BACKGROUND:Amyotrophic lateral sclerosis (ALS) is a devastating disease leading to death within 3-5 years in most cases. New approaches to treating this disease are needed. Here, we report a successful therapy.

CASE REPORT:In a 49-year-old male patient suffering from muscle weakness and fasciculations, progressive muscular atrophy, a variant of ALS, was diagnosed after extensive examinations ruling out other diseases. Due to supposed mercury exposure from residual amalgam, the patient's teeth were restored. Then, the patient received sodium 2,3-dimercaptopropanesulfate (DMPS; overall 86× 250 mg in 3 years) in combination with α-lipoic acid and followed by selenium. In addition, he took vitamins and micronutrients and kept a vegetarian diet. The excretion of metals was monitored in the urine. The success of the therapy was followed by scoring muscle weakness and fasciculations and finally by electromyography (EMG) of the affected muscles. First improvements occurred after the dental restorations. Two months after starting therapy with DMPS, the mercury level in the urine was increased (248.4 µg/g creatinine). After 1.5 years, EMG confirmed the absence of typical signs of ALS. In the course of 3 years, the patient recovered completely.

CONCLUSIONS:The therapy described here is a promising approach to treating some kinds of motor neuron disease and merits further evaluation in rigorous trials.

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We present four incidental cases that developed partial myelitis following the administration of hepatitis B vaccine in 1998. The first two cases, a 33-year-old man and a 42-year-old woman developed progressive sensory symptoms without motor involvement within 4 weeks following the vaccination. Their magnetic resonance imaging (MRI) disclosed similar lesions consistent with myelitis at their cervical spinal cord. A comparable inflammatory lesion was seen at the T9-T10 levels of the spinal cord in the third case, who was a 40-year-old woman presenting with numbness in her legs and urinary retention following the vaccination. The fourth case who was a 42-year-old woman, presented with sensory symptoms in her left extremities, which developed 3 months after the vaccination. Her MRI showed a hyperintense lesion at C6. She also had two tiny lesions in her cranial MRI. In all cases, there was no history of preceding infections and no clinical evidence suggestive of any other disorders that may cause myelopathy. All patients recovered completely within 3 months with the exception of the third patient who developed new neurological symptoms after 12 months. Similar clinical and imaging presentation of myelitis following hepatitis B vaccination within a 1 year period with no other demonstrable clinical and laboratory evidence for any other disorder raise the probability of a causal link between these two events.

A healthy 5 year old girl developed herpes zoster in the dermatome supplied by the ophthalmic branch of the fifth cranial nerve 40 months after varicella vaccination. She was admitted to our hospital because of high fever and painful vesicular lesions over the left side of her forehead. She was treated successfully with systemic and topical acyclovir without developing herpetic keratoconjunctivitis. Our acute and convalescent phase evaluations showed that non-specific cellular and humoral immunity was normal. This is the fourth case of herpes zoster developing in an immunocompetent child following vaccination. Unlike the previously reported cases, our patient required hospitalization mainly to prevent ocular involvement. The issue concerning whether the universal introduction of varicella vaccination of normal children will reduce the incidence of the subsequent occurrence of herpes zoster must await further studies involving longer follow-up periods.

PURPOSE:In this study, the case of a healthy pediatric patient who presented with herpes zoster (HZ) stromal keratitis after vaccination with live attenuated varicella vaccine (Varivax) and subsequent booster is described.

METHOD:This is a retrospective case review.

RESULTS:A 6-year-old girl with no medical history presented with HZ ophthalmicus and stromal keratitis. She had received the original Varivax vaccine at 1 year of age and a booster 1 year before presentation. Topical prednisolone acetate was started with subsequent improvement in inflammation and visual acuity. However, the patient was unable to be completely tapered off the steroids because of reactivation.

CONCLUSIONS:HZ ophthalmicus with stromal keratitis is a rare but potentially damaging manifestation of the varicella zoster virus in the pediatric population. Long-term data regarding reactivation rates in the post-vaccination era are still limited. Close follow-up is needed to ensure resolution of the infiltrates, and reactivation may require long-term steroid therapy.

PURPOSE:To report a case of herpes zoster virus sclerokeratitis with anterior uveitis following vaccination with live attenuated varicella vaccine (Oka strain).

DESIGN:Case report.

METHODS:The case records of the patient were reviewed retrospectively. Pertinent literature citations were identified using MEDLINE.

RESULTS:A 9-year-old boy presented with herpes zoster ophthalmicus 3 years following vaccination with live attenuated varicella vaccine (Oka strain). Examination of the affected eye revealed a moderate follicular response on the palpebral conjunctiva, decreased corneal sensation, mildly elevated intraocular pressure, diffuse anterior scleritis with marginal keratitis, and a moderately severe anterior uveitis. Amplified DNA from fluid taken from the base of a cutaneous vesicle produced wild-type varicella zoster virus (VZV) DNA, not Oka strain.

CONCLUSIONS:Herpes zoster virus infection needs to be considered in all patients who present with scleritis, keratitis, or anterior uveitis, regardless of their varicella vaccination status.

AIM: Find an association between hepatitis B vaccine-related systemic lupus erythematosus and HLA. Material: A 27-year-old woman who developed a lupus nephritis after the administration of hepatitis B vaccine.

METHODS: We studied HLA antigen expression on lymphocytes and genomic haplotype. Class I-II HLA antigen typing was performed by the microlymphocytotoxicity test with the standard NIH method, and Class I-II HLA allele typing by polymerase chain reaction, using single-strand oligonucleotide dot-blot kits.

RESULTS: The serological haplotype was HLA A24/25, B18 (Bw6)/-, C-/-, DQ7/-, DR11(5)/52. The genomic haplotype was A*2403/2504, B*1825/1825, C*1207/ 1207, DRB1*1102/1132, DRB3*0202/0202, DQA1*0505/0505, DQB1*0301/0301. Then we sought for analogies with haplotypes known to be related to other systemic AID. Since we have found HLA alleles typical both of systemic lupus erythematosus and Sjogren's syndrome, the persistence of ENA-SSA positivity was highly suspicious for a possible overlap syndrome.

CONCLUSIONS: Hepatitis B vaccine can potentially trigger both the onset or the exacerbations of several autoimmune disorders, including systemic lupus erythematosus, by reduced immune complex clearance or molecular mimicry. This study represents the first report on the association between hepatitis B vaccine related systemic lupus erythematosus and HLA. Probably autoimmune reactions triggered by vaccines occur only in predisposed subjects, in which antigen presentation influenced by HLA haplotypes leads to the autoimmune cascade. More studies are needed to corroborate our hypothesis. They could disclose new pathways in the field of prevention.

PROBLEM:Post-vaccination autoimmune phenomena are a major facet of the autoimmune/inflammatory syndrome induced by adjuvants (ASIA) and different vaccines, including HPV, have been identified as possible causes.

METHOD OF STUDY:The medical history of three young women who presented with secondary amenorrhea following HPV vaccination was collected. Data regarding type of vaccine, number of vaccination, personal, clinical and serological features, as well as response to treatments were analyzed.

RESULTS:All three patients developed secondary amenorrhea following HPV vaccinations, which did not resolve upon treatment with hormone replacement therapies. In all three cases sexual development was normal and genetic screen revealed no pertinent abnormalities (i.e., Turner's syndrome, Fragile X test were all negative). Serological evaluations showed low levels of estradiol and increased FSH and LH and in two cases, specific auto-antibodies were detected (antiovarian and anti thyroid), suggesting that the HPV vaccine triggered an autoimmune response. Pelvic ultrasound did not reveal any abnormalities in any of the three cases. All three patients experienced a range of common non-specific post-vaccine symptoms including nausea, headache, sleep disturbances, arthralgia and a range of cognitive and psychiatric disturbances. According to these clinical features, a diagnosis of primary ovarian failure (POF) was determined which also fulfilled the required criteria for the ASIA syndrome.

CONCLUSION:We documented here the evidence of the potential of the HPV vaccine to trigger a life-disabling autoimmune condition. The increasing number of similar reports of post HPV vaccine-linked autoimmunity and the uncertainty of long-term clinical benefits of HPV vaccination are a matter of public health that warrants further rigorous inquiry.

To investigate the association between human papillomavirus (HPV) vaccination and autoimmune manifestations compatible with systemic lupus erythematosus (SLE) or SLE-like disease, the medical history of six women who presented with SLE or SLE-like disease following HPV immunization was collected. Data regarding type of vaccine, number of immunization, family and personal, clinical and serological features, as well as response to treatments were analyzed. In the reported cases, several common features were observed, such as personal or familial susceptibility to autoimmunity or adverse response to a prior dose of the vaccine, both of which may be associated with a higher risk of post-vaccination autoimmunity. Favorable response to immunosuppressant was observed in all patients. In the current study, a temporal association between immunization with HPV vaccine and the appearance of a spectrum of SLE-like conditions is reported. Additionally, among the patients described, several common features were observed that may enable better identification of subjects at risk. Further studies are required to assess the safety of immunization with the HPV vaccine in patients with autoimmune-rheumatic diseases or in subject at risk of autoimmunity as well as the potential beneficial effect of preventive immunosuppressants.

A 17-year-old girl reported generalised urticaria, eyelid angioedema, rhino-conjunctivitis, dyspnoea and wheezing 1 h after third intramuscular administration of quadrivalent human papilloma virus vaccine (Gardasil). She was treated with antihistamine, and corticosteroids with prompt relief of rhinitis and dyspnoea, while urticaria and angioedema lasted 24 h. Intradermal test with Gardasil, which contains polysorbate 80 (PS80), resulted positive, while skin tests with the bivalent vaccine were negative. Prick test performed with PS80 resulted positive in the patient and negative in ten healthy controls. The CD203 basophil activation test result was negative for PS80 at all the tested dilutions and specific IgE was not found. As flu vaccine was recommended, the authors skin tested two flu vaccine, one containing PS80 (Fluarix, GSK), which resulted positive and another flu vaccine with no adjuvant or preservative (Vaxigrip, Sanofi Pasteur MSD), which gave negative results. The patient then received Vaxigrip without adverse reactions.

INTRODUCTION:Hypotonic-Hyporesponsive Episode (HHE) is an adverse event after vaccination, mainly associated with whole-cell pertussis vaccines. It is characterized by a sudden onset of muscle flaccidity, reduced response to stimuli and pallor or cyanosis. Although the HHE is infrequent, it is considered a severe adverse event.

OBJECTIVE:To report a case of HHE following the administration of the whole-cell pertussis combination vaccine (DTwP-HB-Hib), which is included in National Im munization Program (PNI) of Chile, and to contributing to the knowledge of this adverse event in the country.

CASE REPORT:A 6-month-old infant, 3 hours post-vaccination with the third dose of DTwP-HB-Hib vaccine, presented a decreased level of consciousness that was interpreted as atonic seizure but finally considered as EHH. The infant progressed favorably after 2 hours of clinical observation and was discharged 24 hours later. Parents were suggested to continue the immunization schedule of the infant with acellular pertussis vaccines as a preventive measure.

CONCLUSIONS:The lack of knowledge about the EHH may discourage childhood immunization. Therefore, it is important for the medical staff to inform parents of the patients about this benign, self-limited and non-recurrent adverse event. In these cases, it is recommended to continue the immunization schedule of the infant with acellular pertussis vaccines.

A 7-month-old boy became difficult to arouse, was limp and had blue extremities 8 hours after immunization with intravenous poliovirus, diphtheria-tetanus toxoids-acellular pertussis, Haemophilus influenzae type b-hepatitis B virus and pneumococcal vaccines. The hypotonic-hyporesponsive episode had resolved by the time the infant was seen in an emergency department 1 hour later. The report describes hypotonic-hyporesponsive episode, encourages reporting of vaccine-associated adverse events and discusses prognosis and implications for subsequent immunization.

Three case studies of postvaccinal parkinsonism (PVP) demonstrated signs and symptoms identical to conventional diagnostic standards of idiopathic Parkinson's disease (PD). PVP is a sub-type of acute disseminated encephalomyelitis (ADE) that also includes postinfectious parkinsonism (PIP) and postinfectious encephalomyelitis (PIE). All ADE has a unitary pathology consisting of monophasic perivenous inflammation followed by demyelination compared with PD in which Lewy bodies are present in only 75% of studies. We hypothesize that: (1) The seminal event in PD is latent viral invasion emanating from cranial and dorsal root ganglia. (2) Viruses intermittently invade and damage neuropigmented cells secondary to perivenous demyelination. This may explain the numerous clinical and pathological manifestations of PD. Evidence is presented that this pathoetiology probably accumulates subclinically over a long timespan prior to Levy body formation and presentation of clinical signs. This hypothesis has key features similar to one previously published that will be summarized concerning multiple sclerosis.

The hepatitis B virus (HBV) is an important international cause of infectious acute and chronic liver diseases. HBV vaccines were developed to combat the potential life-threatening effects of HBV infection. Published case histories, retrospective reviews and analyses of epidemiological data report on the onset of immune-mediated myelitis after recombinant HBV vaccination, mostly in adults with a presumed genetic/immunologic predisposition. However, HBV vaccination has not borne out to be a significant trigger of serious autoimmune events, including acute myelitis, in populations at large over prolonged observation periods after immunization. Published study methods lack the sensitivity to categorically establish a causal relationship between exposure to HBV vaccine components and immune mediated myelitis, but in practice the faint possibility of such a link should not be totally rejected.

Vaccination of children with aluminium sensitisation against diphtheria and tetanus presents a problem, since vaccine without aluminium against these potentially serious infectious diseases is no longer available. This case report presents a 7-month-old boy who, after his second immunisation with DTaP-IPV, developed an itching injection site nodule. Before being tested for aluminium sensitisation, he was vaccinated again with DTaP-IPV without developing any reactions. This third vaccine dose was administered intramuscularly.

Paralytic poliomyelitis is rare in the United States because of the success of universal childhood immunization and the Global Polio Eradication Initiative. Poliovirus vaccine was introduced in the 1950s. Since then, the United States has eliminated indigenous wild poliovirus transmission, controlled imported wild poliovirus cases, and, through a vaccine policy change (i.e., from live, attenuated oral polio vaccine [OPV] to inactivated polio vaccine [IPV]), eliminated vaccine-associated paralytic polio (VAPP) cases. The most recent VAPP case occurred in 1999. The primary risk for paralytic polio for U.S. residents is through travel to countries where polio remains endemic or where polio outbreaks are occurring. This report describes the first known occurrence of imported VAPP in an unvaccinated U.S. adult who traveled abroad, where she likely was exposed through contact with an infant recently vaccinated with OPV. This case highlights the previously unrecognized risk for paralytic polio among unvaccinated persons exposed to OPV during travel abroad.

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BACKGROUND:The first chiropractic adjustment given in 1895 was reported to have cured deafness. This study examined the effects of a single, initial chiropractic visit on the central nervous system by documenting clinical changes of audiometry in patients after chiropractic care.

CASE PRESENTATION:Fifteen patients are presented (9 male, 6 female) with a mean age of 54.3 (range 34-71). A Welch Allyn AudioScope 3 was used to screen frequencies of 1000, 2000, 4000 and 500 Hz respectively at three standard decibel levels 20 decibels (dB), 25 dB and 40 dB, respectively, before and immediately after the first chiropractic intervention. Several criteria were used to determine hearing impairment. Ventry&Weinstein criteria of missing one or more tones in either ear at 40 dB and Speech-frequency criteria of missing one or more tones in either ear at 25 dB. All patients were classified as hearing impaired though greater on the right. At 40 dB using the Ventry&Weinstein criteria, 6 had hearing restored, 7 improved and 2 had no change. At 25 dB using the Speech-frequency criteria, none were restored, 11 improved, 4 had no change and 3 missed a tone.

CONCLUSION:A percentage of patients presenting to the chiropractor have a mild to moderate hearing loss, most notably in the right ear. The clinical progress documented in this report suggests that manipulation delivered to the neuromusculoskeletal system may create central plastic changes in the auditory system.

Epidermolysis bullosa (EB) is a rare inherited genetic disease characterized by an abnormal response of the skin and mucosa to mechanical trauma. Dystrophic EB (DEB) is very often associated with many extra cutaneous complications. Those complications involve either epithelial associated tissues or other organs. In particular, several renal complications have been described for DEB in the recessive form, such as amyloidosis, post-infection glomerulonephritis, upper and lower urinary tract obstruction and IgA-Nephropathy (IgAN). In the cases reported below we have two patients diagnosed with DEB that showed compromised renal function and proteinuria. The switch of the normal diet toward a gluten free diet resulted beneficial for both patients, since renal function was rescued and proteinuria cured. Moreover, a general health status improvement was recognised, given that nutritional condition was ameliorated and bone growing enhanced. Furthermore, in both patients the presence of autoantibodies anti-COL7 indicating an autoimmune form of the disease. Therefore, patients received low doses of betametasone useful to reduce inflammatory state and to control immune system function. In conclusion, our results prompt us to hypothesized that in these patients, due to the fragility of the intestinal mucosa, the absence in the diet of gluten may be beneficial.