Juniper Publishers Journal of Case Studies is an international, peer reviewed open access journal, focussed on bringing out premium quality case reports in all areas of basic medical sciences and clinical specialities. ‘JOJCS’ aims to serve as a repository of medical case reports that can go a long way in enriching the professional knowledge of medical practitioners around the world.
The rural woman in a developing country bears the family’s load. The recourse when sickness strikes is to a rural hospital, especially a Missionary one. This case illustrates the enormity of the possible diseases, the combination here being ovarian serous cystadenoma, torsion and pyogenic salpingitis with schistosomiasis. Salpingo ophorectomy led to uneventful recovery.
Of late years, the British scene was clouded by those disparaging histology reports required by distant hospitals [1]. Elsewhere [2], one of us examined the falsity of this issue with reference to the developing community known as the Igbos of Nigeria [3]. Accordingly, a case is presented showing the variety of the abdominal conditions met in a young woman for whom salpingo ophorectomy was the answer from the junior author (DT) in the hinterland Mater Hospital, Afikpo. The interconnected etiologic elements were unraveled by the senior author (WO) at a Reference Pathology Laboratory situated in the Capital City, Enugu, Nigeria.
EC, a 25-year-old para 1 patient, came to Mater Hospital, Afikpo. Menses was normal. She was experiencing severe abdominal pain and constipation for 3 days running. On examination by the junior author (DT), this revealed a tender mass in the left iliac fossa. Hence, acute abdomen due to organ torsion was diagnosed. Laparotomy revealed both torsion and infection. Peritonitis was apparent and the omentum was adherent in the pelvis. The enlarged torsed left ovary was situated in the Pouch of Douglas. The uterus appeared normal. Therefore, salpingo ophorectomy was performed. Her recovery was uneventful.
The specimens were examined at the Reference Pathology Laboratory at Enugu, the Regional Capital City. In the main, there was a 14 x 10 x 5cm cystic mass, which was largely smooth surfaced and was attached to a 2.5cm tube measuring 10cm long. This tube was distended in parts with gelatinous and greenish purulent looking matter. On section, the cyst contained watery brownish fluid. Its wall was paper thin for the most part. Microscopy revealed serous cystadenoma, acute salpingitis and the ovarian typical ova of Schistosoma haematobium. See Figure 1.
The serous cystadenoma is an interesting lesion of the ovary [4]. It has been viewed from such angles as hugeness in not only an adolescent girl [5] and a young woman [6] but also in a postmenopausal woman [7]. Its striking associations have been recorded with fibrothecoma [8], fibroma [9], and contralateral ovary which was torsed [10].
The above cases were reported from India, Turkey and Iran. Therefore, our case stands out from Nigeria in exhibiting the combination with not only torsion and salpingitis but also schistosomiasis. This particular worm infestation has been reported locally as regards the appendix [11] and urinary bladder [12,13].
Our case merits documentation as regards ovarian serous cystadenoma and its associated lesions. However, concerning the advancing edge of science, it suffices to exemplify with molecular genetic analysis [14].
On a mundane note, this paper exemplifies the work done by Missionary Physicians in the hinterlands of a developing community. In this context, co-authorship was affected earlier concerning such topics as schistosomiasis in ovarian thecoma [15], carcinoma-in-situ of the vagina [16], uterine procidentia [17], and abdominal paragonimiasis [18].
In the final analysis, Birmingham (UK) authors emphasized that the establishment of a histopathology data pool facilitates epidemiological analysis [19]. Their concept was verified in the present paper which depended on such a data pool in a developing community.
Background:Guillain-Barre Syndrome is a severe demyelinating polyneuropathy associated with multiple phenotypic variants including Miller-Fisher and Bickerstaff’s Encephalitis. The aim of this article is to outline the clinical characteristics of these variants, as well as the utilization of diagnostic autoantibodies to determine clinical course, treatment and prognosis of those with GBS.
A 45-year-old morbidly obese female with no significant past medical history presented to the emergency department with a two-day history of progressively worsening double vision, bilateral ptosis, and retro-orbital headache. She had been in her normal state of health with the notable exception of an upper respiratory infection and a persistent dry hacking cough that began approximately two weeks prior to presenting to the emergency department.The patient also noted a change in her voice, and difficulty swallowing solids, although she had attributed this to her viral illness. She was unable to focus on the computer screen at work due to the progressive double vision. Her visual disturbances were specifically described as horizontal double vision, with no change by near or distant vision. She otherwise denied any weakness, sensory changes, or unsteady gait.
Presenting physical examination was notable for multiple cranial nerve abnormalities. Her Pupils were dilated to 6mm bilaterally and unreactive to light bilaterally. Extraocular movements revealed bilateral abduction and vertical gaze deficits. She also exhibited bilateral ptosis which was more severe on the left, as well as a faint left facial droop. Several hours after arrival, the patient rapidly deteriorated. She was noted to have a more dysphonic voice and complete eyelid paresis. She also developed a slight ataxic gait. Her muscle strength was preserved, and deep tendon reflexes remained intact throughout. Her mental status was not affected to the most part.
Admission Computerized tomography (CT) of the head revealed questionable hypodensity in the midbrain (which was read later by the radiologist as likely artifact). Computed tomography angiogram (CTA) of the head and neck was negative for any vascular abnormality. Unfortunately, due to the patients’ large body habitus, she was unable to fit into the magnetic resonance imaging (MRI) machine. Repeat head CT at later time showed no changes compared to previous head CT.
The patient continued to show a decline in neurological and respiratory status which ultimately required intubation. Due to her rapid decline and deficits that localized to the brainstem, she underwent a significant workup investigating possible vascular etiologies, infectious etiologies as well as a possible acute demyelinating process. Treatment with intravenous immunoglobulin (IVIG) or plasma exchange was deferred initially secondary to the broad diagnostic possibilities. Conventional cerebral angiogram was pursued and it was negative for aneurysm, vasculitis changes or any other abnormality.
Cerebrospinal fluid(CSF) studies showed elevated IgG/Alb ratio to 0.28(normal range 0.00-0.21), normal glucose 50mg/ dl, normal protein 28mg/dl, and no pleocytosis.Electromyogram (EMG) and nerve conduction study(NCS) was performed which was normal but had limited interpretation due to ICU artifact. Preliminary laboratory investigation into myasthenia gravis, thyroid pathology, autoimmune, infectious, or metabolic etiologies were all negative.
She was treated with 5 days of intravenous immunoglobulin (IVIG). Due to prolonged intubation and inability to liberate from the ventilator, the patient underwent tracheostomy and percutaneous endoscopic gastrostomy (PEG) tube placement. Her symptoms minimally improved. She was offered plasma exchange; but she declined. At the end of her hospitalization, GQ1b antibody result returned positive. She declined placement to a skilled nursing facility and she was discharged to home with tracheostomy and PEG tube training.
At 1-month clinic follow up, her diet was advanced by speech therapy and she could tolerate soft foods. She still had unsteady gait and required the assistance of a rolling walker. Her diplopia and ptosis improved, but the slight left facial droop persisted.
Autoimmune diseases affecting the Central Nervous System are not uncommon, and disease pathology can represent a spectrum of demyelinating disease or inflammatory process. The traditional clinical diagnostic criteria for GBS includes progressive motor weakness of more than one limb with areflexia [1]. CSF will often show elevated protein after one week of symptoms [1], and electrodiagnostics studies will show nerve conduction slowing or complete conduction block in up to 80% of patients[1]. Not all nerves are affected, and the electromyography is often normal in the first few weeks of illness[1]. The combination of clinical, laboratory and electrodiagnostic criteria to diagnose GBS were established by both neurologists and non-neurologists to simplify the diagnosis; however, it has been discovered that multiple variations of GBS have now been described which do not meet the original criteria.
The most common form of Guillain-Barre syndrome is acute inflammatory demyelinating polyneuropathy (AIDP). AIDP comprises 80-90% of GBS and is characterized by progressive ascending paralysis, with selective leg weakness (56%), arm and leg weakness (32%), and arm weakness (12%) [7-9]. A prodromal illness of upper respiratory infection or diarrhea, most commonly with campylobacter jejuni, is identified in 60- 70% of cases [7,9]. Electromyography shows demyelination with or without axonal loss with reduced H reflex in 97% and F waves in 84% with increased. Demyelination features are present in at least two different nerves [9].
The second most common form of GBS is acute motor axonal neuropathy (AMAN) and represents 5-10% of GBS cases in the United States. AMAN is a rapidly progressive weakness of both upper and lower extremities, sparing the face, with a loss of reflexes[10]. Like AIDP, AMAN is typically preceded by a respiratory infection or campylobacter jejuni[5,11]. AMAN does not have sensory or peripheral nerve demyelination [12] and electrophysiology shows axonal degeneration with reduced or absent distal compound muscle action potentials [10]. Conduction block can also be seen on EMG which can show reversibility; however, without evidence of remyelination [10]. The associated ganglioside autoantibodies for AMAN are listed in Table 1.
In addition to AMAN, acute sensorimotor axonal neuropathy (AMSAN) is also characterized by rapidly progressive weakness; however, AMSAN differs in that it also includesaxonal damage to the dorsal and ventral roots, causing prominent sensory manifestations [13,8]. Over the course of the disease, AMSAN can also involve small fibers causing burning pain and autonomic symptoms [14].
Miller Fisher syndrome (MFS), as mentioned briefly above, is a variant of GBS which is characterized by ophthalmoplegia, ataxia, and areflexia [2]. Descriptions of the syndrome were first published by Dr. Miller Fisher in NEJM in 1956 [15]. Patients can have severe ataxia with normal muscle strength. While areflexia is part of the triad, 43% of patients have normal reflexes [16]. Miller Fisher syndrome has more recently been associated with the anti-GQ1b antibodies and is positive in 85% of cases [2,4]. Bickerstaff encephalitis is similar to MFS with the exception of abnormal mental status examination and abnormal reflexes. Patients with this variant of GBS have hyperreflexia, rather than areflexia seen in MFS, and encephalopathy in addition to ophthalmoplegia and ataxia [17].Bickerstaff encephalitis, is also associated with the anti-GQ1b antibody [18].
Uncommon variants of GBS include acute pan-dysautonomia, pure sensory GBS, and acute bulbar palsy. Acute pandysautonomia is characterized by symptoms such as diarrhea, vomiting, dizziness, abdominal pain, orthostatic hypotension, urinary retention, pupillary abnormalities, variable heart rate and decreased sweating/salivation/lacrimation [19,20]. Reflexes are typically diminished or absent and patients can have variable sensory symptoms [19,20]. Acute pan-dysautonomia is a self-limiting disease process that completely resolves, the reason it is considered a variant of GBS [20]. Pan-dysautonomia is associated with antibodies to AChR and values greater than 1.00nmol/L were present in 50% of cases [19]. Elevated titer levels after 1 month correlated with increased symptoms [19].
Pure sensory GBS is also known as sensory ataxic neuropathy in some literature [21]. Pure sensory GBS is characterized by sensory symptoms, ataxia, from involvement of the large sensory fibers, and areflexia [21-23]. Antibody to GD1b is present in 46% of pure sensory GBS [21]. Ophthalmoplegia is not present in pure sensory GBS; however, GQ1b antibody has been positive in 50% of patients [21]. Sensory nerve conduction studies show decreased to absent sensory potentials as early as 6 days from symptom onset [22-25]. Motor nerve conduction studies are normal [22-25]. Follow up sensory nerve conduction studies show improved amplitude and velocity at 3-6 months; however, not complete recovery [22-25]. Patients have complete to near complete resolution of sensory symptoms within 1-3 months; however, patients have persistent areflexia with or without mild ataxia [22-25].
Our patient represented a combination of different GBS variants including MFS (ataxia and ophthalmoplegia), Bickerstaff encephalitis (normal to hyperreflexia), and pharyngeal- cervicalbrachial weakness (hoarseness and dysphagia). All of which share the same antibody (GQ1b). We can propose the term “Anti- GQ1b syndrome” to describe such patients who do not fit under one diagnosis, but rather under the spectrum of this disease[26].
When considering other possible CNS involvement from autoimmune causes many other autoantibodies should be considered, and a wide variety of neurological disturbances including encephalopathy, sleep disorders, epilepsy and neuropathy can be included in the differential diagnosis [27]. These types of autoantibodies can be nuclear, cytoplasmic, intracellular, or surface antigens (Examples of which are noted in Table 2 [27-31]. The site of the antigen is not specific to the clinical syndrome, and a wide spectrum of disease can be noted with a singular autoantibody, whether this was produced by a secreting tumor, or idiopathically. Similarly, you can surmise that Gq1b autoantibodies will also have a spectrum of disease rather than multiple discrete entities.
