General paresis of insane: A rarity or reality?

There is a widely held clinical opinion that syphilis has disappeared. Contrary to this, there are sporadic cases being reported across the country. The classical cases of neurosyphilis have given way to the milder asymptomatic forms, making them even more difficult to be diagnosed. Conventional presentation of neurosyphilis such as tabes dorsalis and general paresis of insane are read in textbooks only and rarely encountered in clinical practice in the 21(st) century.

Syphilis is believed to be brought to India by the Portugese in the 16th century. Though its prevalence is decreasing, it is yet to be eradicated.[1] Psychiatric manifestations appear late in the course of the disease and can range from subtle changes in personality, affective, and psychotic symptoms to cognitive decline like delirium and dementia.[2] Asymptomatic cases and cases with ill-defined syndrome have become more common than the classic presentation of tabes dorsalis and general paresis.[3]

CASE REPORT

We present here a case of a 54-year-old male who had presented to the Psychiatry O.P.D. of S.C.B. Medical College, Cuttack with dementia like features. He was an employee of Orissa state. He had presented with complaints of forgetfulness, irritability, crying spells, fearfulness, and urinary and fecal incontinence. He was unable to take care of himself and was unable to go to work.

The patient was apparently alright 2 years back. His symptoms had started with features of withdrawn behavior which the family members considered to be due to work-related stress. He used to keep to himself and would not share his concerns or day to day happenings. After about 3–4 months, he became hyperactive. He would often get up early in the morning and would rush for doing his daily activities. He had become more talkative and querulous over minimum provocation. His spending behavior had also increased. He was taken to a psychiatrist who diagnosed him to be having bipolar affective disorder with the current episode manic. He was started on 750 mg of divalproex and 10 mg of haloperidol. After about a month of therapy his symptoms had subsided. Though the excitement had abated, the patient had problems of forgetfulness. He often would venture out and forget the way back. He needed assistance in day to day activities. He could no longer attend to his work. He had started remaining fearful and would prefer to stay indoors and keep the doors and windows of the house closed. He would often burst into tears for no apparent reason. He would soil his trousers with urine or stool. In spite of taking the treatment, his condition was deteriorating.

He gave a history of suffering from a penile ulcer 15 years back which was treated. He denied having contact with any commercial sex worker or extramarital affair and had no history of substance use. General physical examination was within normal limits. Neurological evaluation was also normal.

Mental status examination revealed an appropriately dressed middle-aged man. He took the chair when offered by the examiner but did not greet the examiner in spite of the examiner's greeting. An eye-to-eye contact and rapport could only partially be established. He appeared mildly agitated and had soft speech. Productivity of speech was also decreased. He appeared distressed and started crying for which he could give no reason. He had ideas of persecution but denied any perceptual disturbances. There was no disorder of thought form. Higher mental function tests showed that he was conscious and cooperative. Orientation to time and place was impaired. He had intact orientation to persons. His attention span was decreased, immediate, and recent memories were impaired but remote memory was intact. Both calculation and abstract thinking were impaired. Insight was judged to be partial. Judgment was impaired in all dimensions. The MMSE score was 6 which indicated severe cognitive impairment.

A provisional diagnosis of dementia was made, he was hospitalized, and neuropsychological evaluation and appropriate investigations were planned. Neuropsychological tests included PGI-BBD which gave a score of 32 suggesting significant cognitive dysfunction indicating organic brain pathology. Routine laboratory investigations were all within normal limits. Special lab tests pertaining to dementia were within normal limits. CT scan of brain was normal. Serum VDRL was positive in 1:128. Serological tests of treponemal variety were planned. The serum treponema pallidum hemagglutination test (TPHA) was positive in 1:2560. Both IgM and IgG, florescent treponemal antibody absorption test (FTA-ABS) was positive. CSF was also tested for CNS infection. CSF VDRL was positive. CSF protein was 50 mg/dl, sugar 52 mg/dl (raised), leucocyte count was 20/cu.mm/ (raised). He was diagnosed as having dementia due to neurosyphilis.

He was started on 24 million units of aqueous penicillin G I/V qid and donepezil 5 mg. He was discharged after 20 days with minimal improvement in MMSE scores and was advised to report after 1 month. After 1 month his VDRL test was positive in 1 in 16 dilutions.

DISCUSSION

This case had classical presentation of general paresis of insane as it typically started with affective symptoms which gradually progressed to cognitive decline leading to frank dementia. General paresis of insane is a parenchymal brain disease where antibiotics can only stop progression of the disease but cannot bring complete cure.[4] A high index of suspicion as needed to diagnose the cases of neurosyphilis which generally lack the typical neurological and ophthalmological findings. This case underlines the need to consider the diagnosis of neurosyphilis when investigating for the cause of dementia in middle age populations. In addition to appropriate medical management, prompt diagnosis would also help in arresting the transmission of the disease in the community.