Otto Frankl

Hypothyroidism masquerading as polymyositis

A group of patients is described who presented with proximal muscle weakness and elevated muscle enzymes. They were initially believed to have polymyositis and one of them was even treated as such over a long period of time. The patients were subsequently found to have hypothyroidism and responded well to thyroid hormone replacement. Hypothyroidism should always be considered in the differential diagnosis of polymyositis. A polymyositis-like picture has to be regarded as a symptom and not as a disease since it occurs in several metabolic, infectious, malignant and drug-induced diseases.

Campylobacter Enteritis Associated with Recurrent abortions in Agammaglobulinemia

Abstract. Campylobacter enteritis and habitual abortions occurred in a 28‐year‐old patient suffering from agamma‐globulinemia. Campylobacter jejuni has been recently recognized to be a common treatable cause of diarrhea in man. Since bacterial overgrowth of the small intestine is increased in hypo‐ or agammaglobulinemia, Campylobacter is expected to be found in those patients. As this patients abortions might have been due to the same infection, Campylobacter should be considered in the differential diagnosis of habitual abortions, especially if associated with diarrheal disease. This treatable disease must be sought in every case of agammaglobulinemia and gastro‐intestinal complaints, before complicated diagnostic procedures are performed or potentially toxic drugs are administered.

Corticosteroid responsive pulmonary hypertension in systemic lupus erythematosus

SummaryPrimary pulmonary hypertension is an irreversible and fatal disorder. Every effort should therefore be made to discover all the other treatable diseases which may be associated with pulmonary hypertension. The association of systemic lupus erythematosus and pulmonary hypertension was rarely reported in the past. We add another case in which pulmonary hypertension was the presenting symptom of systemic lupus erythematosus (SLE). In contrast to the previously reported cases, our patient responded well to corticosteroids. It is assumed that this favorable response was due to the relatively early stage of the disease, when the histopathologic pulmonary changes were still in the reversible inflammatory stage.

Guillain-Barré syndrome associated with acute cytomegalovirus mononucleosis syndrome.

A case of Guillain-Barré syndrome (GBS) in a young patient who suffered from an acute cytomegalovirus (CMV) infection is presented. The diagnosis was reached following the isolation of the virus from the urine and the demonstration of high titers of both IgM and IgG antibodies in the serum. In contrast to previous cases in which GBS was merely associated with positive serology for CMV, but with almost no clinical manifestations of CMV mononucleosis syndrome, our patient had a full blown picture of acute CMV mononucleosis, including peripheral lymphocytosis, splenomegaly, hepatic involvement and hemolysis. Severe transient hypocomplementemia was also noted.

Multifocal Neuropathy and Vocal Cord Paralysis in Relapsing Fever

A 19-year-old soldier developed multifocal neuropathy following an infection with tick-borne Borrelia. He suffered from involvement of the right accessory nerve, the right brachial plexus and the left recurrent laryngeal nerve. As was expected, all these complications were reversible. In contrast to louse-borne relapsing fever, neurological manifestations in the tick-borne variants are not common. To the best of our knowledge, vocal cord paralysis has never been described before.

The Significance of the Cerebrospinal Fluid Examination in the Management of Chlorpropamide-induced Hypoglycemia

Chlorpropamide-induced hypoglycemia is often overlooked, misdiagnosed, and mistreated, because of the atypical, insidious, and intermittent clinical picture and because of the normal serum glucose level in some of the patients when arriving at the hospital. These facts are demonstrated in three case reports. Since the correction of low glucose levels in the cerebrospinal fluid occurs hours after its correction in the serum, examining and at times following the cerebrospinal fluid glucose levels in patients with chlorpropamide-induced neuroglycopenia will enable physicians to diagnose and cure more patients. A high index of suspicion should exist in the presence of any atypical encephalopathy, mainly in the elderly diabetic patient treated with chlorpropamide and suffering from impaired cerebral, hepatic, or renal function. By suspecting and identifying neuroglycopenia, disabling residual deficits and even death could eventually be prevented.

Oral contraceptive-induced chorea

A case history of a patient with (OC) oral contraceptive-induced chorea is described. An 18 year old woman experienced involuntary movements of both upper extremities; she had received OCs 2 months prior to the onset of those symptoms. In addition to these choreoathetoid movements the patient exhibited grimaces exacerbated by stress. All laboratory procedures were normal as were electrocardiogram and electroencephalogram. OCs were discontinued and propranolol (4 times/day) was administered in an attempt to suppress choreiform movement. This was unsuccessful; however 1 month after OC discontinuance reduction in these movements was noted and grimaces disappeared. Within 10 weeks complete resolution was observed. Although the etiology of OC-induced chorea is not known clinicians should be aware of this potential side effect as chorea can be eliminated by stopping the drug.

Cerebral involvement in primary mixed cryoglobulinaemia

Primary mixed cryoglobulinaemia was diagnosed in a 65-year-old woman and confirmed on post-mortem examination. The patient had the classical findings of purpura, arthralgia, renal failure, mixed cryoglobulins in the serum and rheumatoid factor activity. The patient presented with haemolytic anaemia and later developed rectal bleeding, but the most prominent feature was an acute psychosis with electroencephalographic changes, that was attributed to the disease and responded well to corticosteroids. Primary mixed cryoglobulinaemia should be included in the differential diagnosis of central nervous system involvement in systemic connective tissue diseases. This complication should not be missed since it seems to respond well to corticosteroids and/or to plasmapheresis if diagnosed in time.

Corticosteroid induced remission of oesophageal involvement in mixed connective tissue disease

Oesophageal involvement is known to be one of the most severe and resistant manifestations of connective tissue diseases, mainly progressive systemic sclerosis (PSS). A patient who had manifestations of systemic lupus erythematosus, PSS and polymyositis is described. Since some features of mixed connective tissue disease (MCTD) respond to corticosteroid treatment, fluocortolone was administered and both clinical and radiological remission of the oesophageal involvement were observed. At the same time, there was also a marked improvement of the cutaneous lesions. In view of the described observation, it seems advisable to try this treatment for oesophageal manifestations of MCTD.

Recurrent transient ischemic attacks associated with thrombocytosis in rheumatoid arthritis

SummaryThrombocytosis may appear in rheumatoid arthritis, nevertheless, thromboembolic phenomena have rarely been recorded. This case describes a 71-year old patient suffering from long-standing seropositive and nodular rheumatoid arthritis with severe pulmonary involvement. During an exacerbation of her disease and following the appearance of thrombocytosis, several episodes of transient ischemic attacks occurred. The neurological manifestations were right facial nerve paralysis, paraesthesia of the right cheek and dysarthria. The patient was treated successfully by antiaggregants, anticoagulants and busulfan.