Functional limitation of the masticatory system in patients with bulbar involvement in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) with bulbar dysfunction affects the motor neurons responsible for controlling the muscles in the jaw, face, soft palate, pharynx, larynx and tongue. This cross-sectional study aimed to determine the functional limitation of the jaw in patients with ALS and bulbar dysfunction who had upper motor neuron (UMN), lower motor neuron (LMN) or balanced involvement. One hundred and fifty-three patients with ALS and 23 controls were included. All participants answered using the 8-item Jaw Functional Limitation Scale (JFLS-8). Patients with ALS were grouped by neurologic examination as follows: non-bulbar ALS, bulbar UMN-predominant ALS; bulbar LMN-predominant ALS; and bulbar balanced (UMN + LMN) ALS. Jaw limitation between the different groups was compared using the Kruskal-Wallis test. Patients with non-bulbar ALS had similar mandibular limitations to healthy participants. Only patients with balanced UMN and LMN bulbar manifestations reported greater difficulties in chewing soft food or in jaw mobility compared to the non-bulbar ALS group. Patients with bulbar involvement also had greater difficulties in chewing tough food or chicken and in swallowing and talking compared to the non-bulbar group, regardless of whether UMN or LMN predominant. No significant differences were found between the groups in smiling and yawning difficulties. Bulbar involvement in patients with ALS is associated with functional limitation of the masticatory system. However, balanced bulbar UMN and LMN involvement is associated with the worst impairments in chewing soft food and in opening the jaw widely.

Experimental Naegleria fowleri meningoencephalitis in sheep: light and electron microscopic studies.

A sheep infected intranasally with Naegleria fowleri of human origin died 7 days after inoculation. The olfactory lobes were distinctly soft and friable. Histologic findings indicated suppurative leptomeningitis and hemorrhagic necrosis in the olfactory lobes. Protozoa were disseminated in the necrotic areas, particularly in perivascular locations; vasculitis was also observed. Meningitis and perivascular cuffing with lymphocytes were evident in the cerebrum, cerebellum, pons, medulla, and cervical spinal cord. Electron microscopy disclosed trophozoites only in the olfactory lobes. The amebae contained a central nucleus with a distinct, electron-dense nucleolus. The cytoplasm contained myelinated figures, lipid-like vacuoles, open vesicles, electron-dense granules, mitochondria, numerous free ribosomes, scant rough endoplasmic reticulum, and occasionally a phagocytized erythrocyte. Trophozoites were grouped close to arterioles, except when phagocytized by a neutrophil or endothelial cell.

A case of keratitis due to Acanthamoeba in New York, New York, and features of 10 cases.

A man in New York, New York, contracted keratitis caused by Acanthamoeba castellanii. The diagnosis was delayed because amoebae were not initially suspected as the infectious organism. The culture isolate and the amoebae in corneal sections were identified as A. castellanii by immunofluorescence using antiserum to plasma membranes of this species. With the rapid agar disk diffusion method, the amoebae were shown to e susceptible to pimaricin (0.5%) and resistant to greater than 1,000-micrograms/ml levels of paromomycin, polymyxin B-bacitracin-neomycin, acriflavine, 5-fluorocytosine, amphotericin B, gentamicin, and trimethoprim-sulfamethoxazole. The infection responded to treatment with pimaricin administered with several other drugs. This infection is the eighth case reported in the literature of acanthamoebic keratitis and emphasizes the need for clinicians to consider acanthamoebic infection in the differential diagnosis of eye infections that fail to respond to bacterial, fungal, and viral therapy.

Primary amoebic meningoencephalitis: a report of two cases and antibiotic and immunologic studies.

In the summer of 1978, two children who had recently been swimming in freshwater lakes in Florida died from primary amoebic meningoencephalitis. Despite early and intensive treatment with amphotericin B, both patients died three to five days after the onset of illness. Amoebae were observed in wet preparations of cerebrospinal fluid and in sections of cerebral tissue and were identified as Naegleria fowleri by the indirect immunofluorescent antibody technique. The amoebae were highly virulent in mice. The isolate of N. fowleri was extremely sensitive in vitro to amphotericin B (minimal inhibitory concentration [MIC], 0.15 microgram/ml), somewhat sensitive to miconazole (MIC, 25 micrograms/ml), and resistant to rifampin (MIC, less than or equal to 100 micrograms/ml). Treatment with amphotericin B (7.5 mg/kg of body weight per day) administered intraperitoneally protected 60% of the mice. Lower doses of amphotericin B alone or in combination with miconazole (100 mg/kg) or rifampin (220 mg/kg) were not protective. These results suggest that amphotericin B remains the single effective agent in treatment of primary amoebic meningoencephalitis.

A fatal case of meningoencephalitis due to a free-living amoeba of uncertain identity--probably acanthamoeba sp.

There are 2 main types of meningoencephalitis caused by free-living amoebae. The first is a well-defined acutely fatal disease resembling fulminating bacterial meningitis. It is caused by the single species Naegleria fowleri. The second is a more poorly defined disease that runs a subacute or chronic course and is characterized by focal granulomatous lesions in the brain. The causative organisms are probably Acanthamoeba sp. in most cases, but it is possible that other genera may be involved. The first case of the subacute form of the disease to be recognized in Australia is described. A 2 1/2-yr-old, previously well girl presented with ataxia and lower motor neurone paralyses. The cerebrospinal fluid was pleocytic and she was thought to be suffering from a relatively minor viral brain-stem encephalitis. Her symptoms persisted in a peculiarly fluctuating way for 30 d when she suddenly collapsed and died from an intracranial haemorrhage. Necropsy showed focal granulomatous lesions associated with necrotizing vasculitis in the basal regions of the brain. The lesions contained well preserved free-living amoebae which were morphologically different from N. fowleri and most closely resembled Acanthamoeba sp. The ultrastructure of the organisms was particularly well preserved and is described in some detail. Immunohistological studies also excluded N. fowleri but were inconclusive for Acanthamoeba or other genera of free-living amoebae. Difficulties with the diagnosis and treatment of this disease are discussed and some practical suggestions are made.

Experimental infection of sheep with Naegleria fowleri of human origin.

A strain of Naegleria fowleri, isolated from a child who died of primary amebic meningoencephalitis in Florida, was instilled in the nostrils of a sheep to determine whether livestock are susceptible to infection with free-living amebae. The animal died 7 days later from amebic infection of the central nervous system. N. fowleri were recovered from the brain and spinal cord of the animal. A control, saline-instilled sheep that had been pair-caged with the infected animal remained healthy.

Keratitis due to Acanthamoeba castellani. A clinicopathologic case report.

Trophozoites and cysts of amebas were found within the necrotic cornea of an enucleated eye. The organism was identified, by indirect immunofluorescent staining using specific antiserum, to be Acanthamoeba castellani. This case report illustrates the difficulty of clinical diagnosis and typical inefficacy of medical therapy shown in other reports of this rare keratitis.

Granulomatous encephalitis, intracranial arteritis, and mycotic aneurysm due to a free-living ameba.

Primary amebic meningoencephalitis and granulomatous amebic encephalitis are well recognized clinicopathological entities caused by free-living amebas. Associated arteritis and "mycotic aneurysms" with infiltration of intracranial arteries by lymphocytes, amebic trophozoites and cysts have not been previously reported. A 26-month-old girl had a 3-week history of encephalitis, characterized, initially, by vomiting and low-grade fever. Subsequently, she developed ataxia, generalized weakness, lethargy, and esotropia. The first CSF showed 490 RBC/microliters, 705 WBC/microliters with 90% mononuclears. Her pupils reacted briskly to light. Moderate nuchal rigidity, nystagmus, fixed downward gaze, anisocoria, bilateral 6th nerve palsy, left arm monoparesis and left Babinski were present. CAT scan revealed slight symmetrical dilatation of anterior horns of lateral ventricles and an area of abnormal enhancement above the 3rd ventricle. She died 14 days after admission, 5 weeks after onset of symptoms. The brain showed focal necrotizing encephalopathy, involving thalami, cerebellum, brain stem, and cervical and upper thoracic spinal cord. Numerous free-living amebic trophozoites and cysts were present within a chronic granulomatous encephalitis. There were trombosis of basilar, posterior cerebral, and vertebral arteries with profuse chronic panarteritis, fibrinoid necrosis, and mycotic aneurysms.

Experimental pneumonitis induced by Naegleria fowleri in mice.

An environmental Naegleria isolate, from a cooling lake receiving thermal additions from an electric power plant, caused both primary amoebic meningoencephalitis (PAM) and pneumonitis in mice after intranasal inoculation. Amoebae, recovered from brains and lungs of mice inoculated with the environmental isolate, produced both brain and lung infections in repeated mouse passages. Electron microscope examination of infected lungs revealed a moderate inflammatory reaction with slight necrosis. Amoebae re-isolated from mice with PAM and pneumonitis were identified as N. fowleri by the indirect immuno-fluorescent antibody technique using anti-N. fowleri serum. The results suggest that in addition to PAM, N. fowleri may be capable of causing subacute and acute respiratory infections in man.

Retrospective identification of Acanthamoeba culbertsoni in a case of amoebic meningoencephalitis.

Acanthamoeba culbertsoni was identified retrospectively in a case of amoebic meningoencephalitis, previously reported by Jager and Stamm (Lancet, 2, 1343, 1972). This is the second report of this species causing secondary infection in man. Positive results were obtained only with anti-A. culbertsoni sera when the brain sections were stained by the indirect immunofluorescence antibody test with various antisera produced against different Acanthamoeba species. Antiserum raised against purified plasma membranes of A. culbertsoni showed once more its highly specific diagnostic value.

Acanthamoeba royreba sp. n. from a human tumor cell culture.

A new species of Acanthamoeba was isolated from a culture of an established line of human choriocarcinoma cells. The identification of this strain, originally called the Oak Ridge strain, and the establishment of a new species for it were based on morphologic, serologic, and immunochemical studies. In general, the structure of the trophozoite did not differ significantly from that of other species of Acanthamoeba, except that a body which more closely resembled a centriole than material described previously as centriolar satellites was observed in trophozoites examined with the electron microscope. The dimensions of the trophozoite were the smallest among the species of Acanthamoeba. The cyst was typical of the genus, but differed from those of other species by its smaller size and the presence of numerous ostioles. Studies of the Oak Ridge strain by immunofluorescence using antisera developed against the isolate and Acanthamoeba culbertsoni, A. castellanii, A. polyphaga, A. rhysodes, A. astronyxis, and A. palestinensis revealed the antigenic uniqueness of the Oak Ridge strain. It was demonstrated by immunoelectrophoretic analyses of the soluble proteins of the Oak Ridge strain that shared approximately 1/2 of its antigenic structure with A. castellanii and A. culbertsoni. The antigenic differences of the isolate from other species of Acanthamoeba were deduced from comparison of the antigenic constitution of these species and the Oak Ridge strain with A. culbertsoni and A. castellanii. Although the strain was initially recognized by its cytopathogenicity for cultures, it did not produce acute infections in mice after intranasal inoculation of 1 X 10(4) ameba/mouse. The foregoing results constituted the basis for the establishment of the Oak Ridge strain as a new species, A. royreba sp. n., in the genus Acanthamoeba.

Isolation of the etiological agent of primary amoebic meningoencephalitis from artifically heated waters.

To determine whether artificial heating of water by power plant discharges facilitates proliferation of the pathogenic free-living amoebae that cause primary amoebic meningoencephalitis, water samples (250 ml) were taken from discharges within 3,000 feet (ca. 914.4 m) of power plants and were processed for amoeba culture. Pathogenic Naegleria fowleri grew out of water samples from two of five lakes and rivers in Florida and from one of eight man-made lakes in Texas. Pathogenic N. fowleri did not grow from water samples taken from cooling towers and control lakes, the latter of which had no associated power plants. The identification of N. fowleri was confirmed by pathogenicity in mice and by indirect immunofluorescence analyses, by using a specific antiserum.

Meningoencephalitis due to Acanthamoeba SP. Pathogenesis and clinico-pathological study.

Amebic Meningoencephalitis (AM) and Primary Amebic Meningoencephalitis (PAM) are infectious diseases essentially confined to the Central Nervous System (CNS) and caused by free-living amebas of the genus Acanthamoeba (A.) and Naegleria (N.) respectively. AM due to A. sp. (Acanthamoeba castellanii and Acanthamoeba culbertsoni) have been reported in chronically ill debilitated individuals, some of them under immunosuppressive therapy, or in immunologically impaired patients without a history of recent swimming in contrast to cases due to N. sp. which usually occurs in healthy, young individuals with a recent history of swimming in man-made lakes or heated swimming pools. AM due to A.sp. is characterized by a subacute or chronic granulomatous meningoencephalitis involving mainly the midbrain, basal areas of the temporal and occipital lobes and posterior fossa structures. CNS lesions in AM are perhaps secondary and the portal of entry in humans is probably from the lower respiratory tract, genitourinary system or skin reaching the CNS by hematogenous spread. The predominant host reaction is usually composed of lymphocytes, plasma cells, monocytes and multinucleated foreign body giant cells. Necrosis is moderate and hemorrhage scant or absent. Cysts as well as trophozoites may be seen within the CNS lesions. PAM is due to Naegleria fowleri and is characterized by an hemorrhagic necrotizing meningoencephalities with an acute inflammatory response. Only trophozoites are found in lesions. The portal of entry is through the olfactory neuroepithelium. CNS tissues fixed in formalin may be used for further identification and taxonomical classification of the causative protoza using immunofluorescent antibody techniques (IFAT) and electron microscopic methods.