-term morbidity of patients undergoing surgery for retroperitoneal sarcoma. METHODS: Patients operated on between January 2002 and December 2011 were eligible for the study. Long-term morbidity was evaluated based on a semistructured clinical interview. Lower limb function was assessed by means of the Lower Extremity Functional Scale (LEFS), a self-report questionnaire with a total score ranging from 0 (low functioning) to 80 (high functioning). Pain was investigated by means of the Brief Pain Inventory--Short Form, with pain intensity scores reported on a scale from 0 (no pain) to 10 (worst pain). RESULTS: Some 243 patients underwent surgery, and 101 of 160 patients who were alive at the time of the investigation responded to the study invitation letter. Finally, 95 patients were enrolled in the study. Sensory impairment of the limbs was reported in 72 patients (76 per cent). The median LEFS score was 60 (i.q.r. 43-73). Mean scores for the pain intensity items varied from 1.23 to 2.68. In multivariable analysis, there was no difference in median levels of creatinine at survey between patients who did or did not undergo nephrectomy (difference between median values 13 (95 per cent c.i. -4 to 30) µmol/l; P = 0.170). CONCLUSION: Severe chronic pain and lower limb motor impairment after multivisceral resection for retroperitoneal sarcomas are rare. Long-term renal function is not significantly impaired when nephrectomy is performed.

Myelin imaging measures as predictors of cognitive impairment in MS patients: A hybrid PET-MRI study.

BACKGROUND: Cognitive impairment is one of the concerns of Multiple Sclerosis (MS) and has been related to myelin loss. Different neuroimaging methods have been used to quantify myelin and relate it to cognitive dysfunctions, among them Magnetization Transfer Ratio (MTR), Diffusion Tensor Imaging (DTI), and, more recently, Positron Emission Tomography (PET) with 11C-PIB. OBJECTIVE: To investigate different myelin imaging modalities as predictors of cognitive dysfunction. METHODS: Fifty-one MS patients and 24 healthy controls underwent clinical and neuropsychological assessment and MTR, DTI (Axial Diffusion-AD and Fractional Anisotropy-FA maps), and 11C-PIB PET images in a PET/MR hybrid system. RESULTS: MTR and DTI(FA) differed in patients with or without cognitive impairment. There was an association of DTI(FA) and DTI(AD) with cognition and psychomotor speed for progressive MS, and of 11C-PIB uptake and MTR for relapsing-remitting MS. MTR in the Thalamus (ß= -0.51, p = 0.021) and Corpus Callosum (ß= -0.24, p = 0.033) were predictive of cognitive impairment. DTI-FA in the Caudate (ß= -26.93, p = 0.006) presented abnormal predictive result. CONCLUSION: Lower myelin content by 11C-PIB uptake was associated with worse cognitive status. MTR was predictive of cognitive impairment in MS.

Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.

Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.

Recurrent neuromyelitis optica in Brazilian patients: clinical, immunological, and neuroimaging characteristics.

Neuromyelitis optica has not been thoroughly studied in Brazilian patients following the discovery of NMO-IgG and its specific antigen aquaporin-4. In this study we aimed to describe the clinical NMO-IgG immunological status and neuroimaging characteristics of recurrent neuromyelitis optica in a series Brazilian patients. We undertook a retrospective study of 28 patients with recurrent neuromyelitis optica, according to 1999 Wingerchuk's diagnostic criteria. Data on NMO-IgG status, clinical features, and MRI findings were analyzed. Three men and 25 women were evaluated. Median age at onset of disease was 26 years (range 7-55); median time of follow-up was 7 years (range 2-14). The mean time elapsed between the first and the second attack was 17 months (median 8.5; range 2-88). NMO-IgG was detected in 18 patients (64.3%). Four patients died due to respiratory failure. Most patients presented with cervical (36%) and cervical-thoracic myelitis (46.4%). Holocord lesion was the most common pattern of involvement (50%) on the axial plane. We did not find a statistical association between myelitis extension and NMO-IgG result. Our series of Brazilian patients showed a younger age of onset than previously reported. In our series, in contrast to previous reports, there was no correlation between the extension of myelitis and NMO-IgG positivity.

Strategies for the delay of surgery in the management of resectable hepatobiliary malignancies during the COVID-19 pandemic.

Objective: We aimed to review data about delaying strategies for the management of hepatobiliary cancers requiring surgery during the covid-19 pandemic. Background: Given the covid-19 pandemic, many jurisdictions, to spare resources, have limited access to operating rooms for elective surgical activity, including cancer, thus forcing deferral or cancellation of cancer surgeries. Surgery for hepatobiliary cancer is high-risk and particularly resource-intensive. Surgeons must critically appraise which patients will benefit most from surgery and which ones have other therapeutic options to delay surgery. Little guidance is currently available about potential delaying strategies for hepatobiliary cancers when surgery is not possible. Methods: An international multidisciplinary panel reviewed the available literature to summarize data relating to standard-of-care surgical management and possible mitigating strategies to be used as a bridge to surgery for colorectal liver metastases, hepatocellular carcinoma, gallbladder cancer, intrahepatic cholangiocarcinoma, and hilar cholangiocarcinoma. Results: Outcomes of surgery during the covid-19 pandemic are reviewed. Resource requirements are summarized, including logistics and adverse effects profiles for hepatectomy and delaying strategies using systemic, percutaneous and radiation ablative, and liver embolic therapies. For each cancer type, the long-term oncologic outcomes of hepatectomy and the clinical tools that can be used to prognosticate for individual patients are detailed. Conclusions: There are a variety of delaying strategies to consider if availability of operating rooms decreases. This review summarizes available data to provide guidance about possible delaying strategies depending on patient, resource, institution, and systems factors. Multidisciplinary team discussions should be leveraged to consider patient- and tumour-specific information for each individual case.

Beyond weakness: Characterization of pain, sensory profile and conditioned pain modulation in patients with motor neuron disease: A controlled study.

BACKGROUND: Motor neuron diseases (MND) represent a group of disorders that evolve with inexorable muscle weakness and medical management is based on symptom control. However, deeper characterization of non-motor symptoms in these patients have been rarely reported. METHODS: This cross-sectional study aimed to describe non-motor symptoms in MND and their impact on quality of life and functional status, with a focus on pain and sensory changes. Eighty patients (31 females, 55.7 ± 12.9 years old) with MND underwent a neurological examination, pain, mood, catastrophizing and psychophysics assessments [quantitative sensory testing (QST) and conditioned pain modulation (CPM)], and were compared to sex- and age-matched healthy controls (HC). RESULTS: Chronic pain was present in 46% of patients (VAS =5.18 ± 2.0). Pain of musculoskeletal origin occurred in 40.5% and was mainly located in the head/neck (51%) and lower back (35%). Neuropathic pain was not present in this sample. Compared to HC, MND patients had a lower cold detection threshold (p < 0.002), and significantly lower CPM scores (4.9 ± 0.2% vs. 22.1 ± 0.2%, p = 0.012). QST/CPM results did not differ between MND patients with and without pain. Pain intensity was statistically correlated with anxiety, depression and catastrophism, and spasticity scores were inversely correlated with CPM (ρ = -0.30, p = 0.026). CONCLUSIONS: Pain is frequently reported by patients with MNDs. Somatosensory and CPM changes exist in MNDs and may be related to the neurodegenerative nature of the disease. Further studies should investigate the most appropriate treatment strategies for these patients. SIGNIFICANCE: We report a comprehensive evaluation of pain and sensory abnormalities in motor neuron disease (MND) patients. We assessed the different pain syndromes present in MND with validated tools, and described the QST and conditioned pain modulation profiles in a controlled design.

Renal angiomyolipomas: At least two diseases. A series of patients treated at two European institutions.

OBJECTIVE: The aim of this study is to analyze the outcome of renal angiomyolipomas (AML) at two European institutions. METHODS: The data were collected from patients with a primary AML who were treated at Gustave Roussy, Villejuif, France and Fondazione IRCCS Istituto Nazionale dei Tumori in Milan, Italy from 1998 to 2014. The specimens were classified as classic AML (C AML) or epithelioid AML (E AML) based on the percentage of epithelioid cells. RESULTS: There were 40 patients identified for the study (35 C AML, 5 E AML). One patient had an associated tuberous sclerosis complex. Six patients (15%) had bilateral AML. The imaging results were significantly different between C/E AML. E AML was associated with fewer bilateral lesions, more renal vein/vena cava extension, and more poor or non-fatty aspects. Surgery/active surveillance (AS)/chemo radiation were applied for 28/11/1 patients, respectively. The median tumor size was significantly smaller (3.75 cm) in patients receiving AS (median 15 cm when surgically resected). The median patient follow-up was 43 months. The three-year overall survival was significantly better for patients with C AML than E AML (100% versus 50%, p < 0.0001). The univariate analysis identified the OS prognostic factors were E AML histologic subtype (p < 0.001), poor/non fatty features (p = 0.002), and renal vein extension on imaging (p = 0.01). CONCLUSION: AML manifests as at least two different entities with significantly different outcomes. Epithelioid subtype, poor/non-fatty features, and renal vein involvement are all associated with worse survival.

Resectable extra-pleural and extra-meningeal solitary fibrous tumours: A multi-centre prognostic study.

BACKGROUND: Extra-pleural and extra-meningeal solitary fibrous tumour (SFT) is a rare sarcoma histotype curable with surgery in the majority of patients. The behaviour of these tumours ranges from indolent/very low grade to malignant/high grade but it is still not possible to accurately predict prognosis after surgery. We have investigated a multi-centre series to stratify the risk of recurrence to patients with SFTs. METHODS: We retrospectively analysed the data from 243 patients who underwent surgery (2002-2011) at four sarcoma referral centres. RESULTS: Upon univariate analysis, hypercellularity, atypia, necrosis, high mitotic rate (ie >4 mitoses/10 HPF) were associated with both disease-free and overall survival. Surgical margins were a significant prognostic factor for disease-free (P = 0.007) but not for overall survival. Unexpectedly, larger tumour size was associated with a better prognosis (P = 0.038) and fewer recurrences (P = 0.024). Upon multivariable analysis, high mitotic rate (hazard ratio, HR = 2.85, P = 0.002), cellular atypia (HR = 1.62, P = 0.015) and hypercellularity (HR = 1.82, P = 0.031) were significantly associated with recurrences. A SFT recurrence score has been provided to stratify risk of recurrence. CONCLUSION: This study provides a prognostic model to stratify risk of recurrence in patients with resectable SFTs. This allows clinician to decide on an optimal follow-up strategy and to select patients that may benefit from adjuvant treatments.

Intramedullary cysticercosis. Case report, literature review and comments on pathogenesis.

A 60-year-old woman developed progressive spastic crural paraplegia and global anesthesia and global anesthesia below T10, succumbing to urinary infection and bronchopneumonia 8 months after the onset of symptoms. At necropsy, the spinal cord was completely destroyed by a necrotic intramedullary cysticercus at T8. Six cysticerci were found in the brain. A review of the 17 published cases of intramedullary cysticercosis (including this one) showed this condition to be clinically indistinguishable from spinal cord tumours. The surgical prognosis was fair in 8 of 11 operated patients. The role of hematogenous and ventriculo-ependymal pathways in the pathogenesis of intramedullary cysticercosis was examined. The topographical distribution of intramedullary cysticerci (5 cervical, 12 thoracic, 2 lumbar, none sacral) was found to be statistically proportional to the blood flow to each of these regions; this favours the hematogenous route of infestation. The low spinal cord blood flow (100 times less than that to the brain), the type of vascularization of the cord (low calibre vessels under low pressure) and peculiarities of the cord tissue (such as its harder consistency) are all thought to be contributory factors accounting for the scarcity of intramedullary cysticerci. No evidence for an ependymal route of spread could be adduced.

Surgical results for multiple primary lung cancers.

OBJECTIVE: The development of a multiple primary lung cancer (MPLC) is not rare in long-term survivors after curative resections. We analysed our experience in order to verify surgical results and long-term survival in our patients. METHODS: From 1971 to 1999, 80 patients with MPLC (two tumours each, total 160) were treated at the Division of Thoracic Surgery of the University of Padua. Our criteria for the definition of a synchronous or metachronous cancer are those proposed by Martini and Melamed. We had 19 patients with a synchronous tumour and 61 patients with a metachronous tumour. We performed 95 lobectomies, 5 completion pneumonectomies and 53 segmentectomies. Of 160 MPLCs, 60 were squamous carcinomas, 78 adenocarcinomas, 8 small cell lung cancers, 9 large cell lung cancers and 5 other tumours. Of 160 MPLCs, 140 were N0 disease (87.5%) and 20 were N1 or N2 disease (12.5%). RESULTS: The overall 30-day mortality was 2.5% (2 patients). Eighteen patients (22.5%) had postoperative complications. Survival at 5 and 10 years for all patients was 72% and 58%, respectively. Five-year survival for patients with metachronous and synchronous disease from the time of initial diagnosis of cancer was 85% and 20% (P=0.001), and 10-year survival was 58% and 0% (P=0.001), respectively. Survival after the development of a metachronous lesion was 51% at 5 years and 20% at 10 years. The 5-year survival of patients with metachronous tumours undergoing standard surgical procedures of the second tumour was 52%; the 5-year survival of patients undergoing atypical or segmental resections was 55%. CONCLUSIONS: Careful follow-up is recommended in all patients surviving curative resection. More accurate selection criteria for MPLC is required. An aggressive surgical approach is justified in patients with MPLC and offers the greatest chance for long-term survival even in the case of limited resection.

Long term results of surgery and chemotherapy in small cell lung cancer.

OBJECTIVE: The aim of the study is to analyse long-term results of patients with small cell lung cancer (SCLC) treated at the same institution according to a prospective study including surgery, chemotherapy, and radiotherapy. METHODS: From 1981 to 1995, 104 patients with a proven histology of SCLC underwent surgery, chemotherapy, and radiotherapy. Fifty-one patients with operable stage I or II lesion received surgical resection followed by adjuvant chemotherapy and radiotherapy. Fifty-three patients with proved SCLC and clinical stage III received induction chemotherapy followed by surgery and radiotherapy. All patients received from four to six courses of chemotherapy and 36 had prophylactic cranial irradiation (PCI). All patients had follow-up for at least 1 year, and survival time was calculated from the date of the diagnosis until death or most recent follow-up. RESULTS: Ninety-six patients were male and eight female. We performed 29 pneumonectomies, eight bilobectomies, 66 lobectomies and one no resection. Regarding the clinical stage, 35 patients (33.6%) had stage I, 16 patients (15.4%) had stage II and 53 (51%) had stage III. Post-operative pathologic staging revealed stage I in 37 patients (35.6%), stage II in nine patients (8.6%), stage III in 45 patients (43.3%), and in 13 patients (12.5%) there was no more tumor. The 30-day mortality was 2% (two patients). Fourteen patients (13.4%) had post-operative complications. Fifty-one patients (49%) had a relapse. The median follow-up was 55 months. Twenty-six patients remain alive and 78 patients have died. The overall 5-year survival rate was 32%, with an estimate median survival time of 28 months; according to the pathologic stage, the survival data were 52.2%, 30% and 15.3% for stage I, II and III, respectively (P < 0.001). The 5-year survival was 41% in patients without SCLC after chemotherapy. CONCLUSION: As with non-small cell lung cancer, survival following surgery and chemotherapy clearly correlates with the stage. At present, it is not clear whether surgery is truly effective for patients with SCLC. In our experience, the complete elimination of small cell lung cancer is associated with an improvement in survival (41% at 5 years).

Pediatric multiple sclerosis report of 14 cases.

We present clinical data from 14 multiple sclerosis (MS) patients who have been admitted to our hospital between January 1980 and May 1992, whose age of onset ranged from 2-15 years. Our patients could be classified as having a clinically definite form of the disease. Initial symptoms varied from minor, such as motor or sensory impairment, bladder dysfunction, to the worst clinical presentation, suggesting diffuse encephalopathy. All the patients had a relapsing-remitting course. We report the paraclinical and laboratory examinations that were done in these patients. Over the period 1980 to 1992 these patients had 39 attacks. CSF analysis was performed in the phase of activity of the disease on 23 occasions and was normal in 12. At least one brain CT scan was performed in 9 patients and showed white matter abnormalities in 6. Cranial magnetic resonance imaging was done in 6 patients and were abnormal in 5. Visual evoked potential (EP) was abnormal in 7 of 8 patients; brainstem acoustic EP was abnormal in 4 of 8 patients and somatosensory EP in 4 of 8. MS is not so rare in childhood and although its diagnosis is essentially a clinical one, paraclinical investigations are of great value in the identification of demyelinating disorders in childhood.

[Retinal periphlebitis in multiple sclerosis. Report of a case]. / Periflebite retiniana na esclerose múltipla. Relato de um caso.

Sheathing of peripheral retinal veins occurs in 10 to 20% of patients with multiple sclerosis and can be seen at any point in the progression of the disease. This finding may represent the retinal correlate of the vascular lesions that are present in the central nervous system of patients with multiple sclerosis. Its pathogenesis is still uncertain. In this paper it is registered the occurrence of retinal periphlebitis as one of the initial manifestations of a patient with multiple sclerosis and it is discussed the diagnostic implications of this finding.

[Intracranial tuberculous arachnoiditis: report of a case]. / Aracnoidite tuberculosa intracraniana: registro de um caso.

The case of a 28-year-old male affected by intracranial tuberculous arachnoiditis with unusual aspects is reported. The patient presented bilateral amaurosis and complete ophthalmoplegia with intrinsic muscle sparing in both sides. The routine laboratory findings showed only persistent leucocytosis and a Mantoux test of 10 mm. Several radiologic studies of the lungs were always normal. The spinal fluid, tomography of the cranial base and carotid angiography were normal. The diagnosis was made by histologic study after craniotomy.

[Miller Fisher syndrome. Report of a case]. / Sindrome de Miller Fisher. Relato de um caso.

The case of a man 34 year-old presenting upward gaze paralyses and external ophthalmoplegia without involvement of the downward gaze, accompanied by cerebellar disorders, generalized areflexia but no muscular weakness, and moderate sensory impairment of the four extremities is reported. The cerebrospinal fluid showed an albuminocytologic dissociation. Recovery was rapid and almost complete, within 3 weeks, remaining minimal paresis of the upward gaze and lateral movement in both eyes. The improvement had been coinciding with the treatment by cortrosina.

HTLV-I associated myelopathy in Brazil: a preliminary report.

In this preliminary report the authors present the finding of a high prevalence (37.5%) of seropositivity of antibodies to HTLV-I tested by Western blotting in a sample of 16 Brazilian patients with chronic myelopathies of undetermined origin.

[Vogt-Koyanagi-Harada syndrome: report of 2 cases]. / Síndrome de Vogt-Koyanagi-Harada: relato de dois casos.

The Vogt-Kayanagi-Harada ssyndrome in two negro female patients with 37 and 40 year-old, respectively, are reported. The clinic, epidemiologic, heredo-familial and immunologic aspects are discussed.

[Neurobrucellosis: report of 3 cases]. / Neurobrucelose: relato de tres casos.

Three cases of probable neurobrucellosis are reported. The diagnosis was made on the basis of immunological tests. Two patients with a clinical picture of meningomyelitis showed a definitive clinical improvement under tetracycline and streptomycin therapy. The immunological reactions found in the record case were even more positive in the spinal fluid than in the blood. In the case 3 with a clinical presentation of cerebral hemorrhage the histopathological studies demonstrated non specific chronic leptomeningitis and local hemorrhages in the caudate nucleus bilaterally. The diagnose and treatment of neurobrucellosis are discussed, stressing the importance of an early therapy.

[Familial periodic paralysis: study of 8 cases]. / Paralisia periódica familiar: estudo de oito casos.

The authors studied 8 patients with Familial Periodic Paralysis on the clinical, hydroelectrolytic, electrophysiologic, histologic and therapeutic points of view. There was significant predominance in males. The onset of the symptoms in our group began under fifteen years of age. The clinical manifestations were similar to those referred in the literature. The hypokalemic form was found in all patients except in one case that had hyperkalemic form. Natremias on the superior border of the normality, were seen in 21.4% of the cases, and in 21.4% the natremias were higher; this fact suggested aldosterone liberation of the muscle. The histologic examination performed in 5 patients, was normal in 20% of them and, in those cases with repetitive and frequent crises, atrophic muscle fibers were seen. Prophylactic treatments were made. The patients with hypokalemic form received supplementary doses of potassium.