COVID-19 and herpes zoster co-infection presenting with trigeminal neuropathy.

BACKGROUND: Varicella-zoster virus (VZV) is a human neurotropic virus that remains in a latent state within ganglionic neurons throughout the entire neuroaxis after the primary infection. When herpes zoster (HZ) leads to trigeminal involvement, the ophthalmic division is the most implicated. COVID-19 has emerged as a viral cause of severe acute respiratory syndrome that has spread all over the world in the last months. Co-infection with COVID-19 and other viruses has been reported, but sparsely, and involving the respiratory viruses. METHODS: The case of a co-infection of COVID-19 with VZV is reported, and the literature reviewed. RESULTS: A 39-year-old immunocompetent man presented with oligosymptomatic infection with COVID-19, which evolved to left facial HZ, affecting the three divisions of the trigeminal nerve. The co-infection was remotely registered, being the respiratory viruses, especially influenza, the most commonly cited association. However, the present case illustrates the emergence of a latent virus infection, which might be favored by the inflammatory response to the former agent (COVID-19). This reaction ascended from the nasal cavity, where trigeminal branches are also placed. CONCLUSIONS: The emergence of latent VZV infection in this rare presentation might illustrate an effect, at least locally, of COVID-19. This virus possibly induced a retrograde reactivation of VZV in a young immunocompetent patient.

Neuroimaging Findings of Zika Virus-Associated Neurologic Complications in Adults.

When the first suspected cases of neurologic disorders associated with the Zika virus were noticed in Brazil in late 2015, several studies had been conducted to understand the pathophysiology of the disease and its associated complications. In addition to its well-established association with microcephaly in neonates, the Zika virus infection has also been suggested to trigger other severe neurologic complications in adults, such as Guillain-Barré syndrome, radiculomyelitis, and meningoencephalitis. Hence, the Zika virus should be deemed a global threat that can cause devastating neurologic complications among individuals in all age ranges. The aim of this review was to further describe neuroimaging findings of Zika virus infection and associated neurologic complications found in adults.

Benign monomelic amyotrophy: a study of twenty-one cases.

A consecutive series of 21 patients with single limb atrophy (monomelic amyotrophy) is reported. Sixteen had lower limb atrophy and five had upper limb involvement. The median age of the onset was 20 years. Characteristic features were sporadic occurrence, wasting confined to one limb, insidious onset with slow progression, stabilizing in 1 to 4 years, and absence of pyramidal signs. All the patients with upper limb involvement were male, however in our cases with lower limb amyotrophy there were no male preponderance. We observed wasting of the entire length of the lower limbs in six patients. There were nine cases with amyotrophy restricted to the leg and one with amyotrophy only in the thigh. In the upper limb in four cases the involvement was distal and in one patient the atrophy was proximal. The electromyographic features were suggestive of anterior horn disease not only in the affected limb but also, in some cases, in clinically uninvolved limb. Cervical or lumbar MRI was normal. MRI of the lower limb disclosed increased signal intensity in the gastrocnemius and soleus muscles in one patient suggesting denervation.

Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease): a clinical variant of the benign monomelic amyotrophy.

Hirayama's disease (HD) is frequently found in Asia, and is rarely referred among westerners. It affects young people with higher incidence in males. It is a focal distal amyotrophy with unilateral or asymmetric bilateral involvement of C7, C8 and T1 innervated muscles. HD appears sporadically and has a benign evolution with clinical stabilization in around one year. We report four young male patients with clinical and electrophysiological alterations described in HD, which were followed-up during 5 years. Electromyographic findings were indicative of lower motor neuron involvement. We analyzed cervical MRI aiming at understanding if a questionable spinal cord compression could be implicated in the pathogenesis, but no abnormality was verified. In view of its clinical, and EMG characteristics, HD is no more than a benign monomelic amyotrophy (BMA) clinical variant, and not a specific disease. This eponym could be considered only for the distal upper limb variant (Hirayama's variant) of the BMA.

Distal sensory polyneuropathy in a cohort of HIV-infected children over five years of age.

BACKGROUND: Peripheral neuropathy in children with human immunodeficiency virus (HIV) infection has not been systematically studied. Objectives. To describe the symptoms and signs of peripheral neuropathy in HIV-infected children and to determine their frequency. METHODS: A cross-sectional study was conducted on a convenience sample from a cohort of children older than 5 years of age at the pediatric HIV outpatient clinic of the Federal University of Rio de Janeiro. Those patients were interviewed and examined systematically for peripheral nerve symptoms and signs. RESULTS: A total of 39 patients were clinically evaluated. Their ages ranged from 5 to 14 years, and 13 patients (34%) had symptoms and signs of peripheral nerve involvement. Distal paresthesia and/or pain plus diminished ankle jerks and/or diminished vibration sense were the most common clinical findings. Symptoms were chronic and fluctuating, and pain was, in general, not severe. Nerve conduction studies primarily revealed axonal changes. CONCLUSIONS: Peripheral neuropathy occurs in one third of HIV-infected children, and, in general, has less severe features than the distal sensory polyneuropathy described in adults. peripheral neuropathy, human immunodeficiency virus, children.

[Critical illness polyneuropathy: case report]. / Polineuropatia do paciente crítico. Registro de caso.

The critical illness polyneuropathy has an acute onset and an axonal predominantly motor nature. It occurs in sepsis or in multiple organ failure usually requiring mechanical ventilation in critical care units. Electroneuromyography corroborates the diagnosis. Usually it courses satisfactorily. We report on a 35-year-old female patient who, after a permanence in a critical care unit due to sepsis and removal of a dead phetus, developed tetraparesis. She had an important improvement in four months. Electromyography showed reduction of amplitude of motor and sensory action potentials, positive waves and fibrillations. The sural nerve biopsy showed axonmyelinic neuropathy. These findings are consistent with those in literature and we believe they support the diagnosis of critical illness neuropathy.

[Ulnar nerve palsy in leprosy without skin changes: biopsy of the superficial branch of the ulnar nerve in the hand]. / Paralisia do nervo ulnar na lepra sem alterações cutâneas. Biópsia do ramo superficial do nervo ulnar na mão.

Leprosy is one of the most common diseases of peripheral nerves in the world. In Brazil it is particularly frequent, being a major health problem. In tuberculoid leprosy the ulnar nerve is the most common affected nerve. Sometimes there are no skin changes. In these cases in spite of nerve thickening only the nerve biopsy is capable to make a specific diagnosis. We performed a biopsy in the dorsal sensory branch of the ulnar nerve in the hand in 17 patients with ulnar palsy with thickening of the nerve in the elbow, without skin changes. The pathological findings consisted mainly of: loss of fibers (14 cases), inflammatory infiltration (13), fibrosis (12), demyelination and remyelination (9), presence of granuloma (6) and presence of bacilli (5 cases). We conclude that in case of ulnar nerve palsy in leprosy without skin changes, the biopsy of the dorsal sensory branch of this nerve in the hand is a good procedure for the diagnosis of leprosy.

[Study of ultrastructural alterations in gastrocnemius muscle of C57 BL10 mice after prolonged ethanol ingestion]. / Estudo morfológico no músculo gastrocnêmio de camundongos C57 BL10 submetidos à ingestão prolongada de etanol.

The effects of chronic alcoholism on gastrocnemius muscle of well-nourished mice were morphologically studied to test the direct toxic role of ethanol on skeletal muscle. Thirty male young adult C57BL10 mice were divided in two groups: Group A (control) consisting of ten mice that drank water and Group B (alcoholic) consisting of twenty mice that drank 25% ethanol. All mice were allowed a balanced laboratory chow. The animals were kept on this ad libitum regimen under the same conditions of environment for 48 weeks and were weighed once a week. The daily dietary consumption and caloric intake were estimated, the animals having had a substantial weight gain, showing no signs of malnutrition. At the end of the experiment the animals were killed for morphological studies. No abnormalities were observed by conventional microscopy. Striking deviations from normal were verified by electron microscopy in all specimens. Dilatation of sarcoplasmic reticulum was a common feature, sometimes resulting in the formation of large vesicles and involving the terminal cisternae with the displacement of the triads. Areas of narrowing, splitting and loss of myofibrils were seen. Zones of complete disorganization of miofibrils could be occasionally observed. Mitochondria were generally normal. Peculiar tubular aggregates seen commonly in periodic paralysis and other human pathological conditions, were encountered in both control and alcoholic mice. Intramuscular nerves and neuromuscular junctions were normal. Important abnormalities in muscle capillaries were observed, consisting of swelling of endothelial cells and thickening of the basal lamina. A diffuse microvesicular lipid infiltration was seen in the cytoplasm of the hepatocytes which seems to be a further evidence of the toxic role played by ethanol. We concluded that prolonged ingestion of ethanol, representing 14.4% of total calories, produces in the gastrocnemius muscle of well-nourished C57BL10 mice a distinct spectrum of ultrastructural changes which reflects a direct toxic effect on the skeletal muscle. These alterations are similar to those described in human chronic alcoholic myopathy.

[Sural nerve biopsy in myotonic muscular dystrophy]. / Biópsia do nervo sural na distrofia muscular miotônica.

Twelve patients with myotonic dystrophy were studied to look for the involvement of the peripheral nervous system in this disease. All of them showed the main signs and symptoms of the disease. They did not have another causes to justify a polineuropathy. They were submitted to sural nerve biopsy with counting of myelinated fibers and histogram. Patients showed a reduction in the number of myelinated fibers and in two patients the histogram was unimodal. We concluded that polineuropathy may be another multisystemic manifestation of myotonic dystrophy.

[Charcot-Marie-Tooth disease. Clinical study in 45 patients]. / Doença de Charcot-Marie-Tooth. Estudo clínico em 45 pacientes.

Charcot-Marie-Tooth (CMT) disease is the commonest inherited peripheral neuropathy. The clinical study of 45 patients with CMT is presented. They were derived from Antonio Pedro Hospital of Universidade Federal Fluminense in Niteroi, RJ, Brazil. Such patients could be divided by the motor conduction velocity in two types: a demyelinating form or type I (11 cases) and an axonal form or type II (34 cases). The disease was inherited as an autosomal dominant trait in 23 patients and as an autosomal recessive trait in 7 cases. In 15 patients the disorder was sporadic. The age of onset was in most of our cases before the 20 years. All of them had distal weakness in lower limbs. 38.2% had also distal weakness in upper limbs. 80% had distal wasting of the lower limbs and 50% had distal wasting of upper limbs. The tendon reflexes were absent in 64% in lower limbs and in 28% in upper limbs. The sensitive impairment in the distal regions of the extremities was mild in most patients. We found enlargement of peripheral nerves in 7 patients of type I. Pes cavus was present in 21 cases and scoliosis in 7. We found postural tremor of hands in 6 patients. In 9 cases there were rare features as mental retardation, trigeminal nevralgia, optic atrophy, deafness and calf enlargement. In most of our cases the clinical course was very slow progressive. A greater severity was seen in our sporadic cases.

[Charcot-Marie-Tooth disease. Electromyographic studies in 45 patients]. / Doença de Charcot-Marie-Tooth. Estudos eletromiográficos em 45 pacientes.

The electrophysiological studies of 45 patients with Charcot-Marie-Tooth disease (CMT) are presented. The nerve conduction of the motor median and ulnar nerves permitted us to separate our patients in two types: type I (demyelinating) with motor nerve conduction (MNC) below 38 m/s (11 cases) and type II with MNC normal or above 38 m/s (34 cases). In type I there was no correlation between reduction in MNC and clinical severity. It was not possible to classify the disease on the sural nerve sensory action potential (SAP). They were unobtainable in most cases. In many patients with CMT type II the MNC was normal. In the cases the sural SAP was absent or reduced. We concluded that the MNC study is the best useful test to classify CMT disease in type I and type II.

[Charcot-Marie-Tooth disease. Study of sural nerve biopsy in 41 patients]. / Doença de Charcot-Marie-Tooth. Estudo da biópsia do nervo sural em 41 pacientes.

We studied the pathological findings of sural nerve biopsy in 41 patients with Charcot-Marie-Tooth (CMT) disease. They were previously classified by the median motor conduction velocity (MCV) in two types. Type I (demyelinating) with 9 patients and type II (axonal) with 32 cases. In type I we found loss of myelinated fibers (unimodal histogram), demyelinated and remyelinated axons with numerous onion bulb formations. In one case there was thickness of myelin with thin axons (axonal atrophy). In type II there were seven patients with normal sural nerve biopsy. In 25 cases there were mild to severe loss of myelinated fibers. In 5 patients the number of myelinated fibers was increased due to the great regeneration of the axons. The electron microscopic studies in type II showed in a few cases small onion bulbs and in one case axonal atrophy. In type I there was no correlation between clinical severity and the loss of myelinated fibers, but there was relationship between the low MCV and the intensity of myelinated fibers. In type II we did not found any correlation between clinical course, MCV and pathological findings.

Trophic ulcers in the carpal tunnel syndrome.

A patient with carpal tunnel syndrome (CTS) and trophic ulcers is described. Despite the healing of the ulcers after surgery for CTS, the severe sensory deficit and the electrophysiological tests have not shown any significant improvement. We think these findings argue against the hypothesis of the sensory deficit being responsible for the trophic ulcers. We favor a major role for the sympathetic disturbances as the main cause for those lesions.

Clinical and morphological study of calf enlargement following S-1 radiculopathy.

Calf enlargement following sciatica is a rare condition. It is reported the case of a 28-year-old woman who complained of repeated episodes of lower back pain radiating into the left buttock and foot. One year after the beginning of her symptoms, she noticed enlargement of her left calf. X-ray studies disclosed L5-S1 disk degeneration. EMG showed muscle denervation with normal motor conduction velocity. Open biopsies of the gastrocnemius muscles were performed. The left gastrocnemius muscle showed hypertrophic type 2 fibers in comparison with the right gastrocnemius. Electron microscopy showed mildly increased number of mitochondria in these fibers. A satisfactory explanation for denervation hypertrophy has yet to be provided.

[Polyneuropathy caused by ethylene oxide. Report of a case with clinical, electrophysiological and histopathological studies]. / Polineuropatia devida ao óxido de etileno. Registro de caso com estudo clinico, eletrofisiológico e histopatológico.

A man who worked as an operator in a factory of sterilization of heat-sensitive materials has been exposed to ethylene oxide for seven years. He developed a mild sensori-motor polyneuropathy. The electromyography and nerve condition studies showed an axonal degenerative type of neuropathy. The sural nerve biopsy revealed mild loss of myelinated fibers, some fibers with axonal degeneration, some clusters of regeneration and few rows of myelin ovoids in the teased nerve fiber preparation. This is the first report of ethylene-oxide polyneuropathy in Brazil.

Tapia's syndrome caused by Paracoccidioidis brasiliensis.

Tapia's syndrome is due to extracranial involvement of the XIIth nerve and the recurrent laryngeal branch of the Xth nerve. There is ipsilateral paralysis of vocal cords, soft palate and tongue. The main causes are parotid and other tumors or injuries to the high neck. We describe here a Brazilian female patient who presented with a lesion in the nasal mucosa, and soon after had dysphagia and dysphonia. Examination revealed paralysis of the soft palate, vocal cords and tongue ipsilaterally. Microscopic examination of the lesion in the nasal mucosa revealed the presence of the fungus. The patient was treated with sulfonamide and ketoconasol.

[Axonal polyneuropathy in Chagas disease]. / Polyneuropathie axonale dans la maladie de Chagas.

We report the case of a 44-year-old woman presenting with chronic symmetrical, sensitive polyneuropathy on the lower limbs in the course of Chagas' disease. The electrophysiological findings were in keeping with axonal degeneration. The histological data displayed axonal degeneration with perivascular inflammatory mononuclear cells in the epineurium, with some neutrophils and eosinophils. Mononuclear cells surrounding endoneurial vessels were observed. Laboratory data did not suggest neither a mixed connective tissue disease nor a collagen vascular disorder. Hematological disease, malignancies, drug-or medicine-induced neuropathy were ruled out. The polyneuropathy in this case was probably related to Trypanosoma cruzi infection on account of the presence of high levels of anti-T. cruzi antibodies, and an immune mechanism might play a role in the vasculitic process.